Dermatitis Herpetiformis


Dermatitis Herpetiformis
 is chronic, recurrent dermatitis, characterized by severe itching and formation of tiny vesicles, and small bullae arising on normal or reddened skin. Patient complains of an intensely itchy eruption which is wide spread and has a tendency to grouping.

Dermatitis Herpetiformis is more common in the age group of 30 to 40 years, males being affected more than females in the ratio of 2 : 1. It may occur in children. There is a high occurrence of HLA-B8 and HLA-DR3 in such patients. In some patients there is associated gluten sensitive enteropathy.

Clinical features

The primary lesion is an erythrmatous papule or tiny firm topped vesicle or bulla or urticaria like wheal. Scratching results in excoriations and crust formation. Lesions are arranged in groups and are distributed symmetrically over extensor surfaces (elbows, knees, buttocks, scapula, sacral area, scalp, and face and hair line). The eruption is polymorphic. Owing to severe itching it is uncommon to see many intact vesicles and the clinical picture usually is of excoriated and scabbed erythematous, papules, erosion, scars and pigmentation. Gluten overload worsens the condition.

Immuno fluorescence studies show IgA deposits in dermal papillary tips. In addition compliment C3 is also deposited. Circulating immune complexes are always found. Biopsy from early erythematous papule shows micro abscesses at the tip of dermal papillae with infiltration of neutrophils and eosinophils.

Mal-absorption studies show steatorrhoea and abnormal D-Xylose absorption in 30-70% of cases. In addition there is presence of anemia.

Diagnosis

It is mainly clinical. Detection of IgA deposits in normal and perilesional skin is pathognomic. Biopsy is confirmatory.

Course. The disease runs a chronic course over several years with remissions and relapses. It may become milder and finally disappear.

Treatment of dermatitis herpetiformis

Dapsone is the drug of choice. Dose is 100-200 mg daily. It is gradually reduced to 25 to 50 mg per day. Besides this in patients who do not tolerate Dapsone. Sulphapyridine in dose of 1.0 to 1.5 U daily is given with plenty of fluids. It controls itching and development of vesicles. The dose is gradually reduced as the symptoms are controlled. Regular total and differential white cell counts are essential as the drug may produce leucopoenia. Local treatment consists of application of calamine lotion. If there is secondary infection, antibiotics help but sometimes they may aggravate the condition.

Diet should be gluten free and this type of diet is known to suppress the disease.

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