A case of congenital insensitivity to pain with anhidrosis, an intractable ulcer of the oral mucosa, and fever of unknown origin

• ARAI Chiharu

  Department of Oncological Science (Oral and Maxillo-Facial Surgery), Oita University Faculty of Medicine

• MATSUMOTO Yusi

  Department of Oncological Science (Oral and Maxillo-Facial Surgery), Oita University Faculty of Medicine

• FURUKAWA Masahide

  Department of Oncological Science (Oral and Maxillo-Facial Surgery), Oita University Faculty of Medicine

• SAEKI Maki

  Department of Oncological Science (Oral and Maxillo-Facial Surgery), Oita University Faculty of Medicine

• MIZUKI Harumi

  First Department of Oral and Maxillofacial Surgery, School of Dentistry, Iwate Medical University

• YANAGISAWA Shigetaka

  Department of Oncological Science (Oral and Maxillo-Facial Surgery), Oita University Faculty of Medicine

Bibliographic Information

Other Title

• 口腔粘膜の難治性潰ようおよび不明熱を呈した先天性無痛無汗症の１例

Abstract

We report a case of congenital insensitivity to pain with anhidrosis (CIPA), a intractable ulcer of the oral mucosa, and fever of unknown origin. A 4-month-old girl was referred to our clinic on May, 1999 because of an ulcer of the lower gingiva and the oral floor, accompanied by a high fever. The present episode of ulcer and high fever began 1 month before presentation. The patient was malnourished as an infant, and physical findings were essentially normal. There was an ulcer, measuring 3×3cm on the lower gingiva and the oral floor. We treated the ulcerative lesion, and she was admitted to the Department of pediatrics to improve physical status. Clinical examination did not reveal the cause. CT and MRI scans of the head showed no abnormal findings. While we were perplexed about the diagnosis, CIPA was suggested when the patient showed painless behavior at venipuncture, biting her finger. CIPA was diagnosed by the sweating test, biopsy of nerve and skin, and mutations in the TRKA/NGF receptor gene. The ulcer of the gingiva and oral floor rapidly improved in response to exercise restraint of an arm. Afterward, ulcers of inferior lingual surface and buccal mucosa appeared with teeth eruption, but the ulcers improved after milling the teeth.

Journal

• Japanese Journal of Oral and Maxillofacial Surgery

  Japanese Journal of Oral and Maxillofacial Surgery 50 (12), 765-768, 2004

  Japanese Society of Oral and Maxillofacial Surgeons

Keywords

• congenital insensitivity to pain with anhidrosis (CIPA)
• intractable ulcer
• fever of unknown origin

Details

• CRID: 1390001206533476736
• NII Article ID: 10018618613
• NII Book ID: AN00189163
• DOI: 10.5794/jjoms.50.765
• ISSN: 21861579
• ISSN: 00215163
• Web Site: https://search.jamas.or.jp/link/ui/2005128717
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
• Abstract License Flag: Disallowed