fibrin and Pemphigoid--Benign-Mucous-Membrane

The first case of an infant affected with a rare, disseminated variant of benign cicatricial pemphigoid is described, showing the same ultrastructural features of initial blister formation as an adult patient. These consist in edematous changes within the superficial dermis caused by vesiculation or dissolution of cellular and noncellular connective tissue elements, coalescing into subepidermal blisters. Differential diagnosis excludes other nonhereditary bullous disorders because of the ultrastructure of the dermo-epidermal junction in nascent blisters and in perilesional skin. In spite of evident clinical, histological, and immunohistological similarities as well as controversial and confusing immunological studies, acquired epidermolysis bullosa can be clearly separated from our case by a diagnostic hallmark on the electron-microscopical level, i.e., band-like IgG depositions beneath the basal lamina. This is demonstrated in comparing the two cases of disseminated cicatricial pemphigoid with three patients suffering from acquired epidermolysis bullosa, thus providing evidence that disseminated cicatricial pemphigoid and acquired epidermolysis bullosa are two distinct nosologic entities.

Topics: Adult; Child, Preschool; Connective Tissue; Diagnosis, Differential; Epidermolysis Bullosa; Female; Fibrin; Humans; Immunoglobulin G; Immunohistochemistry; Male; Microscopy, Electron; Middle Aged; Pemphigoid, Benign Mucous Membrane; Skin; Skin Diseases, Vesiculobullous

Twenty-seven patients (19 females and 8 males) with a clinically diagnosed desquamative gingivitis were used in this study. Twenty-seven additional patients with oral and skin diseases and with gingival involvement other than that of the desquamative gingivitis type served as controls. Biopsy gingival specimens were sectioned and stained with H and E. The rest of the tissue specimen was cut with a cryostat and used for immunofluorescent assays of the tissue-bound antibodies (IgG, IgA, IgM, C3 anf fibrin). Circulating antibodies were determined by using the indirect immunofluorescence method in serum samples. Normal human oral mucosa and several animal epithelial tissues were used as substrates. The immunopathologic findings suggest the diagnosis of lichen planus in the presence of linear or granular fibrin deposition at the basement membrane zone/mucosal submucosal interface with or without cytoid bodies and cicatricial pemphigoid, in the presence of circulating and/or tissue-bound immunoglobulins and C3 in a linear continuous pattern along the BMZ. It is suggested that an accurate diagnosis of the underlying disease of the desquamative gingivitis can be made on the bases of the clinical, histopathologic, immunopathologic and follow-up findings.

Topics: Adult; Aged; Complement C3; Female; Fibrin; Fluorescent Antibody Technique; Gingival Diseases; Gingivitis; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Lichen Planus; Male; Middle Aged; Pemphigoid, Benign Mucous Membrane