Epidermolysis bullosa acquisita and inflammatory bowel disease.
Immunologic studies on lesional and nonlesional "normal" -appearing skin of a 32-year-old man with epidermolysis bullosa acquisita (EBA) revealed the following: (1) Noninflammatory subepidermal bullae formation in the periodic acid-Schiff (PAS)-positive basement membrane; (2) linear deposits  of polyclonal IgG, Clq, C4, C3, properdin, and factor B at the dermoepidermal junction; IgA and IgM were not found; (3) broad subbasal lamina electron-dense amorphous material (EDAM) lacking amyloid features by electron microscopy; EDAM was not associated with anchoring fibers; (4) cleavage plane below the EDAM. Gastrointestinal endoscopy showed inflammatory erosions in the duodenum. Microscopy of the involved duodenum showed a mixed inflammatory infiltrate, subepithelial edema, microvesiculation, and granular deposits of IgG and C3, but no EDAM. However, EDAM and immune reactants were found in the esophagus. EBA is a distinct entity characterized by the presence and location of EDAM ultrastructurally, and it may represent a gastrointestinal-cutaneous syndrome with an immune pathogenesis.
D000328:Adult; D003165:Complement System Proteins / Q000032:analysis; D003424:Crohn Disease / Q000150:complications* / Q000276:immunology / Q000473:pathology; D004386:Duodenum / Q000648:ultrastructure; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000276:immunology / Q000473:pathology; D004947:Esophagus / Q000648:ultrastructure; D005455:Fluorescent Antibody Technique; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008297:Male; D012867:Skin / Q000648:ultrastructure
D007136:Immunoglobulins; D003165:Complement System Proteins