Background:Langerhans cell histiocytosis (LCH) a rare disease with various clinical presentations characterized by the accumulation of CD1a-positive immature dendritic cells. This study was done with the aim of documenting the various clinical presentations of LCH occurring in Indian population. Methods: All histopathologically proven cases of LCH were retrieved retrospectively over a period of 5 years (January 2013- December 2017) at Institute of Child Health and included in our study. Results: Out of 14 cases observed as LCH, 11 cases (78.57%) were between 0-3 years of age, with male predominance. Bone involvement was the commonest presentation seen in 10 cases (71.43%), of which skull was commonly involved in 8 cases (80%). Isolated skin involvement was seen in 3 cases (21.43%), next to bone involvement. 1 case (7.14%) presented with pure lymph node involvement. Conclusion: Lesions presenting as isolated skin involvement is not very rare which can be seen in children upto 3 years of age and in our study, it was observed in 3 cases (21.43%) which is higher than the previous observations [15,16]. Other unusual presentations like sternum, mandible (alveolus) involvement, pure lymph node involvement can also occur. Skin involvement being the second commonest of the presentations next to bone lesions and hence warrants a skin biopsy in susceptible age group (0-3 years) with chronic skin lesion and an associated skeletal survey, so that it offers the clinician a road to the diagnosis and proper treatment is initiated to the patient at the earliest. Follow up of cases is needed to rule out relapse.

Keywords: Langerhans cell histiocytosis; Various presentations; Skin involvement.