European Journal of Dermatology


Darier’s disease with esophageal carcinoma Volume 10, issue 6, September 2000


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Department of Dermatology, Surugadai Nihon University Hospital, 1-8-13 Kanda-Surugadai, Chiyoda-Ku, Tokyo 101-8309, Japan.

We report a patient with longstanding Darier’s disease (DD) involving the mucous membrane of the esophagus who developed esophageal carcinoma. A number of small white plaques were observed around the carcinoma in the postoperative specimens of the esophageal mucosal epithelium. Histopathological findings of the specimens near the carcinoma showed suprabasal clefts and villi formation, features consistent with DD, with no evidence of malignancy. There was some speculation on the pathogenic relationship between esophageal carcinoma and DD in this patient, but nothing was confirmed. Immunohistochemistry of lesional skin examined with antibody against desmogleins (Dsgs) 1 + 2 showed a diffuse pattern throughout the cytoplasm in most of the suprabasal acantholytic cells, whereas some of the spherical acantholytic cells showed a ring pattern surrounding the nucleus. In electron microscopy, these areas stained with Dsgs corresponded to dispersed tonofibrils in the suprabasal acantholytic cells and perinuclear aggregation of tonofibrils in the spherical acantholytic cells. This implies that in DD patients, some affinity remains after acantholysis between Dsgs and intermediate filaments, possibly through other desmosomal components.