English

Abstract

AIM: Hand-Schuller-Christian Disease is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. It represents triad of diabetes insipidus, bone lesions and exophthalmos. Usually begins at the age of 2 and 6 years. 30%- develop skin lesions and 80%- develop bone lesions (cranium being preferentially involved). MATERIAL & METHOD: A 5-year-old boy was admitted to the Kempegowda Institute of medical sciences hospital, department of orthopaedics with pain in left hip since 2 weeks. The child was small for age, cachexic, mildly lethargic but occasionally irritable. He was referred to skin OPD for seborrheic dermatitis like picture over the scalp and multiple hypopigmented macules over the abdomen. Patient also had multiple boggy defects in the skull and exophthalmos and also history of long term continous ear discharge and on and off fever. Cervical, axillary and inguinal lymphadenopathy were present. RESULT: Skin biopsy findings were consistent with histiocytosis. CD1a and S100 tumor markers were positive. Patient was put on chemotherapy-ETOPOSIDE+CYTOBLASTIN+BETAMETHASONE REGIMEN to which he responded successfully. CONCLUSION: Disorders like HAND-SCHULLER- CHRISTIAN DISEASE detected early can be effectively managed improving the overall survival, quality of life and disability free living.