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Ecthyma | Eczema or dermatitis | Elastosis perforans serpiginosus | Ephelides | Epidermal naevi | Epidermolysis bullosa | Erythema multiforme | Erythema nodosum | Erythrasma | Erythroderma and exfoliative dermatitis | Eye bags

ECTHYMA

Ecthyma is a deep ulcerative form of impetigo. It can affect people of all ages but is more common in children, elderly people and people with diabetes or who are immunocompromised from disease or medication.

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ECZEMA OR DERMATITIS

Eczema or dermatitis refers to an inflammation of the skin characterised by redness, swelling, weeping and scaling. It is usually itchy and constant scratching leads to lichenification (leathery thickening of the skin). Doctors divide eczemas into two broad groups:

Exogenous (exo means external and gen means production in Greek) eczemas are caused by external factors such as allergy to cement and plaster or irritation from chemicals, soaps and detergents (see contact dermatitis). Endogenous (endo means internal) eczemas, on the other hand has to do with the skin's make-up or constitution. Hence, they are also called constitutional eczemas. Endogenous or constitutional eczemas cannot be cured whereas exogenous eczemas can be if the causal substance can be avoided.

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Ectodermal dysplasia (ED)


Ectodermal dysplasia (ED) refers to a group of genetic disorders characterised by defects in the hair, nails, teeth, skin, nails and sweat gland function. Other tissues of ectodermal origin, such as the ears, eyes, lips, mucous membranes of the mouth, nose, throat and central nervous system, may be affected as well. The ectoderm is the outermost layer of cells that forms the tissues mentioned above during feotal life. All ectodermal dysplasias are present from birth and are non-progressive. Dysplasia literally means “abnormal tissue growth.”

Cause
Most cases are inherited although less common, EB occurs without a family history, in which case it is due to spontaneous mutation.
ED is caused by mutation or deletion of certain genes.

The different types of ectodermal dysplasia are caused by the mutation or deletion of certain genes located on different chromosomes. Because ectodermal dysplasias are caused by a genetic defect they may be inherited or passed on down the family line. In some cases, they can occur in people without a family history of the condition, in which case a spontaneous mutation has occurred.

The combination of physical features a person has and the way in which it is inherited determines if it is an ectodermal dysplasia. For example, hypohidrotic ectodermal dysplasia affects the hair, teeth and sweat glands while Clouston syndrome affects the hair and nails.

More than 180 different types of ectodermal dysplasias exist. Yet, most types share some common symptoms, ranging from mild to severe. The early diagnosis of a specific type will help identify which combination of symptoms the person has or will have.

Types
More than 180 subgroups have been described based on the presence or absence of the four primary ectodermal dysplasia (ED) defects:The 180+ ectodermal dysplasias are recognized and named based on the specific combination of symptoms shown in affected individuals. The pattern of these features is important when a physician tries to make a formal diagnosis.

The following are the most common types of ectodermal dysplasia but it has to be said theat the largest group is diagnosed as just Ectodermal Dysplasia, type unspecified.

Hypohidrotic (anhidrotic) ED (HED) - inherited in one of three patterns: X-linked recessive, autosomal recessive and autosomal dominant.
Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome (EEC) - Mutation in the TP63 gene on chromosome 3q28. Autosomal dominant
Clouston syndrome - Also called hidrotic ectodermal dysplasia because affected individuals sweat normally and exhibit no heat intolerance. Autosomal dominant disorder.
Ankyloblepharon (fused eyelids) Ectodermal Dysplasia Clefting (AEC Syndrome) - also known as Rapp-Hodgkin Syndrome. Hay-Wells Syndrome is probably the same. Mutation in the TP63 gene on chromosome 3q28. Autosomal dominant.
Tooth nail syndrome
Focal Dermal Hypoplasia (FDH) (also known as Goltz syndrome)
Incontinentia Pigmenti (IP)

Symptoms
The signs and symptoms of ectodermal dysplasia depend on the structures that are affected. These are often not apparent at birth and are often only picked up during infancy or childhood.

Affected organ
Hair
Scalp and body hair may be thin, sparse, and light in colour
Hair may be coarse, excessively brittle, curly or even twisted

Nails
Fingernails and toenails may be thick, abnormally shaped, discoloured, ridged, slow growing, or brittle
Sometimes nails may be absent
Cuticles may be prone to infection

Teeth
Abnormal tooth development resulting in missing teeth or growth of teeth that are peg-shaped or pointed
Tooth enamel is also defective
Dental treatment is necessary and children as young as 2 years may need dentures

Sweat glands
Eccrine sweat glands may be absent or sparse so that sweat glands function abnormally or not at all
Without normal sweat production, the body cannot regulate temperature properly
Children may experience recurrent high fever that may lead to seizures and neurological problems
Overheating is a common problem, particularly in warmer climates

Other signs and symptoms include:
Lightly pigmented skin, in some cases red or brown pigment may be present. Skin can be thick over the palms and soles and is prone to cracking, bleeding and infection.
Skin may be dry and is prone to rashes and infection.
Dry eyes occur due to lack of tears. Cataracts and visual defects may also occur.
Abnormal ear development may cause hearing problems.
Cleft palate/lip.
Missing fingers or toes (digits).
Respiratory infections due to lack of normal protective secretions of the mouth and nose.
Foul smelling nasal discharge from chronic nasal infections.
Lack of breast development.

Treatment

There is no specific treatment for ectodermal dysplasia. Management of the condition is by treating the various symptoms. Patients often need to be treated by a team of doctors and dentists, rather than a sole practitioner.

Patients with abnormal or no sweat gland function should live in cooler climates or in places with air conditioning at home, school and work. Cooling water baths or sprays may be useful in maintaining a normal body temperature.
Artificial tears can be used to prevent damage to the cornea in patients with defective tear production. Saline sprays can also be helpful.
Saline irrigation of the nasal mucosa may help to remove purulent debris and prevent infection.
Early dental evaluation and intervention is essential.
Surgical procedures such as repairing a cleft palate may lessen facial deformities and improve speech.
Wigs may be worn to improve the appearance of patients with little or no hair.

Most people with ectodermal dysplasia can lead a full and productive life once they understand how to manage their condition. Special attention must be paid to children if sweating and mucous production abnormalities are present. Recurrent high fevers may lead to seizures and neurological problems.

Ehlers-Danlos syndrome (EDS)

Ehlers-Danlos syndrome (EDS) is the name given to a group of inherited disorders caused by a defect in connective tissue synthesis and structure. This leads to fragile, sagging skin, and loose joints. fragile tissue and blood vessels. EDS may occur in males and females of all races and usually first appears in young adults.

The Ehlers-Danlos syndromes (EDS) are currently classified in a system of thirteen subtypes. Each EDS subtype has a set of clinical criteria that help guide diagnosis; a patient’s physical signs and symptoms will be matched up to the major and minor criteria to identify the subtype that is the most complete fit. There is substantial symptom overlap between the EDS subtypes and the other connective tissue disorders including hypermobility spectrum disorders, as well as a lot of variability, so a definitive diagnosis for all the EDS subtypes—except for hypermobile EDS (hEDS)—also calls for confirmation by testing to identify the responsible variant for the gene affected in each subtype.

Classical EDS (cEDS) - A final diagnosis requires confirmation by molecular testing. More than 90% of those with cEDS have a heterozygous mutation in one of the genes encoding type V collagen (COL5A1 and COL5A2). Rarely, specific mutations in the genes encoding type I collagen can be associated with the characteristics of cEDS. Classical EDS is inherited in the autosomal dominant pattern.
Skin hyperextensibility and atrophic scarring; and
Generalized joint hypermobility (GJH).

Vascular EDS (vEDS) - Patients with vEDS typically have a heterozygous mutation in the COL3A1 gene encoding type III collagen. Autosomal dominant.
Family history of vEDS with documented causative variant in COL3A1;
Arterial rupture at a young age;
Spontaneous sigmoid colon perforation in the absence of known diverticular disease or other bowel pathology;
Uterine rupture during the third trimester in the absence of previous C-section and/or severe peripartum perineum tears; and
Carotid-cavernous sinus fistula (CCSF) formation in the absence of trauma.

A genetic defect causes reduced amounts of collagen, disorganisation of collagen that is usually organised into bundles, and alterations in the size and shape of collagen. The type of EDS a patient has depends on how collagen metabolism has been affected. For example vascular EDS is caused by decreased or absent synthesis of type III collagen.

