[Kaposi-Juliusberg's syndrome complicating Darier's disease"]

Kaposi-Juliusberg's syndrome is a severe herpes simplex virus cutaneous infection, accompanied by general signs. Darier's disease is a rare genodermatosis characterised by keratinisation disorders.

In a 51 year-old patient suffering from Darier's disease since the age of 20, a diffuse vesicular eruption occurred following a herpes eruption on the lower lip. Culture of a vesicle revealed a type 1 Herpes simplex virus. The diagnosis of Kaposi-Juliusberg's syndrome was made.

Kaposi-Juliusberg's syndrome is a "classical" herpes complication of atopic dermatitis. It may also develop on other predisposing territories such as acantholytic dermatitis: pemphigus vulgaris, Hailey-Hailey's disease and Darier's disease. Although a rare complication and life threatening for the patient, Kaposi-Juliusberg's syndrome can now be treated efficiently with intravenous and subsequently oral aciclovir.