Varicella | Varicose eczema | Vasculitis | Vitiligo

Varicella or chickenpox is a viral infection that causes fever and blisters which crust over and heal after 2 to 3 weeks. It usually affects children but can also occur in adults who may develop a more severe and sometimes even fatal infection. The person becomes immune to further attacks of varicella but may develop herpes zoster (shingles) instead, later on in life.

Cause

• Varicella-zoster virus (VZV) which is transmitted through airborne droplets, skin to skin contact or contact with infected articles. The incubation period is 1 - 3 weeks. After infection, the varicella-zoster virus travels up a nerve and remains dormant (inactive) in the nerve ganglion (nerve relay station). If activated (usually in late adult life), the virus causes a more localised infection known as shingles (see herpes zoster).
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  Symptoms
  
• Most commonly seen in children under 10 years, but can affect any age
• Preceding fever, malaise (feeling of illness), headache and myalgia (muscle ache).
• Pink to red spots which develop a blister in the centre giving rise to the appearance of a "dewdrop on a rose petal".
• The vesicles crust over a 8 to 12 -hour period.
• Usually affects the trunk, scalp and face more than the limbs, although any part of the body may be affected.
• The eruption occurs in crops so that at any one time different evolutionary stages may be seen.
• Crusts will loosen after another 1 - 2 weeks.
• Rash is itchy.
• In total, lesions take 2 - 3 weeks to clear.

  Varicella (chickenpox) Click on image for larger view

  
  Complications

• Scarring which increases in the presence of secondary bacterial infection. These are often depressed (anetoderma) but may be thickened (hypertrophic scars).
• Secondary bacterial infection causing cellulitis, erypelas, funruncles and rarely, septicaemia (blood poisoning)
• Pneumonia (lung inflammation).
• Encephalitis (brain inflammation), Reye syndrome, Guillain-Barré syndrome and encephalitis.
• Disseminated infection can occur in immunocompromised individuals and can be fatal
• Death.
• Chickenpox in pregnant women carries additional risks for both mother and child.
• Severe chickenpox in pregnant mothers with increased risks of varicella pneumonia and other complications.
• Congenital varicella syndrome may develop if infection occurs in the first 3 months of pregnancy, resulting in an underweight baby with eye and brain abnormalities, shortened limbs, skin scarring, cataracts and growth retardation..
  Infection with varicella in the later stages of pregnancy can cause premature delivery or neonatal chickenpox infection. This is particularly serious if the mother becomes infected 7 days before birth
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• What you can do
• You should consult a doctor.
• Take antihistamines to relieve itching.
• Avoid scratching as it encourages secondary infection and scarring.
• Apply cool compresses.
• Apply calamine lotion to reduce itching.
• Stay in isolation. Varicella is infectious 5 days before the onset of the rash until all the eruptions have crusted.
• Take paracetemol for fever relief. Do not take aspirin as this has been associated with the development of Reye syndrome, a life-threatening condition causing brain and liver inflammation.

  What the doctor may do

• Diagnosis of chickenpox is usually made on the presence of its characteristic rash and the presence of different stages of lesions simultaneously. A clue to the diagnosis is in knowing that the patient has been exposed to an infected contact within the 10–21 day incubation period. Patients may also have prodromal signs and symptoms.
• Laboratory tests are often undertaken to confirm the diagnosis.
• PCR detects varicella virus in skin lesions and is the most accurate method for diagnosis.
  Culture of blister fluid is time consuming and is less frequently performed.
  Serology (IgM and IgG) is most useful in pregnant women, or prior to prescribing immune suppression medication to determine need for pre-treatment immunisation.
  For most healthy patients with chickenpox symptomatic therapy is usually all that is required.
• Prescribe oral acyclovir or valcyclovir which helps to reduce the severity of symptoms and hasten healing
• Prescribe topical or oral antibiotics for secondary bacterial infection.
• Give post-exposure prophylaxis in immunocompromised persns, pregnant women <20 weeks and newborns using Varicella zoster immune globulin (VZIG)
• After 20 weeks of pregnancy woman can be offered oral antiviral treatment.
  
  Prevention
• A vaccine is now available to prevent varicella.

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Varicose eczema or stasis dermatitis is a form of eczema that occurs on the legs of patients with chronic venous insufficiency. Women are more likely to be affected because they have a higher risk of developing varicose veins. It may be a precursor to more problematic conditions, such as venous leg ulceration and lipodermatosclerosis

Cause

• Poor return of blood from the leg veins to the heart due to faulty valves in the veins due to past deep venous thrombosis or DVT or unknown causes, results in blood pooling in the veins and back pressure, causing fluid to collect in the tissues. An inflammatory reaction occurs.

  Symptoms

• Redness, dryness and scaling, occasionally with weeping and crusting on the lower portion of the legs, especially on the inside of the ankles.
• Orange-brown pigmentation of the skin results from haemosiderin deposition.
• Itching is common.
• Atrophie blanche (white irregular scars surrounded by red spots)
  Fibrosis or stiffening of the skin leading to lipodermatosclerosis and a reversed “Champagne bottle” deformity of lower leg (narrowing at the ankles)
• Varicose veins are usually (but not always) present.
  
