Multiple subcutaneous Juvenile Xanthogranulomas with a blue
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A 5-week-old infant was referred for evaluation of subcutaneous nodules on his scalp, trunk, groin, and extremities. Many lesions were present since birth, but the mother reported the appearance of new nodules appearing throughout the body over the last 2 weeks. The infant had an uncomplicated vaginal delivery at 38 weeks, at which time he was noted to have petechiae and purpura covering much of his body and several nodules on his trunk and extremities. Laboratory values at birth revealed a platelet count of 25,000, and the baby spent 2 weeks in the neonatal intensive care unit where alloimmune thrombocytopenia, unrelated to his skin nodules, was eventually diagnosed. He received multiple platelet transfusions and IV immunoglobulins infusions with eventual resolution of his thrombocytopenia.

The skin examination in office found at least 28 diffuse mobile subcutaneous nodules on the trunk, head, groin, and extremities, many of which had an overlying bluish hue. A punch biopsy of a nodule on the thigh was performed. Immunohistochemistry stained positive for CD68 and negative for S100 and CD1a. These results were consistent with the diagnosis of JXG, and the patient was referred to rule out ocular involvement.

Follow-up at 4 months found the same distribution of subcutaneous nodules but with the orange-to-tan coloration typical of JXG. At 10 months, follow-up examination found only residual hyperpigmented macules, and at 12 months there was no evidence of JXG present on examination.

This case of JXG with 28 subcutaneous nodules with overlying blue hue represents a highly atypical variant of JXG with a unique clinical presentation.