表皮母斑症候群の1例  [in Japanese] A Case of Epidermal Nevus Syndrome  [in Japanese]

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Author(s)

    • 佐藤 直樹 SATO Naoki
    • 愛媛大学大学院医学系研究科感覚皮膚医学 Department of Dermatology, Ehime University Graduate School of Medicine
    • 藤山 幹子 TOHYAMA Mikiko
    • 愛媛大学大学院医学系研究科感覚皮膚医学 Department of Dermatology, Ehime University Graduate School of Medicine
    • 青木 恵美 AOKI Megumi
    • 愛媛大学大学院医学系研究科感覚皮膚医学 Department of Dermatology, Ehime University Graduate School of Medicine
    • 橋本 公二 HASHIMOTO Koji
    • 愛媛大学大学院医学系研究科感覚皮膚医学 Department of Dermatology, Ehime University Graduate School of Medicine
    • 木村 美佳 KIMURA Mika
    • 愛媛大学大学院医学系研究科頭頚部感覚器外科 Department of Otolaryngology, Ehime University Graduate School of Medicine
    • 羽藤 直人 HATO Naoto
    • 愛媛大学大学院医学系研究科頭頚部感覚器外科 Department of Otolaryngology, Ehime University Graduate School of Medicine

Abstract

10ヵ月,女児。出生時より左後頭部から左頬部,耳介にかけて表面が粗造な褐色の局面を認めた。生後9ヵ月頃より顔面神経麻痺が出現し,当科初診時,局面は外耳道内まで肉眼的に連続し,外耳道はほぼ腫瘍で充満していた。頭部CT検査では中耳真珠腫の所見であり,外耳から中耳にかけて連続した病変により,顔面神経の圧迫を生じたと考えられた。病理組織学的に,左側頭部病変では角層は肥厚し,表皮は顆粒層の肥厚を認め乳頭状に増殖していた。外毛根鞘の発達した異常な毛包が多数認められ,未熟な毛包を欠くことから脂腺母斑を否定し,表皮母斑と診断した。左乳突洞内腫瘍は,病理組織学的に皮膚付属器を認めないこと以外は左側頭部病変と同様であった。左視神経乳頭形成異常,網膜萎縮,先天性内斜視を合併しており,表皮母斑症候群と診断した。

We report a case of 10-month-old baby who presented with a brown rough plaque on her scalp, cheek and auricle. the plaque was found to continue into the external auditory canal. Facial nerve palsy appeared at nine months after birth. A cranial CT scan revealed the continuous involvement of a tumor from the external ear to the middle ear, which consisted of a keratinous mass. The CT results suggested that the tumor was an aural cholesteatoma. The cause of the facial nerve palsy was the compression of the nerve by the tumor mass. The histology of the temporal plaque showed hypertrophic epidermis with hyperkeratosis, hypergranulosis, and prominent papillomatosis, and many hair follicles with hypertrophic outer root sheaths. The histological findings in the left antrum mastoideum were similar to the left temporal lesion, except for the absence of hair follicles. Because of other complications including left opticopupillary dysplasia, retinal atrophy, and congenital esotropia, we diagnosed her as having epidermal nevus syndrome.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 72(4), 353-355, 2010-08-01

    Western Division of Japanese Dermatological Association

References:  3

Codes

  • NII Article ID (NAID)
    10026636874
  • NII NACSIS-CAT ID (NCID)
    AN00183881
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    03869784
  • NDL Article ID
    10806402
  • NDL Source Classification
    ZS39(科学技術--医学--皮膚科学・泌尿器科学)
  • NDL Call No.
    Z19-69
  • Data Source
    CJP  NDL  J-STAGE 
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