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  Differential diagnosis
  Gravitational eczema is often misdiagnosed as cellulitis. Cellulitis is nearly always unilateral, tender and has a well demarcated edge
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  Varicose (stasis) ulcer. Click on image for larger view

  Complications

• Ulceration.
• Secondary bacterial infection.
• Allergies often develop to topical medications used on the area.

  What you can do

• You should consult a doctor.
• Avoid scratching as this may cause ulceration and secondary bacterial infection.
• Apply saline compress if the area is weepy.
• Take antihistamines to reduce itch.
• Moisturise regularly
• Lose weight if obese.
• Avoid constipation by taking high fibre diet.
• Don't stand for long periods.
• Take regular walks.
• Put up the feet as often as possible. Raise the foot of the bed by about 10 inches to help return blood.
• Avoid constricting garments.
• Exercise or stretch the legs regularly.
• Wear compression bandages or supportive stockings.

  What the doctor may do

• Prescribe topical steroids.
• Prescribe antibiotics for secondary bacterial infection.
• Conduct patch tests to determine the cause of allergy, if necessary.
• Sclerotherapy (injections to force closure of the varicose veins) or surgery and endovascular laser to remove the varicose veins.
• Pinch grafts to help ulcers heal.
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Vasculitis refers to an inflammation of the blood vessel walls due to an immunological reaction. It may be localised and confined to the skin (cutaneous vasculitis) or systemic, affecting most of the body's vasculature (systemic vasculitis). Inflammation leads to narrowing of the lumen (channels) of blood vessels leading to narrowing, thrombosis (clotting) or haemorrhage, leading to ischaemia (lack of blood supply) to downstream organs, including heart, kidneys, intestines, muscles and nervous system. Because of their inflammatory nature, many vasculitis syndromes are accompanied by constitutional symptoms.

The focus of this discussion is on cutaneous vasculitis which can be classified, according to size of the blood vessels involved into:

Small vessel vasculitis

• Cutaneous small vessel vasculitis or CSSV (syn. leucocytoclastic vasculitis)
• Henoch-Schönlein purpura (HSP) - (fever, arthralgias, abdominal pain, hematura)
• Exercise-induced vasculitis
• Septic vasculitis
• Small vessel vasculitis associated with connective tissue disorders
• Urticarial vasculitis
• Waldenstrom’s hypergammaglobulinaemic purpura
• Mixed cryoglobulinaemia

Larger vessel vasculitis

• Polyarteritis nodosa (PAN), which is subdivided into:
  • Microscopic polyarteritis
  • Cutaneous PAN
  • Systemic PAN
    
• Granulomatous vasculitis
  • Wegener’s granulomatosis (syn. granulomatosis with polyangiitis)
  • Churg-Strauss syndrome (syn. allergic granulomatous angiitis)
    
• Giant cell arteritis eg Takayasu’s arteritis (temporal arteritis is rarely associated with cutaneous signs)
• Large vessel vasculitis associated with connective tissue disorders
• Nodular vasculitis (syn. erythema induratum of Bazin and of Whitfield)
  
  Others skin conditions classified as vasculitis
• Rheumatoid nodules
• Behçet’s syndrome
• Erythema elevatum diutinum
• Granuloma faciale
• Lymphomatoid granulomatosis
• Inflammatory bowel disease
• Sarcoid
• Haematological malignancies.
• Fibrin thrombi: present in segmented, hyalinizing vasculitis
  
  Causes
  There are many causes of vasculitis including:
• Idiopathic, eg no cause is identified in up to 50% of cases of cutaneous small vessel vasculitis
• Infection
  • Viral - many different viruses have been implicated including hepatitis B and C
  • Bacterial - several bacterial infections can be associated with vasculitis including streptococcal infections (Henoch Schonlein purpura), other causes of subacute bacterial endocarditis, meningococcal infection, and tuberculosis (associated with some cases of nodular vasculitis)
• Medications and other drugs
  • Are more likely to be associated with small vessel vasculitis, but are occasionally associated with systemic vasculitis
  • Blood eosinophilia is noted in up to 80% of cases with systemic involvement
  • The most common drugs implicated are penicillins, minocycline, quinolones, 'sulfa' drugs, NSAID and other analgesics, thiazides and other diuretics, anticonvulsants, allopurinol, vaccines, thiouracil, anticoagulants such as warfarin and coumarin, cocaine and other illicit drugs
• Connective tissue disorders such as lupus erythematosus and rheumatoid arthritis.
• Abnormal proteins, either in type or quantity eg cyroglobulins, hypergammaglobulinaemia, hypocomplementaemia
• Inflammatory bowel disease
• Haematological, lymphoma (cancer of the lymph glands) and other malignancies
• Physical factors eg exercise-induced vasculitis
  
