Juvenile Xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytosis, often affecting infants and younger children. JXG usually presents with 1 or more erythematous or yellowish nodules that are most commonly located on the head or neck. On the contrary, we present a rare case of juvenile xanthogranuloma which presented as a large swelling in the gluteal region-a location that has been rarely described in literature. The baby underwent excision of gluteal swelling. Histopathology demonstrated a lymphohistiocytic lesion characterized by proliferation of mononuclear medium-sized histiocytes along with admixed multinucleated giant cells, variably lipidized xanthomatous and Touton-type giant cells. Immunohistochemistry showed immunopositivity for CD68 (Diffuse) and CD4 (Diffuse). Cutaneous JXGs are usually self-limiting and do not require treatment, unless large and causing functional impairment as seen in the case described. Systemic cases may even warrant chemotherapy.
Case Report
History-A 1-month-old child was brought with insidious onset right gluteal swelling over one month. The swelling was non-tender, with reddish discoloration of the overlying skin.