Disseminated Langerhans cell histiocytosis in a 51-year-old man with cutaneous involvement and multiple endocrinopathies

Langerhans cell histiocytosis (LCH) is a rare disorder arising from the abnormal proliferation and subsequent deposition of CD1a+ myeloid dendritic cells in various organs. Clinical features are heterogeneous and primarily depend on specific organ systems involved, ranging from skin rash and osteolytic skull lesions to treatment-refractory multiorgan failure in severe cases. Although LCH predominantly affects the pediatric population, an increasing number of adult-onset cases are being reported.1, 2 We describe the case of a 51-year-old man with disseminated (multisystem) LCH stemming from an in-frame, oncogenic BRAF mutation.