Kaposi Julius-berg Syndrome on Congenital Ichthyosis, about a Case

Introduction: Kaposi-Julius berg syndromeis a rare complication of infection with the virus of the 'herpes, often occurring on a pre-existing skin disease. It mainly affects children, the management of this syndrome is based on antiviral medication with antibiotic therapy. In the absence of diagnosis and early treatment, its prognosis is severe.

Observation: We present the first case of kaposi juliusberg syndrome on congenital ichthyosis described. This is a 5-year-old boy followed for congenital ichthyosis since birth, under keratolytic treatment and emollients. He was hospitalized for febrile rash made of lesions vesicular, pustular and crusty. He also presented with bilateral keratitis. The clinical examination showed a fever of 39◦C, an altered general condition with submandibular lymphadenopathy. The skin examination revealed a dozen vesicles and pustules in a bouquet on the face and scalp, meliceric crusts. antibiotic therapy with amoxicillin-clavulanic acid was instituted. The evolution was rapidly favorable. The clinical examination of the mother revealed a recurrence of oral herpes at the origin of the contagion, motivating the introduction of a preventive treatment for recurrences in her.

Conclusion: This observation shows that it is essential to inform the family and friends of patients with ichthyosis of the risk of severe herpes infection and to avoid all contact with the herpes virus as far as possible.