Head and neck manifestations of tumoral calcinosis

Intraoral facial and laryngeal features of tumoral calcinosis are reviewed in six patients of Jewish-Yemenite descent. Extraoral features included calcified masses, erythematous patches, and angular cheilitis. Oral soft tissue findings included papillary hyperplasia of the lip vermilion and velvety-red macules on the tongue, palate, and buccal mucosa. Gingival findings included advanced periodontitis and an erythematous marginal gingivitis. In all patients, orthognathic evaluation revealed a concave profile, retruded maxilla, and relative mandibular prognathism. Although previously reported clinical dental abnormalities were not present, extracted teeth demonstrated abnormal dentin. Hoarseness was present in all patients and was the earliest appearing feature in two patients. The appearance of these head and neck findings preceded the classical manifestations of tumoral calcinosis and may be helpful in the early diagnosis of this entity. The varied reported features of tumoral calcinosis support the notion that this disease may present multiple formes frustes with variable clinical expressivity.