A Case of C9 Deficiency Associated with Impetigo Contagiosum-like Eruptions

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  • TSUBOI Satoshi
    Department of Dermatology, Graduate School of Biomedical Sciences, Hiroshima University
  • MIHARA Shoji
    Department of Dermatology, Graduate School of Biomedical Sciences, Hiroshima University
  • IWAMOTO Kazuma
    Department of Dermatology, Graduate School of Biomedical Sciences, Hiroshima University
  • SHINDO Hajime
    Department of Dermatology, Graduate School of Biomedical Sciences, Hiroshima University
  • HIDE Michihiro
    Department of Dermatology, Graduate School of Biomedical Sciences, Hiroshima University
  • MOCHIZUKI Mitsuru
    Mochizuki Clinic
  • HATANAKA Michiyo
    Department of Medical Technology, Kobe Tokiwa University
  • KITANO Etsuko
    Department of Medical Technology, Kobe Tokiwa University
  • KITAMURA Hajime
    Department of Nutritional Sciences for Well-being, Kansai University of Welfare Sciences

Bibliographic Information

Other Title
  • 膿痂疹様皮疹を契機に診断した補体 C9 欠損症の 1 例
  • 症例 膿痂疹様皮疹を契機に診断した補体C9欠損症の1例
  • ショウレイ ノウカシンヨウ ヒシン オ ケイキ ニ シンダン シタ ホタイ C9 ケッソンショウ ノ 1レイ

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Abstract

A six-year-old boy was admitted to our hospital with an eight-month history of refractory seropapules and crust on his body, especially on arms and legs. He was treated as impetigo contagiosum with oral and topical antibiotics and topical steroid, but the eruptions on his elbows and knees did not disappear. A specimen from his elbow showed a focal spongiotic change in the epidermis and massive lymphocytic and neutrophilic infiltration and fibrinoid degeneration of vessels in the upper and middle dermis, suggesting primary or secondary vasculitis. Complement deficiency was suspected, because total hemolytic complement activity (CH50) in serum was lower than the detection limit, and CH50 in plasma was only 9.4 U/ml. In contrast, the amounts of C3 and C4 were within the normal range, and an HCV antibody test was negative. The titer of CH50 in his serum was 24% of that in a normal healthy serum pool tested in normal ion strength buffer, and decreased to 4% in low ion strength buffer, suggesting a deficiency of C9. Moreover, the amount of C9 in his serum was less than the limit of detection, and up to 94% of normal serum CH50 activity was restored when purified C9 was added to his serum. We thus confirmed the diagnosis of C9 deficiency.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 74 (6), 585-588, 2012

    Western Division of Japanese Dermatological Association

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