Skin hyperextensibility: it is easy to pull the skin away from the body and once released it retracts to its original state.
Skin fragility: the skin easily splits. Wounds heal very slowly resulting in gaping fish-mouth or cigarette paper scars.
Epicanthic folds: skin folds between the eyes make the bridge of the nose appear wide.
Molluscoid pseudotumours: small spongy lumps 2-3cm in diameter over pressure points such as the knees and elbows.
Nodules: small, firm lumps just below the skin surface on the arms and shins.
Hypermobility: joints bend more than usual. The fingers are most often affected but all joints can be involved. The joints may dislocate but can be popped back painlessly.
Bruising and haematomas may arise after trivial injuries.
Internal collagen defects: heart murmur (mitral valve prolapse) and arterial/intestinal/uterine fragility or rupture (usually associated with the Vascular Type).
scoliosis at birth and scleral fragility (associated with the Kyphoscoliosis Type)
poor muscle tone (associated with the Arthrochalasia Type)

ELASTOSIS PERFORANS SERPIGINOSA (EPS)

This is a rare disorder in which abnormal elastic tissue is being pushed out of the skin (a process known as transepidermal elimination). It usually appears in early childhood or young adulthood and males are more commonly affected.

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Elastomas or elastin naevi include:

Isolated elastomas
Buschke-Ollendorf syndrome
Elastosis perforans serpiginosa
Buschke-Ollendorf syndrome is a rare hereditary disorder where there is an increased accumulation of elastin in the dermis (elastoma). Lesions may be present at birth but more usually appear within the first year of life. They are firm yellowish wrinkled nodules often group together to form plaques. The abdomen, back, buttock, thighs or arms are commonly affected. Other manifestations of the syndrome appear with time and may include osteopoikilosis (inherited bone disorder identified on X-Ray), eye disorders and spinal problems. autosomal dominant connective tissue disorder due to mutations in the LEMD3 gene (607844) on chromosome 12q14.

Elastosis perforans serpiginosa (EPS) is a perforating disorder. In this case, abnormal elastic fibres are extruded through the epidermis. It presents in adolescence and typically affects one or more sites on the face, neck and/or arms. Groups of scaly papules are generally arranged in an arc or ring shape. EPS is associated with other disorders of connective tissue such as Marfan syndrome, Ehler Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum and Down syndrome.

 

Elephantiasis nostras verrucosa (ENV)

Elephantiasis nostras verrucosa (ENV) is a rare form of chronic lymphedema that causes progressive cutaneous hypertrophy. It can lead to severe disfiguration of body parts with gravity-dependent blood flow, especially the lower extremities. Various factors can cause obstruction of the lymphatic system and result in ENV. Clinically, ENV is characterized by nonpitting edema and superimposed hyperkeratotic papulonodules with a verrucose or cobblestone-like appearance. (Early-stage lesions might exhibit pitting edema; late-stage lesions exhibit nonpitting edema.) It needs to be differentiated from pretibial myxedema, filariasis, lipedema, chromoblastomycosis, lipodermatosclerosis, and venous stasis dermatitis (Table 1).1

edema and fibrosis of the skin. Several conditions that block lymphatic drainage can induce lymphedema, including neoplasms, trauma, radiation treatment, congestive heart failure,1 obesity, hypothyroidism,6 chronic venous stasis,2 and filarial infection.

The pathogenesis of ENV is still unclear. It is conceivable that first the lymphatic channels are damaged and blocked due to one or more of the above-mentioned conditions, and excessive protein-rich fluid accumulates in the dermis and subcutaneous tissues. Second, the protein-rich fluid decreases oxygen tension and might decrease the immunity of the skin. Third, poor immunity increases the skin’s susceptibility to infection by micro-organisms. Finally, there is swelling, fibrosis, and disfiguration of the affected areas.4 Hence, a vicious cycle begins, as the underlying conditions predispose the skin to microbial infections.

Elephantiasis nostras verrucosa is commonly observed in gravity-dependent parts of the body, especially in the lower extremities. In addition, other sites including the upper extremities, abdomen, buttocks, face, or scrotum might be involved.7–9 Elephantiasis nostras verrucosa usually begins at the dorsal aspect of the foot and then progresses to the proximal parts of the limbs. In the beginning, the lesion presents as mild and persistent pitting edema. Later, the affected area loses its elasticity and eventually has a hypertrophic, verrucose, cobblestone-like appearance. During the physical examination, observation of the Kaposi-Stemmer sign—inability to pinch the dorsal aspect of skin at the base of the second toe5—is characteristic of lymphedema. This phenomenon is attributed to skin thickening caused by lymphedema.

Although the clinical presentation is distinct, other diseases such as venous stasis dermatitis, filariasis, lipedema, chromoblastomycosis, lipodermatosclerosis, and pretibial myxedema should be clearly differentiated from ENV (Table 1).1–5

The diagnosis of ENV is mainly based on patient history, physical examination, and typical cutaneous lesions. To identify causes of secondary lymphedema, skin biopsy and imaging techniques including computed tomography, magnetic resonance imaging, lymphangiography, and lymphoscintigraphy can be necessary.

In the management of ENV, it is crucial to treat the underlying causes. Lymphostasis can be managed conservatively using medical bandages, compression stockings, and mechanical massages. Elastic bandage compression is reported to be an effective treatment.10 Diuretics and systemic antibiotics might be needed to reduce edema and control infection. In addition, hyperkeratotic plaques can be treated with topical keratolytics or systemic retinoids.1 Owing to the teratogenicity of systemic retinoids, it is important to provide contraception to female patients before treatment. Patients should also receive careful monitoring of serum lipids and liver function. Surgical intervention can be considered in recalcitrant cases when the response to medical treatment is poor.11 However, unsatisfactory outcomes are common in the management of advanced stages of ENV.


Enlarged pores

Enlarged pores are depressions in the facial skin surface that contain one or more openings to the ducts carrying sweat and oil from their respective eccrine glands and sebaceous glands.

Enlarged pores can be seen at all ages and in all ethnic groups. Certain ethnic groups may have larger pores, particularly those of African and Indian ancestry. Pores often appear larger with age.

Factors that may lead to enlarged pores include:

Increased sebum production
Hair follicle size
Use of comedogenic products
Loss of skin elasticity with age
Sun damage.
Acne is associated with enlarged pores — when sometimes open comedones (blackheads) can be seen within a pore. Inflammatory acne may cause enlarged pores through weakening sebaceous gland and hair follicle openings, making them more prone to blockage.

Treatments that focus on preventing and shrinking large pores are not very effective. They include:

Avoidance of skin creams that induce blackheads and whiteheads
Weight loss (which is reported to reduce sebum production)
Chemical peels
Topical nicotinamide
Plant-derived copper chlorophyllin complexes
L-carnitine
Topical retinoids.
Oral treatments that are used for acne may also help. These include:

Combined oral contraceptives
Spironolactone
Isotretinoin.

Physical treatments targeting the sebaceous glands may help enlarged pores. These include:

Laser treatment
Radiofrequency microneedling (skin needling).

Eosinophilic cellulitis (Wells syndrome)

What is Wells syndrome?

Wells syndrome is a rare condition of unknown cause. It is also called ‘eosinophilic cellulitis’.

What does Wells syndrome look like?

Typically the rash is preceded by itching or burning skin and consists of markedly swollen nodules and plaques (lumps) with prominent borders. The patches are usually bright red at first, frequently looking like cellulitis, then fade over four to eight weeks, leaving green, grey or brown patches. They can blister. The rash most commonly occurs on the limbs, but may also affect the trunk.

The patient often feels very tired and has a fever in approximately 25% of cases.

Wells syndrome
Wells syndrome
Investigations

A blood count may reveal increased numbers of white blood cells called eosinophils – these are often associated with allergy or insect bites.

The diagnosis of Wells syndrome can be established by a skin biopsy finding of typical histopathological features with many eosinophils and characteristic ‘flame figures’. However, flame figures are not diagnostic of Wells syndrome and can be seen in other conditions that have increased numbers of eosinophils.

An important part of the management of patients with Wells syndrome is to exclude underlying causes such as parasitic disoders (e.g. a worm infestation) or an allergic contact dermatitis with the help of the appropriate tests.

