Ben Hamida S K, Kédadi H, Derbel F, Kchir M, Bouhaoula H, Hadj S M, Mestiri A, Zouari R, Dougui M H
Service de médecine interne, Hôpital des forces de sécurité intérieure de la Marsa, Tunis.
Presse Med. 2003 Sep 27;32(31):1455-6.
Hypertrophic osteoarthropathy (HOA) is a syndrome associating hippocratic fingers, arthropathy and periostosis of long bones. Currarino's disease, considered at present as a clinical form of primary HOA, is characterized by the absence of pachydermia.
A 24-year-old Caucasian man, consulted for a painful swelling of both ankles that had developed over the past year. Clinical examination revealed hippocratic fingers without pachydermia. The ankles were swollen. The X-rays showed periosteal apposition and an acro-osteolysis. In view of this triad: arthropathy, hippocratic fingers and periostosis, primary HOA without cutaneous involvement or Currarino's disease was diagnosed. The search for a secondary cause remained negative. Clinical improvement was obtained after 15 months with non-steroidal anti-inflammatory drugs (NSAID) and colchicine.
Although exceptional, primary HOA without cutaneous involvement is a genetic disease which must not be ignored.
肥厚性骨关节病(HOA)是一种伴有杵状指、关节病和长骨骨膜增生的综合征。目前被认为是原发性HOA临床形式的库拉里诺病,其特征是没有厚皮症。
一名24岁的白种男性,因过去一年出现的双踝疼痛性肿胀前来就诊。临床检查发现有杵状指但无厚皮症。双踝肿胀。X线显示骨膜增生和肢端骨质溶解。鉴于这三联征:关节病、杵状指和骨膜增生,诊断为无皮肤受累的原发性HOA或库拉里诺病。对继发性病因的检查结果为阴性。使用非甾体抗炎药(NSAID)和秋水仙碱治疗15个月后临床症状改善。
尽管罕见,但无皮肤受累的原发性HOA是一种不可忽视的遗传性疾病。