Congenital macronodular juvenile xanthogranuloma of the eyelid.

Abstract

A large congenital eyelid mass prevented an infant from elevating his eyelid successfully. A small punch biopsy was initially interpreted as "consistent with a dermatofibrosarcoma protuberans." On the basis of this diagnosis, radical surgery was advised. Dermatofibrosarcoma protuberans, however, rarely affects children and seldom appears in the head and neck area. Because of the discrepancy between clinical and pathologic diagnoses, additional biopsies were performed, which disclosed the lesion to be a juvenile xanthogranuloma. The mass regressed after intralesional injections of corticosteroids. This is the first clinicopathologic description of congenital macronodular juvenile xanthogranuloma affecting the eyelids. The variability in the histologic composition of this large lesion may lead to a mistaken diagnosis of an aggressive condition that may provoke unnecessary radical surgery.