Bullous pemphigoid (BP) is an autoimmune bullous disease of the elderly. Patients' auto-antibodies are directed against BP180, a transmembrane hemidesmosomal glycoprotein of basal keratinocytes. Between 1989 and 1998, we identified 115 patients with BP in the Department of Dermatology at the University of Würzburg. This is one of the largest groups of patients with BP studied to date. In all patients, direct and/or indirect immunofluorescence microscopy studies were positive. The average age (+/- standard deviation) of the patients was 75 +/- 12 years, the median was 78 years. The youngest patient was 39 years of age; the oldest, 99 years. 54% of the patients were female and 46% male. In 24% of the patients, an involvement of oral, and in 7% of genital mucous membranes was seen. 98% of the patients complained about pruritus. By direct immunofluorescence microscopy, linear deposits of C3 and/or IgG were detected at the basement membrane zone of perilesional skin biopsies. By indirect immunofluorescence microscopy using NaCl-separated human skin, 87% of cases revealed circulating serum antibodies whereas on monkey esophagus, only 72% of sera were found positive. In 85% of the patients, we detected elevated serum levels of total IgE before the initiation of treatment. In those sera that were analyzed for reactivity with BP180, autoantibodies to the immunodominant NC16 A domain of BP180 were detected in 89% by immunoblot analysis and in 93% by ELISA. Therefore, in most patients, BP can be diagnosed serologically.
An exact history was taken and clinical, endoscopic and histologic studies were performed in 14 family members of a case of Gardner's syndrome with triple symptomatology documented with biopsy and autopsy findings. Among the symptom-free probands five cases of colonic polyposis and three cases with gastric polyps were found. All polyps were histologically adenomatous. Dysplasias grade I and II were found repeatedly, in one 16-year-old adolescent there was already severe dysplasia within the gastric mucosal polyps. A warning is given against sub-classification of familial colonic polyposis and other syndromes within the definition of Gardner's syndrome. In future during diagnostic investigations for cases of Gardner's syndrome isolated gastric polyposis should be sought for as well as monosymptomatic colonic polyposis. Diagnostic procedures and treatment should depend on endoscopic and histological findings. Regular follow-up with endoscopic biopsies are to be encouraged not only in the diseased cases but also in all family members available.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.