Langerhans cell histiocytosis

Langerhans cell histiocytosis(LCH) appears to arise from Langerhans cell and comprises a spectrum of clinical disease previously described in the literature by a variety of eponyms including histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe syndrome. This rare disorder occurs in all groups, predominently affecting children & young adults. LCH has a wide spectrum of clinical features. The differentiation of several forms of this disease is primarily a clinical and not a histologic one. The radiographic characteristics include the appearance of solitary "intraosseous" lesions, the multiplicity of "alveolar bone" lesions, the bone lesions, periosteal new bone formation, and slight root resorption. Prognosis of a single bone lesion, is known to be excellent. In contrast, disseminated disease has seen associated with a chronic course, a high rate of morbidity and late consequences, and possible mortality. Treatment of LCH remains problematic. Treatment of multisystem disease, where organ function is being compromised has generally been with high-dose systemic corticosteroids or multiple chemotherapy.