Treatment

Oral corticosteroid treatment with prednisone can lead to a dramatic improvement within days and the course is typically tapered over one month. Other treatments include minocycline, dapsone, griseofulvin, ciclosporin and oral antihistamines.

Mild cases may respond to topical steroid therapy alone.

eosinophilic fasciitis

Eosinophilic fasciitis is a rare scleroderma-like disorder characterised by inflammation, swelling and thickening of the skin and fascia (fibrous tissue that separates different layers of tissues under the skin). It affects the forearms, the upper arms, the lower legs, the thighs, and the trunk (in order of decreasing frequency). The disease is considered by some to be a deep variant of the skin condition, morphoea.

Eosinophilic fasciitis
Eosinophilic fasciitis
What causes eosinophilic fasciitis and who gets it?

The cause of eosinophilic fasciitis is unknown but it may have something to do with abnormal immune responses as hypergammaglobulinaemia and antinuclear antibodies are present. In addition, toxic, environmental, or drug exposures have been implicated.

It affects females and males, children and adults, with most cases occurring between the ages of 30 and 60 years.

What are the clinical features of eosinophilic fasciitis?

Patients usually present suddenly with painful, tender, swollen and red extremities. Within weeks to months patients develop stiffness and affected skin becomes indurated, creating a characteristic orange-peel appearance over the surfaces of the extremities. In severely affected areas, the skin and the subcutaneous tissue are bound tightly to the underlying muscle. This creates a woody-type appearance.

In 50% of cases, the disease is precipitated by an episode of strenuous physical exercise or activity. Other signs and symptoms that may be present include:

Malaise, weakness, fever and weight loss
Joint contractures of the elbows, wrists, ankles, knees and shoulders
Carpal tunnel syndrome
Inflammatory arthritis
How is eosinophilic fasciitis diagnosed?

Laboratory studies in early active disease show eosinophilia, elevated ESR and polyclonal immunoglobulin G. However, full thickness skin biopsy that includes the dermis, subcutaneous fat and fascia is necessary to confirm a diagnosis of eosinophilic fasciitis, which has specific pathological features.

What is the treatment for eosinophilic fasciitis?

Treatment of eosinophilic fasciitis is directed at preventing tissue inflammation. Oral corticosteroids are the mainstay of treatment, with most patients responding well to moderate-to-high doses of corticosteroids particularly if started early in the course of the disease. Continued low doses may be required for 2-5 years. In some cases, the disease may resolve spontaneously.

Other drugs that may be used in conjunction with corticosteroids include:

Nonsteroidal anti-inflammatory agents (NSAIDs)
Hydroxychloroquine
Colchicine
Cimetidine
Azathioprine
Cyclosporine
Cyclophosphamide
Methotrexate
A physiotherapist is also important in the overall management of eosinophilic fasciitis to prevent and treat joint contractures.

Eosinophilic folliculitis

Eosinophilic folliculitis is a recurrent skin disorder of unknown cause. It is also known as "eosinophilic pustular folliculitis" or "Ofuji disease". Skin biopsies of this disorder find eosinophils (a type of immune cell) around hair follicles – hence its name.

There are several variants of eosinophilic folliculitis.

All of them present with itchy papules (bumps) or pustules. Eosinophilic folliculitis is rare and more often affects males than females. Variants include:

Classic type – this occurs most commonly in Japan
Eosinophilic folliculitis associated with advanced Human Immunodeficiency Virus (HIV) infection
Infantile eosinophilic folliculitis
Cancer-associated eosinophilic folliculitis
Medication-associated eosinophilic folliculitis
What does eosinophilic folliculitis look like?

Eosinophilic folliculitis presents with red or skin-coloured dome shaped papules (bumps) and pustules. It may look rather like acne or other forms of folliculitis. The papules mostly appear on the face, scalp, neck and trunk and may persist for weeks or months. Less commonly, urticarial lesions are seen (these are larger red irritable wheal-like patches similar to urticaria). Palms and soles may rarely develop similar papules and pustules, but in such cases the condition should not be called "folliculitis" as there are no follicles in these areas.

Longstanding cases may develop dermatitis or a form of prurigo, presumably because of the itching and scratching.

Eosinophilic folliculitis of HIV
Eosinophilic folliculitisEosinophilic folliculitisEosinophilic folliculitisEosinophilic folliculitis
How is eosinophilic folliculitis diagnosed?

Skin biopsy reveals eosinophils under the skin surface and around the hair follicles and sebaceous glands. In many cases blood tests show a mild rise in eosinophil cells and immunoglobulin-E (IgE), and reduced IgG and IgA levels.

Eosinophilic folliculitis is often a feature of immunodeficiency. Eosinophilic folliculitis associated with HIV infection presents when levels of CD4 lymphocyte cells drop below 300 cells/mm3, a level at which there is an increased risk of a secondary opportunistic infection. Cases of eosinophilic folliculitis have also reported after bone marrow transplantation before the immune system is back to normal functioning, and in some individuals with inherited immune deficiencies.

What is the cause of eosinophilic folliculitis of HIV?

The cause of eosinophilic folliculitis of HIV is not known. Immunodeficiency appears to lead to increased risk of allergic-type skin diseases. There is no proof that bacterial, fungal or viral secondary infection is the cause, although some researchers have postulated overgrowth of malassezia or demodex (the hair follicle mite) might be involved. Another theory is that there is a change in the immune system causing eosinophils to attack the sebum (oils produced in the skin) of sebaceous gland cells.

What is the treatment for eosinophilic folliculitis?

In patients with HIV, eosinophilic folliculitis is likely to improve or resolve with HAART (Highly Active Anti-Retroviral Treatment), as CD4 cell counts rise above 250/mm3.

Other treatments that may be effective include:

Indomethacin and other nonsteroidal anti-inflammatory drugs are reported effective in up to 70% of cases of eosinophilic folliculitis
Dapsone
Tetracycline antibiotics
Other antibiotics including metronidazole
Phototherapy
Topical steroids
Calcineurin inhibitors such as tacrolimus ointment
Oral antihistamines such as cetirizine
Colchicine
Itraconazole
Permethrin cream (topical insecticide)
Nicotine patches
Isotretinoin and acitretin.

EPHELIDES

Ephelides are freckles most commonly seen in children with fair skins, especially those of Celtic origin.

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Epidermoid cyst

A cyst is a benign, round, dome-shaped encapsulated lesion that contains fluid or semi-fluid material. It may be firm or fluctuant and often distends the overlying skin. There are several types of cyst. The most common are described here.

What is a pseudocyst?

Cysts that are not surrounded by a capsule are better known as pseudocysts. These commonly arise in acne.

Who gets cysts?

Cysts are very common, affecting at least 20% of adults. They may be present at birth or appear later in life. They arise in all races. Most types of cyst are more common in males than in females.

What causes cysts?

The cause of many cysts is unknown.

Epidermoid cysts are due to proliferation of epidermal cells within dermis. Their origin is the follicular infundibulum. Multiple epidermoid cysts may indicate Gardner syndrome.
An epidermal inclusion cyst is a response to an injury. Skin is tucked in to form a sac that is lined by normal epidermal cells that continue to multiply, mature and form keratin.
The origin of a trichilemmal cyst is hair root sheath. Inheritance is autosomal dominant (the affected gene is within short arm of chromosome 3) or sporadic.
The origin of steatocystoma is the sebaceous duct within the hair follicle. Steatocystoma multiplex is sometimes an autosomal dominantly inherited disorder due to mutations localised to the keratin 17 (K17) gene, when it may be associated with pachyonychia congenita. More often, steatocysts are sporadic, when these mutations are not present.
The origin of the eruptive vellus hair cyst is follicular infundibulum. It may be inherited an autosomal dominant disorder due to mutations in keratin gene.
A dermoid cyst is a hamartoma, a developmental error.
The origin of a ganglion cyst is degeneration of the mucoid connective tissue of a joint.
Occlusion of pilosebaceous units (hair follicles) or eccrine sweat ducts leads to a build-up of secretions. This can present as milia.
Occlusion of the orifice of a mucous gland can lead to a fluid-filled cyst in a mucous membrane (lip, vulva, vagina).
A milium is a pseudocyst due to failure to release keratin from an adnexal structure. The origin of primary milium is infundibulum of vellus hair follicle at the level of the sebaceous gland; a tiny version of an epidermoid cyst. The origin of secondary milium is a retention cyst within a vellus hair follicle, sebaceous duct, sweat duct or epidermis.
Pseudocysts in acne are formed by occlusion of the follicle by keratin and sebum.
What are the clinical features of cysts?

