Dermatitis herpetiformis

Dermatitis herpetiformis is a rare disease of younger men. Because of the extremely chronic course and subjective problems significantly reduces quality of life. The skin is red, covered with bubbles and nodules, changes are usually distributed symmetrically on the arms, legs, upper back and the sacral region. The patient complains of severe itching or burning sensation. Sometimes it engages mucous membranes of the oral cavity. Is present sensitivity to gluten (enteropathy) in nearly all patients (similar to celiac disease in children). The treatment is administered a local, symptomatic, antiinflammatory therapy in order to reduce itching (hydrocortisone). Gluten-free diet (based on rice and corn).

Dermatitis herpetiformis Duhring (DHD) is a pruritic, chronic, papillae lovezikulozno (bullous) disease which is associated with asymptomatic (or symptomatic) of gluten sensitive enteropathy (GSE) and granular IgA deposits in the dermal papilla tops. And if has passed than a hundred years since Louis Duhring in 1884 first described the disease, our knowledge of the etiopathogenesis of the disease is still incomplete. In patients with ADHD in a high percentage (85-90%) there is a community with HLA-DR3 and HLA-haplotype DQw2 which probably is involved in the pathogenesis of the disease. Genetic predisposition for sensitivity to gluten may manifested as an isolated disease of the small intestine (coelia) or a skin disease associated with gluten sensitive enteropathy (GSE). The genetic expression of the disease can vary. In patients with DHD most of circulating autoantibodies region of IgA class. IgA antibodies are directed to target antigens, such as the: tissue transgiutaminaze (main -autoantigen endomizijum smooth muscle), gliadin and reticulin. Anti-reticular antibodies react with endomizij smooth muscle and connective tissue under mukozejejunuma. And if the mechanism by which gluten and lead to the formation of deposits in the skin is still unclear, it is assumed that there is stockpiling of circulating immune complexes (gluten + IgA + C3, C5, CSA) in sublaminadensa zone in the dermal papilla peaks which represents the trigger for creation of inflammatory answer stockpiling of neutrophils and create cleft (vesicle, bull).

Clinical picture:

The disease can begin at any age group, but usually occurs between 2 and 4 decades. Men get sick twice as often as women. The disease is intense pruritus; pruritus precedes changes permanent accompanying symptom of the disease. The predilection of the elbows, knees, glutes, limit hair and skin of the forehead, neck and shoulders. Changes are localized symmetrically composed of papules and small, tense vesicles (bula) herpes schedule on erythematous base. Because of the intense pruritus changes are Eksk-Risan, sometimes find it difficult vesicles. Mucous membranes are not affected by the. Gluten sensitive enteropathy associated with DHD may be clinically manifest or asymptomatic. DHD is a chronic disease which are characterized remissions and exacerbations; that they last longer, the DHD is a lower intensity, and 10-30% of patients entering in complete remission. If complications occur, most commonly the result of side effects of therapy (dapsone: mild hemolytic anemia, agranulcitoza, platelet-pence; sulfapyridine: crystallization in the kidneys). Patients with gluten sensitive enteropathy have an increased tendency to develop gastrointestinal tract lymphoma. In some patients the DHD may be associated with atrophy of the stomach and hipoaciditetom, a certain number of patients showing clinical and serological signs of thyroid disease.

Diagnosis and differential diagnosis:

Diagnosis is based on clinical features, histological and immunofluorescence finds. The disease must be differentiated from others, bullous auto-immune diseases. Next to auto immune bullous disease should be taken into consideration and scabies, eczema, prurigo and papular urticaria.

Treatment:

The drug of choice for the treatment of DHD e diaminodifenilsulfon (dapsone). For patients who can not be treated dapsone may be applied sulfapyridine. Dapsone at a dosage of 50-100 mg / day or sulfapyridine in the dose of 2-3 g / day lead to rapid resolution of changes in the skin, but have no effect on the improvement of the pathological finds in the small intestine. Before the therapy introduced dapsone is necessary to determine the level of glucose-6-phosphate dehydrogenase (GPD) in erythrocytes because people with reduced values of GPD at risk to develop severe hemolytic anemia and methemoglobinemia.