  The mechanism of vasculitis is uncertain but is thought to occur through the following:
• Immune complex deposition
  In many vasculitis syndromes a pathogenic role for vascular deposition of immune complexes is suspected. Consequently, many vasculitis syndromes may be related to the prototypical immune complex disease, Serum Sickness, and thus a form of Type III Hypersensitivity. In some cases the complexed antigen is thought to be of exogenous or microbial origin whereas in others it is suspected to be derived from endogenous antigens.
• Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)
  ANCAs are auto-antibodies which react with antigens in the cytoplasmic region of neutrophils. Their presence is associated with several vasculitis syndromes although their precise role in pathogenesis is poorly understood. Furthermore, titers of ANCAs tend to rise and fall with disease activity and so are useful as diagnostic tools for management of these conditions. ANCAs are classified according to their differential specificity to neutrophilic antigens:
  • c-ANCAs stain neutrophils in a cytoplasmic pattern and are observed in Wegener Granulomatosis
  • p-ANCAs stain neutrophils in a perinuclear pattern and are observed in Microscopic Polyangiitis and Churg-Strauss Syndrome
    
    Symptoms
• Purpura (haemorrhagic spots or patches) which is slightly raised. Purpura does not disappear when pessed.
• Blisters, painful nodules (swellings) and ulcers may occur in more severe cases.
• Urticarial vasculitis which is different from ordinary urticaria in that it lasts more than 24 hours and heals with a dark stain.
• Livedo reticularis which is a mottled or net-like bluish or purplish discolouration of the skin. It may be a sign of polyarteritis nodosa which may affect the skin alone (cutaneous polyarteritis nodosa) or the skin and internal organs (systemic polyarteritis nodosa).
• Henoch-Schonlein purpura (HSP) is a type of vasculitis caused by the deposition of IgA in the small blood vessels. It characteristically presents with a triad of vasculitic rash, joint and abdominal pains (sometimes accompanied by constipation or diarrhoea). In some cases it may affect the kidneys, causing haematuria (blood in the urine) and potentially, kidney damage. The symptoms are often preceded by fever. HSP is more common in children but adults may also be affected. It may represent a hypersensitivity reaction caused by streptococcal throat infections. HSP is also known as leucocytoclastic or anaphylactoid purpura.
  
  Who gets HSP?

  
  

  Vasculitis. Click on image for larger view

  Complications
  Complications are usually caused by vasculitis affecting internal organs, for example,
  

• Systemic polyarteritis nodosa may cause fever, joint and muscle pains, nausea, vomiting, abdominal pain, heart failure, high blood pressure, kidney problems, strokes and nerve damage.
• Wegener's granulomatosis may also affect the larger blood vessels, mouth, ear nose and throat (ulcers and destruction of the nasal cartilage leading to a saddle nose deformity), lungs (coughing of blood and breathlessness), eye, kidney and nerve inflammation.
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  Diagnosis

• Skin biopsy - Histology showing inflammatory infiltrate in wall of dermal or subcutaneous vessels; may be neutrophilic, lymphocytic or granulomatous, often red blood cell extravasation, variable fibrinoid necrosis of vessel walls; DIF showing Immunoglobulins, complement or fibrin in the vessel wall.
• Conduct blood, urine and other tests to exclude involvement of internal organs.
  
  Treatment
• Treat with systemic steroids, dapsone, colchicine and immunosuppressive drugs.
• Prescribe non-steroidal anti-inflammatory drugs (NSAIDs) for pain relief.
• Treat the underlying cause, if any.

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Vitiligo or leucoderma occurs when the skin pigment, melanin is not produced. It affects about 1% of the population and is a serious cosmetic problem in dark-skinned individuals. A familial tendency has been observed.

Cause

• Autoimmune disease (self-allergy) in which the body's defence system mistakenly attacks the melanocytes (melanin-producing cells) as though they were foreign.

  Symptoms

• Well-defined milky white patches.
• Occurs most commonly on the face, neck, hands, elbows, knees, the body folds such as the armpits and groin, nipples, genital areas, around orifices (mouth, eyes, anus, umbilicus) and on areas of trauma (eg., along scratch lines).
• May have evidence or a history of other autoimmune diseases such as premature greying of the hair (see canities), alopecia areata, diabetes, pernicious anaemia, Addison's disease and thyroid disease.
  

  Vitiligo. Click on image for larger view

• Complications
• Very occasionally, vitiligo may spread to involve very extensive areas of skin.

  What you can do

• You should consult a doctor.
• Use cosmetic camouflage.

  What the doctor may do

• Exclude other autoimmune diseases or check for antibodies to thyroglobulin and parietal cells (which are increased in autoimmune disorders).
• Prescribe topical steroids.
• Treat with topical or oral PUVA - natural or artificial sunlight in association with psoralens (a photosensitising medicine that may be applied to the skin or taken by mouth).
• Minigrafting which involves removing circular areas of depigmented skin with a biopsy punch and replacing them with grafts taken with the same instrument from other sites such as the buttocks.
• Bleach the remaining skin to a uniform white colour if vitiligo is very extensive.
  

  Keypoints

• The treatment of vitiligo is not very satisfactory.
• Repigmentation is slow and may not be complete, especially over the fingers and bony prominences.
• Repigmentation is unlikely if the area involved has no hair follicles or the hairs there are white.

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