The Loose Cannon! - Cutaneous Manifestation of Langerhans Cell Histiocytosis

A 6-year-old girl presented with symptoms of diabetes insipidus with no neurological abnormalities. Presence of erythematous, dried, plaque like lesions on her scalp had guided the clinicians/paediatricians to liase with different sub-specialities which included dermatologists, pathologists, haematologists, endocrinologists, oncologists, and radiologists. The diagnosis of Langerhans cell histiocytosis (LCH) was rendered and confirmed by positive immunostaining for anti CD1a and/or anti S-100 protein antibodies. Other work up were done for the patient to detect any organs involved and either LCH infiltrates the liver, lungs, spleen or hematopoietic system. The patient was then treated with chemotherapy as according to LCH-IV protocol group 1 initial treatment for multisystem Langerhans Cell Histiocytosis. She responded very well to the therapy and is still under follow up. In this case, we will also be discussing about the importance of genetics of Langerhans Cell Histiocytosis.