Association of juvenile xanthogranuloma with café-au-lait macules.

A 2-year-old boy was referred to the dermatology services for the evaluation of yellowish-brown raised lesions over the face of 3 months' duration. In addition, he had multiple asymptomatic hyperpigmented spots over the trunk, which his parents had noticed at the time of birth. His physical and mental milestones of development were normal. Apart from the cutaneous lesions, the child was otherwise well and there was no history of seizures. He was the only child born to nonconsanguineous parents. No family history of similar lesions was available. The boy weighed 14 kg, had a height of 88 cm, and a head circumference of 48 cm. General physical and systemic examination was normal. Cutaneous examination revealed multiple, yellowish-brown papules over the forehead, sides of the face, and shoulders. The papules measured 0.5-0.8 cm in diameter, were discrete, well defined, oval and flat topped without any surface changes (Fig. 1). Multiple café-au-lait macules varying from 0.5 to 3.0 cm in diameter were present over the trunk (Fig. 2). There was no freckling in the axillary or the inguinal regions and no neurofibromas were present. A diagnosis of juvenile xanthogranuloma was considered. Complete blood counts, urine analysis, hepatic and renal function tests, and serum lipid levels were normal. A skin biopsy from a papule over the shoulder revealed a dense infiltrate in the dermis underlying a normal epidermis. The infiltrate was well demarcated and comprised lymphocytes, eosinophils, and foamy histiocytes along with Touton giant cells and proliferating fibroblasts. This confirmed the diagnosis of juvenile xanthogranuloma. The parents were informed about the benign and self-limiting nature of the disorder and were advised to bring the child for regular follow-up.