Epidermoid cyst

Epidermoid cysts occur on face, neck, trunk or anywhere where there is little hair.
Most epidermoid cysts arise in adult life.
They are more than twice as common in men as in women.
They present as one or more flesh–coloured to yellowish, adherent, firm, round nodules of variable size.
A central pore or punctum may be present.
Keratinous contents are soft, cheese-like and malodorous.
Scrotal and labial cysts are frequently multiple and may calcify.
Epidermoid cyst is also called follicular infundibular cyst, epidermal cyst, keratin cyst.

Epidermoid cyst
CystCystGardner syndrome
Gardner syndrome
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Trichilemmal cyst

90% of trichilemmal cysts occur on scalp; otherwise face, neck, trunk, and extremities.
Most trichilemmal cysts arise in middle age.
In 70% of cases, trichilemmal cysts are multiple.
They presents as adherent, round or oval, firm nodules.
There is no punctum.
The keratinous content is firm, white and easily enucleated.
A trichilemmal cyst is also called pilar cyst.

Trichilemmal cyst
Pilar cystPilar cystsPilar cyst
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Steatocystoma

A solitary steatocystoma is known as steatocystoma simplex.
More often, there are multiple lesions (steatocystoma multiplex) on chest, upper arms, axillae and neck.
The cysts arise in the late teens and 20s due to the effect of androgens, and persist lifelong.
They are freely moveable, smooth flesh to yellow colour papules 3–30 mm in diameter.
There is no central punctum.
Content of cyst is predominantly sebum.
Steatocystoma multiplex
SteatocystSteatocystoma multiplexSteatocystoma multiplex
Eruptive vellus hair cysts

Eruptive vellus hair cysts are present in childhood if familial, and later if sporadic.
Multiple 2–3 mm papules develop over the sternum.
The cysts contain vellus hairs.
Dermoid cyst

A cutaneous dermoid cyst may include skin, skin structures and sometimes teeth, cartilage and bone.
Most dermoid cysts are found on face, neck, scalp; often around eyelid, forehead and brow.
It is a thin-walled tumour that ranges from soft to hard in consistency.
The cyst is formed at birth but the patient may not present until an adult.
Dermoid cysts
Dermoid cystDermoid cystDermoid cyst
Ganglion cyst

A ganglion cyst most often involves scapholunate joint of dorsal wrist.
These arise in young to middle-aged adults.
They are 3 times more common in women than in men.
The cyst is a unilocular of multilocular firm swelling 2–4 cm in diameter that transilluminates.
Cyst contents are mainly hyaluranic acid, a golden-coloured goo.
Ganglion cyst
Ganglion cystGanglion cystGanglion cyst
Mucous/myxoid pseudocysts arise in older adults on distal phalanx
They arise from distal interphalangeal joint, associated with osteoarthritis.
They often present as a longitudinal depression in the nail due to compression on the proximal matrix.
Myxoid pseudocyst
Digital myxoid cystMyxoid cystDigital myxoid cyst
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Labial mucous/myxoid cyst

A cyst in the lip may be due to occlusion of the salivary duct
They are also called mucocoele.
It is a soft to firm firm, 5–15 mm diameter, semi-translucent nodule.
Mucocoele of lip
Mucocoele of the lipMucocoeleMucocoele
Hidrocystoma

Hidrocystoma is a translucent jelly-like cyst arising on an eyelid.
It is also known as cystadenoma, Moll gland cyst, and sudoriferous cyst.
The common solitary translucent eyelid cyst is an apocrine hidrocystoma.
Multiple cysts on the lower eyelid are eccrine hidrocystomas.
Hidrocystoma of eyelid
HidrocystomaHidrocystomaHidrocystoma
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Milium/milia

Milia are 1–2 mm superficial white dome-shaped papules containing keratin
Primary milia arise in neonates (50%), adolescents and adults; they are rarely familial and sometimes eruptive.
Primary milia occur on eyelids, cheeks, nose, mucosa (Epstein pearls) and palate (Bohn nodules) in babies; and eyelids, cheeks and nose of older children and adults.
Transverse primary milia are sometimes noted across nasal groove or around areola.
In milia en plaque, multiple milia arise on an erythematous plaque on face, chin or ears.
Secondary milia arise at the site of epidermal repair after blistering or injury, eg epidermolysis bullosa, bullous pemphigoid, porphyria cutanea tarda, thermal burn, dermabrasion.
Secondary milia are reported as an adverse effect of topical steroids, 5-fluorouracil cream, vemurafenib and dovitinib.
Milia
MiliaEyelid miliaMilia
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Vulval mucous cyst

A vulval mucous cyst is due to occlusion of Bartholin or Skene duct.
It presents as a soft swelling in the introitus of vagina: a posterior swelling is a Bartholin cyst and a periurethral swelling is a Skene cyst.
Comedo and acne pseudocyst

Comedones are pseudocysts formed by occlusion of follicle by keratin and sebum.
The open comedo (whitehead) and closed comedo (blackhead) are small, superficial papules typical of acne vulgaris
Solar comedones arise in sun-damaged skin and are associated with smoking.
Large uninflamed pseudocysts accompany inflammatory nodules in nodulocystic acne and hidradenitis suppurativa.
Comedones
Open comedones
Open comedones
Closed comedones
Closed comedones
Solar comedones
Solar comedones
Pseudocyst of auricle

Pseudocyst of auricle (external ear) follows trauma.
Pseudocyst of auricle
Auricular pseudocyst

Complications of cysts

Rupture of a cyst

The contents of the cyst may penetrate the capsular wall and irritate surrounding skin.
The area of tender, firm inflammation spreads beyond the encapsulated cyst.
Sterile pus may be discharged.
Secondary infection

A ruptured cyst may infrequently become secondarily infected by Staphylococcus aureus, forming a furuncle (boil).
Pressure effect

A dermoid cyst can cause pressure on underlying bony tissue.
A ganglion cyst can cause joint instability, weakness, limitation of motion and may compress a nerve.
A digital mucous cyst may place pressure on the proximal matrix and cause malformation of the nail.
Malignancy

Cutaneous cysts and pseudocysts are non-proliferative benign lesions.
Nodulocystic basal cell carcinoma is a common skin cancer that presents as a rounded nodule and may initially be mistaken for a cyst, but steady enlargement, destruction of the epidermis with ulceration and bleeding occur eventually.
Malignant proliferative trichilemmal cyst is actually a misnomer. It is an extremely rare tumour.
How are cysts diagnosed?

Cysts are usually diagnosed clinically as they have typical characteristics. When a cyst is surgically removed, it should undergo histological examination. The type of lining of the wall of cyst and the cyst contents help the pathologist classify it.

Epidermoid cysts are lined with stratified squamous epithelium that contains a granular layer. Laminated keratin contents are noted inside the cyst. An inflammatory response may be present in cysts that have ruptured.
Trichilemmal cysts have a palisaded peripheral layer without granular layer. Contents are eosinophilic hair keratin. Older cysts may exhibit calcification. The proliferating variety is considered a tumour.
Steatocystoma has a folded cyst wall with prominent sebaceous gland lobules.
Dermoid cyst contains fully mature elements of the skin including fat, hairs, sebaceous glands, eccrine glands, and in 20%, apocrine glands.
The lining of the wall of a ganglion cyst or digital mucous cyst is collagen and fibrocytes. It contains hyalin material.
Hidrocystoma has a thin lining wall of eosinophilic bilaminar cells.
What is the treatment for cysts?

Asymptomatic epidermoid cysts do not need to be treated. In most cases, attempt to remove only the contents of a cyst is followed by recurrence. If desired, cysts may be fully excised. Recurrence is not uncommon, and re-excision may be surgically challenging.

Inflamed cysts are sometimes treated with:

Incision and drainage
Intralesional injection with triamcinolone
Oral antibiotics
Delayed excision biopsy
How can cysts be prevented?

Unknown.

What is the outlook for cysts?

Cysts generally persist unless surgically removed.

