A Case of Cryopyrin-associated Periodic Syndrome

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  • KOBAYASHI Hiroko
    Department of Dermatology, Institute of Biomedical & Health Sciences, Hiroshima University
  • HIRAGUN Takaaki
    Department of Dermatology, Institute of Biomedical & Health Sciences, Hiroshima University
  • HANAOKA Keiko
    Department of Dermatology, Institute of Biomedical & Health Sciences, Hiroshima University
  • YASUMURA Junko
    Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences
  • NISHIKOMORI Ryuta
    Department of Pediatrics, Kyoto University Graduate School of Medicine
  • HEIKE Toshio
    Department of Pediatrics, Kyoto University Graduate School of Medicine
  • HIDE Michihiro
    Department of Dermatology, Institute of Biomedical & Health Sciences, Hiroshima University

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Other Title
  • クリオピリン関連周期熱症候群の 1 例
  • 症例 クリオピリン関連周期熱症候群の1例
  • ショウレイ クリオピリン カンレン シュウキ ネツ ショウコウグン ノ 1レイ

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<p>A 6-month-old male developed daily erythema and wheal without apparent itch starting from the day after birth. Antihistamines did not improve his urticaria-like eruptions. Blood tests showed an increase in the number of peripheral blood leukocytes (29570 cells/mm3) and elevated levels of inflammatory markers (CRP : 4. 65 mg/dl ; SAA : 106. 2 μg/ml). In addition, skin biopsy specimens showed the infiltration of neutrophils into the perivascular area and edema in the dermis. Genetic analysis revealed the heterozygous variant of c.1792A＞T (p.Ile598Phe) in the NLRP3 gene, confirming the diagnosis of cryopyrin-associated periodic syndrome (CAPS). The administration of an humanized anti-human IL-1β monoclonal antibody, canakinumab, induced the prompt disappearance of the urticaria-like eruptions and the normalization of inflammatory markers. It is important to consider CAPS as a differential diagnosis of chronic spontaneous urticaria if wheallike eruptions appear and repeat starting from shortly after birth. The genetic analysis of NLRP3 at an early age enables early treatment with canakinumab, which quickly remits symptoms and could improve the long-term prognosis of patients with CAPS.</p>

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