Aquagenic pruritus associated with juvenile xanthogranuloma

While the relationship of aquagenic pruritus (AP) to haematoproliferative conditions is well-recognized, an association with histiocytic disorders has not previously been described. The case of a child with both AP and juvenile xanthogranulomata (JXG) is reported. The cutaneous lesions were typical of JXG, both clinically and histologically, and in addition there were radiological changes consistent with JXG in the lungs. AP is rare in childhood and the coincidental occurrence with unusually extensive JXG seems unlikely.