EPIDERMAL NAEVI

Epidermal naevi are developmental abnormalities caused by an overgrowth of the epidermis (upper layers of the skin). They appear at birth or during childhood, usually in the first year of life. The abnormality arises from a defect in the ectoderm. This is the outer layer of the embryo that gives rise to epidermis and neural tissue. Epidermal nevus is a clinical term for a family of skin lesions that involve the outer portion of skin, the epidermis, and are distributed in a linear and often swirled pattern. Overall, epidermal nevi are not uncommon congenital malformations, occurring in 1-3 per 1000 births.

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EPIDERMOLYSIS BULLOSA (EB)

Epidermolysis bullosa (EB) is a group of rare inherited disorders characterised by blistering of the skin after minor trauma. Some forms of EB are mild with few blisters but others may be more severe with many blisters on the skin and even inside the body such as the mouth, oesophagus, stomach, respiratory tract, bladder, and elsewhere.There several types of epidermolysis bullosa.

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erysipelas

what is erysipelas?
Erysipelas is a superficial form of cellulitis, a potentially serious bacterial infection affecting the skin.
Erysipelas affects the upper dermis and extends into the superficial cutaneous lymphatics. It is also known as St. Anthony's fire, with reference to the intense rash associated with it.
Who gets erysipelas?
Erysipelas most often affects infants and the elderly, but can affect any age group. Risk factors are similar to those for other forms of cellulitis. They may include:
Previous episode(s) of erysipelas
Breaks in the skin barrier due insect bites, ulcers and chronic skin conditions such as psoriasis, athlete’s foot and eczema
Current or prior injury (eg trauma, surgical wounds, radiotherapy)
In newborns, exposure of the umblical cord and vaccination site injury
Nasopharyngeal infection
Venous disease (eg gravitational eczema, leg ulceration) and/or lymphoedema
Immune deficiency or compromise, such as
Diabetes
Alcoholism
Obesity
Human immunodeficiency virus (HIV)
Nephrotic syndrome
Pregnancy
What causes erysipelas?
Unlike cellulitis, almost all erysipelas is caused by Group A beta haemolytic streptococci (Streptococcus pyogenes). Staphylococcus aureus, including methicillin resistant strains (MRSA),Streptococcus pneumoniae,Klebsiella pneumoniae,Yersinia enterocolitica, andHaemophilus influenzae have also been found to cause erysipelas.
What are the clinical features of erysipelas?
Symptoms and signs of erysipelas are usually abrupt in onset and often accompanied by fevers, chills and shivering.
Erysipelas predominantly affects the skin of the lower limbs, but when it involves the face it can have a characteristic butterfly distribution on the cheeks and across the bridge of the nose.
The affected skin has a very sharp, raised border.
It is bright red, firm and swollen. It may be finely dimpled (like an orange skin).
It may be blistered, and in severe cases may become necrotic.
Bleeding into the skin may cause purpura.
Cellulitis does not usually exhibit such marked swelling, but shares other features with erysipelas, such as pain and increased warmth of affected skin.
In infants, it often occurs in the umbilicus or diaper/napkin region.
Bullous erysipelas can be due to co-infection with Staphylococcus aureus (including MRSA).
Erysipelas
ErysipelasErysipelasErysipelasErysipelas
What are the complications of erysipelas?
Erysipelas recurs in up to one third of patients due to:
Persistence of risk factors
Lymphatic damage (hence impaired drainage of toxins)
Complications are rare but can include:
Abscess
Gangrene
Thrombophlebitis
Chronic leg swelling
Infections distant to the site of erysipelas:
Infective endocarditis (heart valves)
Septic arthritis
Bursitis
Tendonitis
Post-streptococcal glomerulonephritis (a kidney condition affecting children)
Cavernous sinus thrombosis (dangerous blood clots that can spread to the brain)
Streptococcal toxic shock syndrome (rare)
How is erysipelas diagnosed?
Erysipelas is usually diagnosed by the characteristic rash. There is often a history of a relevant injury. Tests may reveal:
Raised white cell count
Raised C-reactive protein
Positive blood culture identifying the organism
MRI and CT are undertaken in case of deep infection.
What is the treatment for erysipelas?
General
Cold packs and analgesics to relieve local discomfort
Elevation of an infected limb to reduce local swelling
Compression stockings
Wound care with saline dressings that are frequently changed
Antibiotics
Oral or intravenous penicillin is the antibiotic of first choice.
Erythromycin, roxithromycin or pristinamycin may be used in patients with penicillin allergy.
Vancomycin is used for facial erysipelas caused by MRSA
Treatment is usually for 10–14 days
What is the outlook for erysipelas?
While signs of general illness resolve within a day or two, the skin changes may take some weeks to resolve completely. No scarring occurs.
Long term preventive treatment with penicillin is often required for recurrent attacks of erysipelas.
Erysipelas recurs in up to one third of patients due to persistence of risk factors and also because erysipelas itself can cause lymphatic damage (hence impaired drainage of toxins) in involved skin which predisposes to further attacks.
If patients have recurrent attacks, long term preventive treatment with penicillin may be considered.
 
erythema ab igne
What is erythema ab igne?
Erythema ab igne (EAI) is a skin reaction caused by chronic exposure to infrared radiation in the form of heat. It was once a common condition seen in the elderly who stood or sat closely to open fires or electric space heaters. Although the introduction of central heating has reduced EAI of this type, it is still found in individuals exposed to heat from other sources.
What are the signs and symptoms and who is at risk?
Limited exposure to heat, insufficient to cause a direct burn, causes a mild and transient red rash resembling lacework or a fishing net. Prolonged and repeated exposure causes a marked redness and colouring of the skin (hyper- or hypo-pigmentation). The skin and underlying tissue may start to thin (atrophy) and rarely sores may develop. Some patients may complain of mild itchiness and a burning sensation.
Erythema ab igne
Erythema ab igneErythema ab igneErythema ab igneErythema ab igne
Localised lesions seen today reflect the different sources of heat that people may be exposed to. Examples include:
Repeated application of hot water bottles or heat pads to treat chronic pain, e.g. chronic backache
Repeated exposure to car heaters or furniture with built-in heaters
Occupational hazard for silversmiths and jewellers (face exposed to heat), bakers and chefs (arms)
What treatments are available?
The source of chronic heat exposure must be avoided. If the area is only mildly affected with slight redness, the condition will resolve by itself over several months. If the condition is severe and the skin pigmented and atrophic, resolution is unlikely. In this case, there is a possibility that squamous cell carcinomas may form. If there is a persistent sore that doesn't heal or a growing lump within the rash, a skin biopsy should be performed to rule out the possibility of skin cancer. Abnormally pigmented skin may persist for years. Treatment with topical tretinoin or laser may improve the appearance.
erythema dyschromicum perstans
 
What is erythema dyschromicum perstans?
Erythema dyschromicum perstans is also called ashy dermatosis (of Ramirez), because of its colour. It is a benign skin condition characterised by well-circumscribed round to oval or irregular patches on the face, neck and trunk that are grey in colour. They may be symmetrical in distribution or unilateral.
Early lesions may be reddish in colour, often with a more pronounced border, and they may be somewhat elevated. However, this phase is not always observed.
The patient is otherwise well with no associated disease or blood test abnormality.
Erythema dyschromicum perstans
Erythema dyschromicum perstansErythema dyschromicum perstansErythema dyschromicum perstans
Who gets erythema dyschromicum perstans?
Erythema dyschromicum perstans most often affects darker skinned patients, most frequently Latin Americans and Indians. However it has also been reported in people of lighter skin colour and various ethnicities.
Erythema dyschromicum perstans may occur at any age but it appears to be more frequent in young adults. Women are affected more often than men.
What causes erythema dyschromicum perstans?
The exact cause for the disease remains unidentified. It is often classified as a variant of lichen planus because of its histopathological features. Over the years, theories have included:
Genetic susceptibility
Toxic effects of chemicals such as ammonium nitrate or barium sulphate
Whipworm infestation
Viral infections
Adverse effect of drugs and medications
What is the differential diagnosis?
Several other skin conditions may appear similar to erythema dyschromicum perstans because they also result in discoloured skin patches.
Lichen planus pigmentosus (erythema dyschromicum perstans may be a variant of this disorder)
Multiple lesions of fixed drug eruption
Postinflammatory hyperpigmentation
Urticaria pigmentosa
Incontinentia pigmenti
Pinta
Leprosy
How is it diagnosed?
In some cases the clinical picture may be classical enough to diagnose the condition. A skin biopsy may need to be performed in other cases to aid diagnosis. This may reveal minor vacuolar degeneration of the basal layer in early lesions, and pigmentary incontinence with dermal melanophages in more established patches.
What is the treatment?
Unfortunately erythema dyschromicum perstans is rather resistant to currently available treatments. It may persist unchanged for years although some cases eventually clear up by themselves.
Treatments that may help improve the appearance include:
Topical steroids
Exposure to ultraviolet radiation
Pigment lasers e.g., Q-switched ruby laser
Chemical peels
The most successful systemic treatment has been clofazimine. Dapsone, griseofulvin, hydroxychloroquine, isoniazid and corticosteroids have been used successfully in a few cases.
 
