2002 Volume 112 Issue 2 Pages 137-142
Pilomatricoma is the most common hair-follicle tumor. In 1965, Cantwell and Reed first reported an association of pilomatricoma with myotonic dystrophy. Since then, many cases have been reported. We report a case of unusual, multiple, giant pilomatricomas in a Japanese 35-year-old man. His younger sister also had congenital myotonic dystrophy. On first examination, he had five lesions in all on the scalp, trunk, and right upper arm. Three of them were 1.5×1 cm, 2×1.5 cm, and 3.1×1.5 cm subcutaneous nodules on the scalp and right upper arm. Two of them were 90×56 cm, and 31×15 mm pediculated giant tumors on the chest and right upper arm. These lesions had enlarged over about 3 or 4 years. Physical examination revealed the typical clinical features of myotonic dystrophy such as male pattern baldness, cataract, atrophy of the sternocleidomastoid, grip myotonia, and percussion myotonia. All of the tumors were renewed, and all of them were found to be benign pilomatricomas.
