Dermatitis herpetiformis is a rare blistering disorder associated with gluten-sensitive enteropathy (coeliac disease) and occasionally other organ-specific autoimmune disorders. The HLA associations (B8, DR3, DQ2 in 80-90% of cases) and immunological findings (endomysial, tissue transglutaminase, reticulin and gliadin autoantibodies may be present in serum) are similar to coeliac disease.
Skin biopsy shows a subepidermal blister with neutrophil microabscesses in the dermal papillae. Direct IMF studies of uninvolved skin show IgA in the dermal papillae and patchy granular IgA along the basement membrane. The jejunal mucosa may show partial villous atrophy but the changes tend to be milder than in coeliac disease.
Dermatitis herpetiformis is commoner in males and can present at any age but is most likely to appear for the first time in young adult life. It presents with small, intensely itchy blisters of the skin. The lesions have a predilection for the elbows, extensor forearms, scalp and buttocks. The tops of the blisters are usually scratched off; thus crusted erosions are often seen at presentation. Remissions and exacerbations are common.
In Dermatitis herpetiformis, changes occur primarily in the dermis. They consist of vascular dilatation, oedema nad cellular infilteration with a predominance of eosinophils and lymphocytes. The bullae are invariably subepidermal with rounded lateral borders. There is deposit of IgA at the epidermo-dermal junction.
There are good prospects of longevity. But the condition is chronic, marked by a fluctuating course. SOme cases clear up rapidly on the removal of the septic focus; others continue for years till the disease wears out or terminate in pemphigus.
This should always be with a gluten-free diet (GFD). Control of the skin disease can be obtained with oral dapsone (50-200 mg daily) or sulphonamides. If a strict GFD is adhered to, oral medication can often be withdrawn after 2 years. The GFD will need to be lifelong. It protects against the rare complication of small bowel lymphoma.
Use of dapsone. Dapsone frequently causes a mild dose-related haemolytic anaemia (which is usually well tolerated) but the haemolysis can be devastating if there is G6PD deficiency. Liver damage, polyneuropathy and aplastic anaemia can also occur rarely, so regular monitoring of a blood count and liver biochemistry is needed.
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