Dermatology - Dermatitis Herpetiformis

Dermatology - Dermatitis Herpetiformis
Dermatitis herpetiformis is a long-lasting, recurring, highly itchy skin rash that often begins between the ages of 20 and 60. Males are afflicted with the condition at a rate that is twice as high as females.
The individual experiences severe and intermittent pruritus, characterized by a sensation of burning or stinging on the skin. In rare cases, pruritus may be completely absent. Symptoms typically occur before the emergence of skin lesions within a timeframe of 8 to 12 hours. The consumption of iodides and excessive intake of gluten are contributing factors that worsen the condition. Approximately 10-20% of patients exhibit laboratory findings indicating malabsorption in the small intestine. Typically, there are no systemic signs present.
Abnormalities
The lesions are characterized by red raised spots or flat patches, small solid vesicles, sometimes containing blood, and occasionally large blisters. The lesions are organized in clusters, which is why they are called herpertiformis, and their distribution is remarkably symmetrical. Scratching leads to the formation of excoriations and crusts. Postinflammatory hyperpigmentation and hypopigmentation develop in the locations where lesions have healed
The presence of clustered papulovesicles in specific areas, along with intense itching, strongly indicates the condition. In most cases, a biopsy of the initial lesions is sufficient for diagnosis. The presence of IgA deposits in the skin surrounding the lesion, as revealed by immunofluorescence, provides the most reliable confirmation. The differential diagnosis comprises several conditions such as allergic contact dermatitis, atopic dermatitis, scabies, neurotic excoriations, papular urticaria, bullous autoimmune diseases including bullous pemphigoid, and pemphigoid gestationis.
Administering Dapsone at a daily dosage of 100-150 mg, gradually reducing it to 50-25 mg, and sometimes as low as 50 mg twice a week, leads to a rapid and significant improvement, frequently observed within a few hours. Prior to initiating sulfones, it is advisable to measure the level of glucose-6-phosphate dehydrogenase. Additionally, it is recommended to assess methemoglobin levels within the first two weeks, and closely monitor blood counts during the initial months. If dapsone is not recommended due to medical reasons, sulfapyridine can be used at a dosage of 1-1.5 g per day. It is important to consume an adequate amount of fluids and closely check the presence of casts in urine as well as renal function. Following a gluten-free diet can perhaps inhibit the disease or enable a decrease in the dosage of dapsone or sulfapyridine, however the reaction is notably sluggish.