Multisystem Langerhans cell histiocytosis in an infant: A case report and review of literature

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease of childhood which originates from marrow-derived immature myeloid dendritic cells of skin and visceral organs with incompletely understood etiopathogenesis. An 11-month-old infant presented with fever, pallor, multiple erythematous, crusted, scaly hypopigmented macules, and shiny colored papules over scalp, forehead, and trunk along with hepatosplenomegaly. Persistent pancytopenia, punched out lesion on a brain scan, and multinucleated giant cells with eosinophilic cytoplasm admixture with eosinophils and lymphocytes on skin biopsy were seen. Immunohistochemistry was positive for CD1a and S100. The patient was treated with vinblastine and steroid, but unfortunately parents did not complete the therapy. A high index of suspicion is necessary to make timely diagnosis and therapy to minimize the frustration felt by parents/patients.