title: [Disseminated cicatricial pemphigoid].
abstract: 1. A case of the very rare "disseminated cicatricial pemphigoid" is presented in a 68-year-old man. Clinically, prurigo-like nodules and keloidiform plaques, tense blisters and tight atrophic scars were disseminated in the occipital region, on the trunk and on the extremities. The mucous membranes were never affected. 2. Histologically, subepidermal blisters were found. The blister roof contained the complete, intact epidermis; in the floor of the blisters, fibroplasia, angioplasia, and a scarce inflammatory infiltrate containing some eosinophils were present. 3. Direct immunofluorescence revealed linear precipitates of IgM and C3 in the basement membrane. Circulating antibodies have not been found. 4. Semithin sections and electron microscopy showed subepidermal microvesicles and fibrin deposits in initial lesion, followed by edema and blister formation within and below the fibrin deposits. 5. Among several trials of treatment, only azathioprine (Imurek) seemed effective.
mesh_terms: D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use; D001485:Basement Membrane / Q000276:immunology; D003176:Complement C3 / Q000032:analysis; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure; D012872:Skin Diseases, Vesiculobullous / Q000473:pathology*
chemical_list: D003176:Complement C3; D007075:Immunoglobulin M; D001379:Azathioprine
vernacular_title: Disseminiertes vernarbendes Pemphigoid.