A Case of Giant Multiple Pilomatricomas Associated with Myotonic Dystrophy

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  • Yoshida Aki
    Department of Dermatology, Iwate University School of Medicine
  • Ohara Manabu
    Department of Dermatology, Iwate University School of Medicine
  • Onodera Hanae
    Department of Dermatology, Iwate University School of Medicine
  • Sato Toshiki
    Department of Dermatology, Iwate University School of Medicine
  • Mori Yasuki
    Department of Dermatology, Iwate University School of Medicine
  • Akasaka Toshihide
    Department of Dermatology, Iwate University School of Medicine
  • Mizuno Masanobu
    Department of Intenal Neurology, Iwate University School of Medicine
  • Hakozaki Mika
    Department of Plastic Surgery, Iwate University School of Medicine
  • Yoshinari Chikara
    Department of Dermatology, Kuji prefectural Hospital

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Other Title
  • 筋緊張性ジストロフィーに合併した多発性巨大石灰化上皮腫の1例
  • キン キンチョウセイ ジストロフィー ニ ガッペイ シタ タハツセイ キョダイセキ ハイカ ジョウヒ シュ ノ 1レイ

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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.

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