Erythema elevatum diutinum (EED)
 
Erythema elevatum diutinum (EED) is a rare type of necrotising vasculitis that is characterised by red, purple, brown or yellow papules (raised spot), plaques, or nodules, found on the backs of the hands, other extensor surfaces overlying joints, and on the buttocks.
Erythema elevatum diutinum
Erythema elevatum diutinumErythema elevatum diutinumErythema elevatum diutinum
Who gets erythema elevatum diutinum?
EED may occur in any age group, but patients are typically between 30 and 60 years old. It occurs equally in men and women.
What is the cause of erythema elevatum diutinum?
EED is classified as a small vessel vasculitis. The cause of EED is not yet defined, but it has been associated with the following conditions:
Granuloma faciale
Recurrent bacterial infections (especially streptococci)
Viral infections (including hepatitis B and HIV)
Haematological diseases
Rheumatological diseases
What are the clinical features of erythema elevatum diutinum?
Lesions usually start as papules or nodules on the backs of the hands.
Other extensor surfaces affected include the knees, elbows, wrists, ankles, fingers and toes. Buttocks, trunk, forearms, legs, palms and soles may also be affected.
Lesions occurring on the face are indistinguishable from granuloma faciale.
Lesions usually appear symmetrically.
Colour of lesions progress over time from yellow or pinkish to red, purple or brown.
Lesions may enlarge during the day and go back to original size overnight.
Rarely, blisters and ulcers may form.
Lesions usually feel firm and freely movable over the underlying tissue.
EED can be symptomless or painful, or cause an itching or burning sensation.
Symptoms can worsen after exposure to cold.
Arthralgia may be present.
How is erythema elevatum diutinum diagnosed?
Several tests are available to establish a diagnosis of EED.
Skin biopsy (most important); it shows leukocytoclastic vasculitis
Direct immunofluorescence
What is the treatment for erythema elevatum diutinum?
EED is a chronic and progressive skin disease that may last as long as 25 years. However, in some cases after evolving over a 5-10 year period it may spontaneously clear.
Medication can be used to limit progression of the disease.
Dapsone is considered the drug of choice for EED, mainly because of its rapid onset of action and clinical experience has shown good responses. However, lesions promptly recur following withdrawal of the drug.
Other drugs that have been occasionally reported to be effective include:
Niacinamide
Colchicine
Hydroxychloroquine
Clofazimine
Cyclophosphamide.
Oral corticosteroids are generally ineffective.
What is the outcome for erythema elevatum diutinum?
EED generally persists for months, years or decades. It often recurs after apparently successful treatment.
 
 
Erythema induratum (Bazin disease)
Erythema induratum (Bazin disease) presents as recurring nodules or lumps on the back of the legs (mostly women) that may ulcerate and scar. It is a type of nodular vasculitis
 
Erythema infectiosum
 
Erythema infectiosum is a common childhood infection causing a slapped cheek appearance and a rash. It is also known as fifth disease and human erythrovirus infection.
What is the cause of erythema infectiosum?
Erythema infectiosum is caused by an erythrovirus, EVB19 or Parvovirus B19. It is a single-stranded DNA virus that targets red cells in the bone marrow. It spreads via respiratory droplets, and has an incubation period of 7–10 days.
Who gets erythema infectiosum?
Erythema infectiosum most commonly affects young children and often occurs in several members of the family or school class. Thirty percent of infected individuals have no symptoms. It can also affect adults that have not been previously exposed to the virus.
Erythema infectiosum
Fifth diseaseFifth diseaseFifth disease
More images of erythema infectiosum ...
What are the symptoms of erythema infectiosum?
Parvovirus B19 infection causes nonspecific viral symptoms such as mild fever and headache at first. The rash, erythema infectiosum, appears a few days later with firm red cheeks, which feel burning hot. This lasts 2 to 4 days, and is followed by a pink rash on the limbs and sometimes the trunk. This develops a lace-like or network pattern.
Although most prominent in the first few days, the rash can persist for up to six weeks at least intermittently, and is most obvious when warm.
Complications of erythema infectiosum
Although usually a mild childhood condition, erythrovirus B19 infection can result in complications. These include:
Polyarthropathy in infected adults (painful, swollen joints)
Aplastic crisis or potentially dangerous low blood cell count in patients with haemolytic blood disorders such as autoimmune haemolytic anaemia and sickle cell disease
Spontaneous abortion, intrauterine death (9%) or hydrops fetalis in 3% of the offspring of infected pregnant women. This can occur if erythema infectiosum occurs in the first half of pregnancy. Parvovirus B19 does not cause congenital malformations. As the risk of an adverse outcome is low, the infection is not routinely screened for in pregnancy
Chronic parvovirus infection in immunodeficient patients, such as organ transplant recipients, causing erythropoietin-resistant anaemia, proteinuria, and glomerulosclerosis in a renal allograft
Rarely, encephalitis, hepatitis, non-occlusive bowel infarction, amegakaryocytic thrombocytopenia, myositis and heart disease
How is the diagnosis of erythema infectiosum made?
In most cases, erythema infectiosum is a clinical diagnosis in a child with characteristic slapped cheek and lacy rash. Parvovirus can cause other rashes such as a papular purpuric gloves and socks syndrome. The diagnosis can be confirmed by blood tests.
Parvovirus serology: IgG, IgM. This test is reported in about 7 days.
Parvovirus PCR is more sensitive. This test is reported in about 3 days.
In situ hybridisation or immunohistochemistry on biopsy specimens
If the child is unwell, or has haemolytic anaemia, a full blood count should be performed. Ultrasound examination and Doppler examination of at-risk pregancies can detect hydrops fetalis.
Treatment of erythema infectiosum
Erythema infectiosum is not generally a serious condition. There is no specific treatment. Affected children may remain at school, as the infectious stage or viraemia occurs before the rash is evident.
The application of an ice-cold flannel can relieve the discomfort of burning hot cheeks.
Red blood cell transfusions and immunoglobulin therapy can be successful in chronic parvovirus infection or during an aplastic crisis.
Hydrops fetalis due to parvovirus infection is treated by intrauterine transfusion.

 

ERYTHEMA MULTIFORME

Erythema multiforme is a hypersensitivity reaction to infections or drugs or othetr trriggers characterised by “target” lesions that are symmetrically distributed with a propensity for the distal extremities. in the past SJS and TEN were considered as the a severe form, erythema multiforme, with its minimal mucous membrane involvement and less than 10 percent epidermal detachment, is now considered as a separate entity.

Consensus classification
According to a consensus definition, Steven-Johnson syndrome was separated from the erythema multiforme spectrum and added to toxic epidermal necrolysis. [3] Essentially Steven-Johnson syndrome and toxic epidermal necrolysis (TEN) are considered severity variants of a single entity. The 2 spectra are now divided into the following: (1) erythema multiforme consisting of erythema minor and major and (2) Steven-Johnson syndrome / toxic epidermal necrolysis (SJS/TEN).
The clinical descriptions are as follows:
Erythema multiforme minor - Typical targets or raised, edematous papules distributed acrally
Erythema multiforme major - Typical targets or raised, edematous papules distributed acrally with involvement of one or more mucous membranes; epidermal detachment involves less than 10% of total body surface area (TBSA).
SJS/TEN - Widespread blisters predominant on the trunk and face, presenting with erythematous or pruritic macules and one or more mucous membrane erosions; epidermal detachment is less than 10% TBSA for Steven-Johnson syndrome / toxic epidermal necrolysis and 30% or more for toxic epidermal necrolysis.

