Congenital lamellar ichthyosis complicated with erosive pseudo pustulosis: A rare clincal entity. About a case and review of the literature

Abstract

Lamellar ichthyosis is a rare congenital disorder that can be encountered by plastic surgeon in a daily practice. Its clinical diagnosis makes it an significant pathology to identify and to know how to treat. We report the case of a patient suffering from lamellar ichthyosis complicated by erosive pseudo pustulosis of the scalp. Our treatment protocol with two intra-lesional delayed-corticoids (Kenacort ®) injections three months apart showed significant clinical improvement of the lesions. Congenital lamellar ichthyosis regroups various clinical presentations. Most of the therapeutic strategies described in the literature involve local and systemic treatments, weighing on patients and leading to modest results. Surgical treatment or hyaluronic injections have also been reported but they raise problematics regarding morbidity and efficiency. Our therapeutic strategy by two Kenacort ® injections three months apart is simple, reproductible and has shown efficiency in the treatment of our patient suffering from congenital lamellar ichthyosis complicated with erosive pseudo pustulosis of the scalp.