Skin lesions and stiff, painful joints develop between 4 and 15 years of age. The cutaneous nodules are found primarily on the hands, ears and the upper extremities. These are nonulcerating, tender, violaceous to brown in color, and firm in consistency. Firm subcutaneous plaques apparent only on palpation are also present. No mucosal lesions or xanthelasmata are present. Deforming, symmetric arthritis of the hands, feet and elbows is frequently seen with periarticular bony resorption. The skin of the legs and feet are thick and lichenified. Histology of the skin lesions shows a granulomatous appearance with a chronic inflammatory infiltrate. No multinucleated giant cells are seen.