Langerhans Cell Histiocytosis: Letterer - Siwe Disease Interest of Dermatological Signs for Diagnosis

Langerhans cell histiocytosis (LCH) is a rare disorder that involves the clonal proliferation of the Langerhans cells. LCH is often diagnosed in childhood, but any age group can be affected, from infancy through adulthood [1]. The cause of this disease is unknown, although many possibilities have been explored. The clinical presentation is highly variable. The severity and prognosis depend on the type and extent of organ involvement [3]. The disease classically presents with three syndromes namely Eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease [4]. Letterer-Siwe disease is the most common and serious of these entities, affecting mainly infants up to two years of age [5]. We present a case of this rare disease, diagnosed after dermatological examination, highlighting its typical aspects.