A male infant was born to a 29-year-old woman (gravida 3, para 2), following an uncomplicated pregnancy and normal vaginal delivery. At birth, a brownish 1-cm nodule was noted on the right side of the upper abdomen. The infant was otherwise healthy.
Serum cholesterol and triglyceride levels were normal. A skin biopsy was performed. Histopathologic examination of the lesion showed lipid-laden histiocytes, admixed inflammatory cells (plasma cells, eosinophils, and neutrophils), and multinucleated giant cells.
Alexander K. C. Leung, MD, and Justine H. S. Fong, MD, of Calgary, Alberta, diagnosed juvenile xanthogranuloma. Typically, the nodules are soft or firm, dome-shaped, yellow, pink, orange, or brown, and vary in size from a few millimeters to 4 cm in diameter. The lesions may be as few as 1 or 2 or as many as several hundred. They are usually present at birth or appear within the first 6 to 9 months of life. The condition is 10 times more common in white than in black children. Affected children are otherwise healthy and have normal lipid levels.
Complete spontaneous involution is the rule. Scarring does not occur; however, residual pigmentation or atrophy of the skin may be noted on occasion.