Electronmicroscopic Study of Familial Benign Chronic Pemphigus / 대한피부과학회지

ABSTRACT

Familial benign chronic pemphigua is a rare disease characterized by a localized recurrent eruption of grouped vesicles and exudative lesions involving the neck, groin, axillae and intertriginous areas. There are no systemic manifestations but chronicity and pruritic lesions creates a significant social and psychological handicap to those afflicted. We presented a case of familial benign chronic pemphigus in a 39 years old man He has had recurrent vesicles, fissures and crust formation in inguinal area for 10 years. Light microscopic findings showed suprabasal vesiculation with acantholysis and villi formation. And electron-microscopic findings showed as follows Bizzare microvilli changes with widened intercellular spaces, followed by loss of desmosome and complete acantholysis. Tonofilament-desmosome complex is disrupted. Many of acantholytic cells are associated with abnormal keratinization which is repreaented by perinuclear thickened bundles of tonofilament arranged in whorls or lumps. Authors diagnosed with clinical symptoms, light and electron-microscopic findings as Hailey-Hailey disease. The lesions were improved by systemic antibiotics and steroid with cold soaking.