Skin-limited Langerhans cell histiocytosis in an adult

Langerhans cell histiocytosis (LCH) is an idiopathic group of disorders characterized histologically by proliferation and infiltration of tissue by Langerhans cell-type histiocytes. This disease can affect various organs. Patients with a single system lesion should be followed carefully. Detection of somatic BRAF-V600E mutation in circulating blood cells or in lesional biopsies has been associated with high-risk clinical characteristics. A 38-year-old male presented to our Dermatology Centre with a 3-month history of a small nodule on his right leg skin. Surgery to remove the lesion was performed. The diagnosis of skin-limited LCH was established. Due to possible systemic spread, the patient was referred to a hematologist for further evaluation. A full-body computed tomography (CT) scan did not show any infiltrates in other organs. Bone marrow aspirate and biopsy were performed; no Langerhans cells were detected. Sometimes skin lesions may represent the most clinically evident manifestation of potentially life-threatening multisystem disease.