May affect any age group but is most common in young adults (20–40 years of age).

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Erythema nodosum is a type of panniculitis (inflammation of the fat) characterised by painful inflamed nodules (large swellings) on the limbs, most commonly on the shins.

Generally, it is idiopathic, although the most common identifiable cause is streptococcal pharyngitis. Erythema nodosum may be the first sign of a systemic disease such as tuberculosis, bacterial or deep fungal infection, sarcoidosis, inflammatory bowel disease, or cancer. Certain drugs, including oral contraceptives and some antibiotics, also may be etiologic. The hallmark of erythema nodosum is tender, erythematous, subcutaneous nodules that typically are located symmetrically on the anterior surface of the lower extremities. Erythema nodosum does not ulcerate and usually resolves without atrophy or scarring. Most direct and indirect evidence supports the involvement of a type IV delayed hypersensitivity response to numerous antigens. A deep incisional or excisional biopsy specimen should be obtained for adequate visualization. Erythema nodosum represents an inflammatory process involving the septa between subcutaneous fat lobules, with an absence of vasculitis and the presence of radial granulomas. Diagnostic evaluation after comprehensive history and physical examination includes complete blood count with differential; erythrocyte sedimentation rate, C-reactive protein level, or both; testing for streptococcal infection (i.e., throat culture, rapid antigen test, antistreptolysin-O titer, and polymerase chain reaction assay); and biopsy. Patients should be stratified by risk for tuberculosis. Further evaluation (e.g., purified protein derivative test, chest radiography, stool cultures) varies based on the individual. Erythema nodosum tends to be self-limited. Any underlying disorders should be treated and supportive care provided. Pain can be managed with nonsteroidal anti-inflammatory drugs.

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Erythema marginatum rheumaticum
 
Erythema marginatum rheumaticum – This is a characteristic type of annular erythema that occurs in about 10% of first attacks of ARF in children; it is very rare in adults. The rash can be difficult to detect in dark-skinned people. When present, it is found on the trunk and upper arms and legs, but almost never on the face, palms or soles. The rash appears as pink or red macules (flat spots) or papules (small lumps), which spread outwards in a circular shape. As the lesions advance, the edges become raised and red, and the centre clears. The lesions are not itchy or painful, and sometimes go unnoticed by the patient. The lesions can fade and reappear within hours, reappearing in hot conditions. Erythema marginatum may persist intermittently for weeks to months, even after successful treatment of ARF.
 
Erythema toxicum neonatorum
 
Erythema toxicum neonatorum is common and benigh eruption in newborns, affecting more than 50% of full term babies. It appears to be less common in premature babies. Erythema toxicum neonatorum may be present at birth but more often appears during the second or third day of life.
 
Typical lesions consist of erythematous, 2- to 3-mm macules and papules that evolve into pustules. Each pustule is surrounded by a blotchy area of erythema, leading to what is classically described as a “flea-bitten” appearance. Lesions usually occur on the face, trunk, and proximal extremities. Palms and soles are not involved.
Diagnosis
The diagnosis of toxic erythema of the newborn is usually made on clinical grounds.
Although toxic erythema of the newborn is benign and requires no treatment, a number of differential diagnoses should be considered.

ERYTHRASMA

Erythrasma is a superficial bacterial infection of the body folds such as the toe webs, groin and armpits. It is more common in warm climates and usually affects young adults. Widespread infections are associated with diabetes mellitus.

Erythrasma is classified into 3 types according to location.

Interdigital erythrasma: between 3rd, 4th and 5th toe web spaces
Intertriginous erythrasma: in armpits, groin, under the breasts and umbilicus
Generalised/disciform erythrasma: on the trunk

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ERYTHRODERMA AND EXFOLIATIVE DERMATITIS

Erythroderma refers to a diffuse reddish inflammation of the skin affecting more than 90% of the body surface. The term exfoliative dermatitis is used when most of the skin is exfoliating or shedding.

The extent of the skin changes can obscure the primary lesion making it difficult to diagnose the underlying cause.

Erythroderma is the clinical presentation of a wide range of cutaneous and systemic diseases (including psoriasis and atopic dermatitis), drug hypersensitivity reactions, and more rarely Sézary syndrome, a leukemic subtype of cutaneous T-cell lymphoma. Although uncommon in pediatric patients, erythroderma may be the clinical presentation of a wide range of acquired and inherited diseases, including infections, inflammatory skin diseases, ichthyoses, and congenital immunodeficiencies.

The mechanism of erythroderma is unknown. Adhesion molecules and their ligands (vascular cell adhesion molecule 1, intercellular adhesion molecule 1, E-selectin and P-selectin) play a major role in endothelial-leucocyte interaction, which affect the binding, transmigration and infiltration of lymphocytes and mononuclear cells during inflammation, injury or immunologic reaction. The rise in adhesion molecule expression stimulates dermal inflammation, which leads to epidermal proliferation and increased production of inflammatory mediators. However, there is no difference in adhesion molecule expression on endothelial cells between different types of erythroderma.Sézary Syndrome is a Th2 disorder with a selective expression of CCR4, whereas inflammatory erythroderma shares an overexpression of both Th1- and Th2- related chemokine receptors, suggesting a different pathophysiologic mechanism.

  • Spread of a pre-existing skin disease such as atopic dermatitis, seborrhoeic dermatitis, varicose eczema, contact dermatitis, psoriasis, pityriasis rubra pilaris and pemphigus foliaceus and bullous pemphigoid, Papuloerythroderma of Ofuji, norwegian crusted scabies.
  • Drug hypersensitivity reactions (see drug eruption) - especially antimalarials, allopurinol (gout medicine), non-steroidal anti-inflammatory drugs, phenytoin (used to treat epilepsy), gold (used to treat rheumatoid arthritis) and penicillin and sulphonamide antibiotics.
  • Underlying cancer - Sézary syndrome (a leukemic subtype of cutaneous T-cell lymphoma), lymphoma and leukaemia.
  • Several very rare congenital ichthyotic conditions and congenital immunodeficiencies. Genetic testing could be beneficial for certain congenital erythrodermas such as Netherton's syndrome and Conradi-Hunermann syndrome. Erythroderma may be the first clinical manifestation of an immunodeficiency syndrome, especially in the presence of diarrhea and profound failure to thrive. Immunodeficiency screening must be considered in these cases.
  • Graft-versus-host disease
  • HIV infection
  • Idiopathic Unknown.


    Symptoms
    Symptoms

  • Diffuse red inflamed skin.
  • Variable degrees of scaling and shedding (exfoliation) as the condition progresses.
  • Itching.
  • Lymphadenopathy is often present but is more often reactive than malignant. In difficult cases, a lymph node biopsy is recommended. However, the pathologist must be informed that the patient is erythrodermic for a reliable histopathological interpretation to be made.Lymph nodes become swollen (generalised dermatopathic lymphadenopathy).
  • Fatigue.
  • Fever.
  • Shivering from excessive heat loss. Failure of the mechanical barrier will result in loss of normal temperature control with failure to maintain the core body temperature.
      Erythroderma.
    Click on image for larger view


    Clues may be present as to the underlying cause.
  • Serous ooze, resulting in clothes and dressings sticking to the skin and an unpleasant smell, is characteristic of atopic erythroderma.
    Persistence of circumscribed scaly plaques in certain sites such as elbows and knees suggests psoriasis.
    Islands of sparing, follicular prominence, orange-hue to keratoderma are typical of pityriasis rubra pilaris.
    Subungual hyperkeratosis, crusting on palms and soles, and burrows are indicative of crusted scabies.
    Sparing of abdominal creases (deck chair sign) is typical of papuloerythroderma of Ofuji.
  •  
  •  
  • Complications
  • Hypothermia (low body temperature) due to increased heat loss from the dilated blood vessels in the skin.Shivering and hypothermia may occur even though the skin feels deceptively warm.
  • Cardiac failure due to increased blood flow through the skin putting a strain on the heart.Persistent inflammation of the skin leads to marked peripheral vasodilation and increased cutaneous blood flow. In combination with increased percutaneous loss of fluid, these can lead to high-output cardiac failure, especially in elderly patients.
  • Hypoalbuminemia (low serum albumin) is common and probably has several causes including increased plasma volume, decreased synthesis, increased metabolism, and protein loss via scaling and exudation.
  • Telogen effluvium and onychodystrophy may develop when erythroderma has been present for some weeks.
  • Iron deficiency anaemia.
  • Weight loss.
    Hypoalbuminemia is common and probably has several causes including increased plasma volume, decreased synthesis, increased metabolism, and protein loss via scaling and exudation.
  • Peripheral edema is common and can be due to a variety of causes, such as a shift of fluid into extracellular spaces, hypoalbuminaemia, concomitant cardiac failure and inflammation resulting from the primary skin disease. Occasionally, the increased permeability is severe enough to justify plasma infusions and parental steroids.
  • Severe pulmonary edema secondary to capillary leak syndrome and adult respiratory distress syndrome (ARDS) is a complication of erythroderma.
  • Colonization of the skin by Stahylococcus aureus is common and can lead to secondary cutaneous infections. Respiratory infections including pneumonia are common.

