ESPE Abstracts

Introduction: Langerhans cell histiocytosis is a neoplastic disorder characterized by proliferation of myeloid dendritic cells. It can involve single system or multisystem with commonly involving sites including skin, bone, central nervous system, lung, hematopoietic system, liver and spleen. It also involves the endocrine system with diabetes insipidus a common presentation in 15-50% cases. It has a broad spectrum of presentation ranging from a relatively benign course to a fulminant progressive disease.

Case Report: We present a case of 4 years old male child presented to us with rash on scalp and multiple lumps on head for 3 months and history of polyuria for 2 months. On physical examination findings were consistent with Langerhans cell histiocytosis in the form of swellings involving frontal and temporoparietal bone which were firm and smooth with no tenderness, greasy erythematous scaly rash involving scalp, and neck region and hepatosplenomegaly. Child was investigated on which he had microcytic hypochromic anemia with atypical lymphocytes on peripheral smear and normal metabolic panel and liver function tests. Histopathological analysis of the lump biopsy showed findings consistent with Langerhans cell histiocytosis. His urine had decreased specific gravity (1.000) spot urine osmolality of 254 mOsmoles/kg and spot serum osmolality 280mOsmoles/kg. So we proceeded further with water deprivation test which was consistent with central diabetes insipidus and other endocrinological investigations including thyroid profile, serum cortisol, serum prolactin and MRI brain pituitary protocol which were all normal. His bone marrow examination was normal with no evidence of malignant cell infiltration. So the child was started on chemotherapy after which size of the swellings decreased, rash improved with overall symptomatic improvement and tab desmopressin for diabetes insipidus was started.

Discussion: Langerhans cell histiocytosis is a malignant disorder which is mainly treated with chemotherapy and management of complications. Poor prognostic indicators include presentation in age less than 2 years, multisystem disease, risk organ involvement (bone marrow, blood, liver, spleen) and unsatisfactory response to initial treatment.