Darier-White disease (DW), otherwise known as keratosis follicularis, is a rare genodermatosis with autosomal dominant inheritance, characterized by loss of adhesion between epidermal cells and abnormal keratinization. The distinctives lesions of Darier's disease include rough papules in seborrhoeic areas, palmoplantar pits, mucosal involvement, and nail changes. DW disease can be occasionally associated with bacterial complications, but rarely with viral ones. Kaposi's varicelliform eruption (KVE) is a secondary herpes simplex virus infection that affects patients in the setting of primary dermatologic conditions. KVE, frequently misdiagnosed as impetigo can be severe, progressing to disseminated infections and potentially life threatening. It occurs with a variety of skin disorders, although association with Darier's disease has rarely been reported in the literature. This report describes a case of KVE in a patient suffering from DW disease, focusing on its clinical course. A review of the literature on Kaposi's varicelliform eruption including disease associations, pathogenesis, and treatment has been also reported.
Kaposi-Juliusberg varicelliform eruption in patient suffering from Darier-White disease: a case report and review of the literature / Molinelli, E; Ricotti, F; Campanati, Anna; Cataldi, I; Ganzetti, G; Liberati, G; Bianchelli, T; Offidani, Annamaria. - In: GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA. - ISSN 0392-0488. - ELETTRONICO. - (2016).
Kaposi-Juliusberg varicelliform eruption in patient suffering from Darier-White disease: a case report and review of the literature
Molinelli, E;CAMPANATI, Anna;OFFIDANI, ANNAMARIA
2016-01-01
Abstract
Darier-White disease (DW), otherwise known as keratosis follicularis, is a rare genodermatosis with autosomal dominant inheritance, characterized by loss of adhesion between epidermal cells and abnormal keratinization. The distinctives lesions of Darier's disease include rough papules in seborrhoeic areas, palmoplantar pits, mucosal involvement, and nail changes. DW disease can be occasionally associated with bacterial complications, but rarely with viral ones. Kaposi's varicelliform eruption (KVE) is a secondary herpes simplex virus infection that affects patients in the setting of primary dermatologic conditions. KVE, frequently misdiagnosed as impetigo can be severe, progressing to disseminated infections and potentially life threatening. It occurs with a variety of skin disorders, although association with Darier's disease has rarely been reported in the literature. This report describes a case of KVE in a patient suffering from DW disease, focusing on its clinical course. A review of the literature on Kaposi's varicelliform eruption including disease associations, pathogenesis, and treatment has been also reported.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.