Association of juvenile xanthogranuloma with cafe-au-lait macules

A 2-year-old boy was referred to the dermatology services for the evaluatio n 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 ment al 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 simi lar lesions was available. The boy weighed 14 kg, had a height of 88 cm, and a head circumference of 4 8 cm. General physical and systemic examination was normal. Cutaneous exami nation revealed multiple, yellowish-brown papules over the forehead, sides of the face, and shoulders. The papules measured 0.5-0.8 cm in diameter, we re discrete, well defined, oval and flat topped without any surface changes (Fig. 1). Multiple cafe-au-lait macules varying from 0.5 to 3.0 cm in diam eter were present over the trunk (Fig. 2). There was no freckling in the ax illary or the inguinal regions and no neurofibromas were present. A diagnos is of juvenile xanthogranuloma was considered. Complete blood counts, urine analysis, hepatic and renal function tests, an d serum lipid levels were normal. A skin biopsy from a papule over the shou lder revealed a dense infiltrate in the dermis underlying a normal epidermi s. The infiltrate was well demarcated and comprised lymphocytes, eosinophil s, and foamy histiocytes along with Touton giant cells and proliferating fi broblasts. This confirmed the diagnosis of juvenile xanthogranuloma. The pa rents were informed about the benign and self-limiting nature of the disord er and were advised to bring the child for regular follow-up.