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Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of dendritic cells, which comprises a wide range of clinical presentations. The term LCH is used to include a spectrum of disorders previously termed: histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease and Hashimoto-Pritzker disease. The etiology of the lesion is still elusive, and the question of whether LCH is a reactive or neoplastic disorder remains unanswered [1]. The LCH mainly affects children under 10 years of age, but can occur at any age. The estimated incidence in adults is about 1–2 cases per million and is lower than that in children [2]. The LCH may affect any organ, but the most common locations include bone [3–6], mucous membranes [7], skin [8], lymph nodes, lung [9], pituitary [10] and liver [11]. The clinical course and prognosis of LCH is diverse ranging from a spontaneously regressing single lesion to a life-threatening multisystem disease with rapid progression and death. This disorder may involve single or multiple organs and has an unpredictable course. Diagnosis of LCH is difficult and may be delayed due to its rarity and wide clinical manifestation.

Letter to the Editor Adult onset of multisystem Langerhans cell histiocytosis with skin and lymph node involvement