ANTI-EPILIGRIN CICATRICIAL PEMPHIGOID - A CASE ASSOCIATED WITH GASTRIC-CARCINOMA AND FEATURES RESEMBLING EPIDERMOLYSIS-BULLOSA ACQUISITA

A 48-year-old woman with anti-epiligrin cicatricial pemphigoid (CP) wh o showed clinical features resembling epidermolysis bullosa acquisita was found to have adenocarcinoma of the stomach. Histological examinat ion of lesional skin demonstrated a subepidermal blister. Direct immun ofluorescence microscopy of perilesional skin revealed linear deposits of IgG and C3 at the basement membrane zone. The patient's serum cont ained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl -split normal human skin as determined by indirect immunofluorescence microscopy, and the lamina lucida as determined by indirect immunoelec tron microscopy. The patient's serum immunoprecipitated laminin-5 from extracts and media of biosynthetically radiorabelled human keratinocy tes, Immunoblot studies showed that the patient's autoantibodies speci fically bound the alpha 3 subunit of this laminin isoform. Fragility o f the skin and bullous lesions disappeared after total gastrectomy, bu t soon reappeared possibly in association with metastatic disease in a lymph node. The possibility that anti-epiligrin CP may develop parane oplastically in some patients is discussed.