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

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-Schüller-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.
A 41-year-old woman was admitted to the Department of Dermatology, Venereology and Allergology in Gdansk due to diffuse erythematous-edematous skin lesions and numerous nodules localized on the face (Figure 1). The physical examination revealed also 2 × 2 cm sized ulcer covered with necrotic tissue on the skin of the left lower extremity and generalized lymphadenopathy. The lesions have been present for 2 years and were initially diagnosed as rosacea. Because of suspicion of discoid lupus erythematosus the patient was also treated with chloroquine phosphate, with no improvement. The histopathological and immunohistochemical examination of the skin did not confirm the diagnosis. During the hospitalization in the Department of Dermatology, the diagnostic tests (magnetic resonance imaging (MRI) of the craniofacial area, computed tomography (CT) of the chest, bone marrow biopsy, chest X-ray, immunophenotypic examination of peripheral blood, ultrasonography of lymph nodes) were performed, but they did not reveal any abnormalities. On the basis of histopathological examination of the skin, mycosis fungoides was initially diagnosed. Treatment with methotrexate at the dose of 25mg per week (4 courses) was ineffective (Figure 2). Cervical lymph node...


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