    An eruption of warty papules resembling seborrheic keratoses, "Murray Williams warts" can occasionally occur following resolution of an inflammatory dermatosis.Longstanding erythroderma may result in pigmentary changes (brown and/or white skin patches).
     
    What you can do

  • You should consult a doctor.

     
    What the doctor may do

  • Hospitalise for management.Investigation should involve hematology, urea and electrolytes, liver function tests and blood culture. Elevated erythrocyte sedimentation rate, raised IgE, anemia, hypoalbuminemia and hyperglobulinemia are frequent findings. Eosinophilia is generally a nonspecific finding, although a highly elevated count may be associated with malignancy, mainly T-cell lymphoma.
    Skin biopsies from several sites may be taken if the cause is unknown. They tend to show nonspecific inflammation on histopathology. Diagnostic features may be present however.
  • Direct immunofluorescence is of benefit if an autoimmune blistering disease or connective tissue disease is considered.
  • Determine and treat the underlying cause.
  • Prescribe oral and or topical steroids.
  • Prescribe antihistamines to reduce itching.

    Treat the complications.
    The course of idiopathic erythroderma is unpredictable. It may persist for a long time with periods of acute exacerbation.
     

    What is penile intraepithelial neoplasia?
    Penile intraepithelial neoplasia is a rare pre-cancerous disease of the outer skin layer (epidermis) of the penis.
    Other names for penile intraepithelial neoplasia include:
    Squamous intraepithelial lesion
    Erythroplasia of Queyrat
    Bowen disease of the penis
    in-situ squamous cell carcinoma of the penis
    P.I.N.
    How is penile intraepithelial neoplasia recognised?
    The diagnosis is often delayed, because penile intraepithelial neoplasia may resemble other conditions such as balanitis, candidiasis, dermatitis and psoriasis.
    Lesions are single or multiple, red plaques on the glans or inner aspect of the foreskin. They may have a smooth, velvety, moist, scaly, eroded or warty surface. The following signs and symptoms may occur:
    Redness and inflammation
    Itching
    Crusting or scaling
    Pain
    Ulcers
    Bleeding
    In the late stages, discharge from penis, difficulty pulling back foreskin or difficulty passing urine
    Images of penile intraepithelial neoplasia
    Who is at risk of penile intraepithelial neoplasia and what causes it?
    Uncircumcised males over 50 years of age are most at risk of getting penile intraepithelial neoplasia, although it may rarely occur in younger men.
    Penile intraepithelial neoplasia is associated with:
    Chronic infection with human papilloma virus (HPV), the cause of genital warts. HPV-16 is the most common type identified.
    Chronic skin disease, especially lichen sclerosus and lichen planus
    Smoking
    Immune suppression by medications or disease
    Chronic irritation by urine, friction or injury to the penile area.
    If left untreated, 10–30% of cases develop into invasive squamous cell carcinoma (cancer) of the penis.
    What is the treatment for penile intraepithelial neoplasia?
    Skin biopsy should be performed to confirm the diagnosis, as it may resemble other forms of chronic balanitis. Biopsy is also essential to rule out invasive squamous cell carcinoma, which requires more aggressive treatment.
    It is important to maintain good genital hygiene. Penile intraepithelial neoplasia can be treated in several different ways. Multidisciplinary care may be necessary.
    5-fluorouracil cream
    Imiquimod cream
    Cryotherapy
    Curettage and cautery
    Laser vaporisation
    Photodynamic therapy
    Radiotherapy
    Excision
    Interferon alpha
    Mohs micrographic surgery appears to be highly effective and the surgical treatment of choice in severe or recurrent cases of penile intraepithelial neoplasia.
    The disease recurs in 3-10% of patients, so close follow-up is necessary to ensure a complete cure.
    Partners of patients with penile intraepithelial neoplasia should be screened for other forms of intraepithelial neoplasia caused by human papilloma virus in the genital area (cervical, vulvar and anal cancer).
    Many national immunisation programmes now include a vaccine against the causative human papillomaviruses HPV-16 and 18. Vaccination of boys and young men should be included, to reduce the risk of developing penile intraepithelial cancer in the future.
     

    extramammary Paget disease

    What is extramammary Paget disease?
    Extramammary Paget disease is an uncommon cancer characterised by a chronic eczema-like rash of the skin around the anogenital regions of males and females. Under the microscope extramammary Paget disease looks very similar to the more common type of mammary Paget disease that occurs on the breast.
    Who gets extramammary Paget disease?
    Extramammary Paget disease most commonly occurs in the vulva of women aged between 50–60 years. It can also affect males of similar age.
    How is extramammary Paget disease classified?
    Extramammary Paget disease has been classified into several subtypes.
    Type 1a primary cutaneous extramammary Paget disease arises from apocrine glands within the epidermis (in situ) or underlying skin appendages
    Type 1b primary cutaneous extramammary Paget disease (15-25%) is associated with invasive Paget disease or adenocarcinoma in situ.
    Type 2 extramammary Paget disease originates from underlying anal or rectal adenocarcinoma
    Type 3 extramammary Paget disease originates from bladder adenocarcinoma
    Sometimes the extramammary Paget disease has been present for 10–15 years before evidence of cancer or metastases appear.
    What are the clinical features of extramammary Paget disease?
    The most common symptom of extramammary Paget disease is mild to intense itching of a lesion found around the groin, genitalia, perineum or perianal area. Pain and bleeding may occur from scratching lesions that have been around for a long time. Thickened plaques may form that can become red, scaly and crusty. Although they may appear similar to eczema, they fail to clear up with topical steroid creams.
    Extramammary Paget disease
    Extramammary Paget diseaseExtramammary Paget diseaseExtramammary Paget disease
    What sites are affected?
    In women the most common area involved is the vulva. First symptoms are usually itching and burning of of one or more persistent plaques. These may spread to the labia, mons pubis, vagina and thighs. Perianal lesions may extend up into the anal canal.
    The location of extramammary Paget disease is useful in predicting the risk of associated cancer. For example, 25-35% of extramammary Paget disease arising near the anus is associated with an underlying colorectal cancer.
    How is extramammary Paget disease diagnosed?
    Skin biopsy of the lesion is performed to get an accurate diagnosis of extramammary Paget disease as there are several other genital skin diseases that may appear similar. Under microscopy, the presence of Paget cells along with other histological findings confirms diagnosis. Special stains may be necessary to distinguish Paget disease from early melanoma (melanoma in situ). See extramammary Paget disease pathology.
    Further tests may include:
    Evaluation of lymph nodes by ultrasound scan or fine needle aspirate
    Search for other malignancies including PAP smear (cervical smear), pelvic imaging to look for underlying cancer, colonoscopy, mammography.
    What is the treatment of extramammary Paget disease?
    Wide local excision, vulvectomy, or if available, margin-controlled surgical excision (Mohs micrographic surgery) is the standard treatment for extramammary Paget disease . Paraffin sections are preferred over frozen sections. The margin is sometimes difficult to define particularly when lesions are spread sporadically throughout the anogenital region. Reconstruction may require skin grafting or flap repair.
    Recurrence is common (30-50%), so patients should be re-examined every 3 months after surgery for the next 2 years, after which annual follow-ups are recommended. Recurrence generally leads to further surgery.
    Non-surgical treatments for recurrent disease may include:
    Radiotherapy
    Laser ablation
    Photodynamic therapy
    5-fluorouracil cream
    Imiquimod cream
     

    EYE BAGS

    Eye bags are a common cause of concern because they make a person look old and less alert.

     
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