TY - CHAP M1 - Book, Section TI - Chapter 147. Cutaneous Langerhans Cell Histiocytosis A1 - Gelmetti, Carlo A2 - Goldsmith, Lowell A. A2 - Katz, Stephen I. A2 - Gilchrest, Barbara A. A2 - Paller, Amy S. A2 - Leffell, David J. A2 - Wolff, Klaus Y1 - 2012 N1 - T2 - Fitzpatrick's Dermatology in General Medicine, 8e AB - |PrintLangerhans Cell Histiocytosis at a GlanceA group of rare disorders that are considered reactive but have a broad spectrum of severity.The reactive vs. neoplastic nature of the disease is debated. A clonal origin for the cells of Langerhans cell histiocytosis (LCH) in genetically predisposed patients is the most probable hypothesis.Histopathologic features consist of a dense infiltrate of histiocytes with a strong epidermotropism. The unifying element is the typical “LCH cell”—a histiocyte with an irregular vesiculated, often reniform nucleus and an abundant, slightly eosinophilic cytoplasm. These cells test are positive for S100 protein, CD1a, and CD207, and contain cytoplasmic Langerhans granules.Cutaneous lesions vary from papules to vesicles, pustules, nodules, and ulcers.The course of LCH ranges from localized self-healing forms to generalized and fatal cases.The most common sites of involvement are the head, trunk, and skin folds.Mucosal lesions are usually ulcerated nodules involving mainly gingival and genital regions.Associated manifestations include diabetes insipidus and exophthalmos.Systemic lesions may affect bones, lungs, bone marrow, liver, spleen, and lymph nodes.Aggressive forms may be treated successfully with vinblastine and other drugs. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=56072702 ER -