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Langerhans Cell Histiocytosis: Treatment Failure With Imatinib

Christina Wagner, MD; Henrik Mohme, MD; Tanja Krömer-Olbrisch, MD; Rudolf Stadler, MD; Sergij Goerdt, MD; Hjalmar Kurzen, MD
Arch Dermatol. 2009;145(8):949-950. doi:10.1001/archdermatol.2009.164.
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Histiocytoses are a heterogeneous group of idiopathic diseases with a localized or disseminated accumulation of histiocytic cells and are classified into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (N-LCH). Lesional cells in LCH are characterized by dendritic cell markers such as S-100 as well as Langerhans cell–specific features such as Birbeck granules and expression of CD1a, langerin, and fascin. Langerhans cell histiocytosis generally occurs during childhood, but an increasing incidence in adults has been reported.1 Multiorgan LCH is associated with a considerable mortality, and so new treatment options beyond established chemotherapy protocols are needed. Herein, we report 2 cases of LCH that did not respond to imatinib, a kinase inhibitor recently used to treat LCH2 and N-LCH.3

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Figure 1.

Case 1. Before treatment, fluid-filled, macerating erosions and papules were present in the intertriginous area.

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Figure 2.

Case 2. Before treatment, symmetrical, scaly, erythematous papules were present on the abdominal area.

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