[Dermatitis herpetiformis].
Dermatitis herpetiformis is a subepidermal autoimmune acquired blistering skin disorder that is associated with intestinal gluten sensitivity. The multifactorial pathogenesis associated genetic background with Major histocompatibility complex II molecules (HLA-DQ2 and HLA-DQ8), its absence has a great negative predictive value and environmental factor with gluten consumption. There is also an increased incidence of autoimmune disorders. Skin lesions are small vesicles and/or bullae, with pruritus, located at the extensor surface of the elbows, proximal forearms and the buttocks. Gastrointestinal manifestations are rare. The biopsies from uninvolved skin establish the diagnostic with a deposition of IgA in the dermal papillae for direct immunofluorescence and in a representative lesion classically neutrophil micro-abcesses within the papillary dermis. The differential diagnosis is clinical with other causes of pruritus and histological with the linear IgA bullous dermatosis. Usually the treatment begin with dapsone for symptomatic skin lesions, it can be considered like a therapeutic test. A gluten-free diet is the treatment of choice and reduce the risk of lymphoma, the major complication.
Lioger
Bertrand
B
CHRU de Tours, service de dermatologie, 37044 Tours cedex, France. Lioger.bertrand@wanadoo.fr
Machet
Marie-Christine
MC
Machet
Laurent
L
D001706:Biopsy; D015984:Causality; D003131:Combined Modality Therapy; D003622:Dapsone / Q000494:pharmacology / Q000627:therapeutic use; D003874:Dermatitis Herpetiformis / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D003937:Diagnosis, Differential; D055050:Diet, Gluten-Free; D019085:Fluorescent Antibody Technique, Direct; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005983:Glutens / Q000009:adverse effects; D006683:HLA-DQ Antigens / Q000276:immunology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis / Q000276:immunology; D016393:Lymphoma, B-Cell / Q000209:etiology; D016399:Lymphoma, T-Cell / Q000209:etiology
D006683:HLA-DQ Antigens; C053186:HLA-DQ2 antigen; C109756:HLA-DQ8 antigen; D007070:Immunoglobulin A; D005983:Glutens; D003622:Dapsone