A case of multisystem Langerhans cell histiocytosis with risk organ (liver) and special site (craniofacial) involvement

Abstract

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of immature dendritic cells that expresses an immunophenotype positive for S100 protein, CD1a and langerin (CD207) and contains cytoplasmic Birbeck granules. We present a case of multisystem LCH with risk organ (liver) and special site (craniofacial) involvement in a 4-year-old boy who presented with hypopigmented papulopustular lesions with scales over his scalp, face and trunk, along with polydipsia, polyuria and pain in the abdomen. Histopathology reports showed diffuse infiltration of large cells with eccentric grooved coffee bean nuclei with epidermotropism and admixed with numerous eosinophils. The patient was started on desmopressin, prednisolone and chemotherapy with vinblastine. Our goal is to expand awareness regarding the entity through this report.

Keywords

• diabetes insipidus
• exophthalmos
• hand–schuller–christian
• histiocytosis
• langerhans cell