Langerhans cell histocytosis--a clinicopathological spectrum

Langerhans cell Histiocytosis--a lesion characterised by proliferation of a variant of epidermal dendritic cell is one of the several syndromes in which histiocytes play a principal role in pathogenesis outside the context of inflammatory and metabolic storage disease. Seven cases were diagnosed as Langerhans cell Histiocytosis over a period of six years. The mode of diagnosis was Fine needle aspiration in 2 cases, open biopsy in four, and both biopsy and FNAC in one. The age range was from 2 months to 58 years and four of the seven patients were males. The lesion was unifocal in 3 cases (two in bone and one involving skin and soft tissue), multifocal in 3 and systemic in one. Electron microscopy was done in one case to demonstrate Birbeck granules. Immunohistochemical demonstration of S 100 protein was done in one case. The clinicopathologic spectrum of Langerhans cell Histiocytosis is brought out in this presentation of seven cases.