Kurc D, De Saint-Père R, Madoule P, Laoussadi S, Caquet R
Rev Med Interne. 1987 Jan-Feb;8(1):79-84. doi: 10.1016/s0248-8663(87)80112-1.
A 35-year old woman presented with pustulosis palmaris et plantaris evolving in acute episodes parallel to those of a rheumatic disease consisting of bilateral sacro-iliac arthritis, manubriosternal fusion and sterno-clavicular arthritis. Her brother had spondylitis compatible with the diagnosis of pustular osteo-arthritis though without pustulosis. He presented with bilateral sacro-iliac arthritis, manubriosternal fusion, sterno-clavicular arthritis and an ossified fragment detached from the antero-inferior angle of C6. The two patients had the same HLA phenotype: A1, A9, B5, B41, CW4, DR2, DR4, absence of B27. In a specimen from the sacro-iliac joint the authors found active bone remodelling and inflammatory osteitis with fibrosis and infiltrates rich in mononucleate cells. An electron microscopic study--to the authors' knowledge, the first to be performed in such cases--demonstrated intracytoplasmic filamentous inclusions in two macrophages of the synovial fringe. Only one other case of the familial form has been published so far. Like the rheumatism of acne conglobata, the spondylitis of pustulosis palmaris et plantaris is one of the causes of sterno-costo-clavicular hyperostosis.
一名35岁女性出现掌跖脓疱病，其急性发作与一种风湿性疾病平行，该风湿性疾病包括双侧骶髂关节炎、胸骨柄融合和胸锁关节炎。她的哥哥患有与脓疱性骨关节炎诊断相符的脊柱炎，但无脓疱病。他表现为双侧骶髂关节炎、胸骨柄融合、胸锁关节炎以及从C6前下角分离出的一块骨化碎片。这两名患者具有相同的HLA表型：A1、A9、B5、B41、CW4、DR2、DR4，无B27。在骶髂关节标本中，作者发现有活跃的骨重塑和炎性骨炎，伴有纤维化和富含单核细胞的浸润。一项电子显微镜研究——据作者所知，这是此类病例中的首例——在滑膜边缘的两个巨噬细胞中发现了胞质内丝状包涵体。迄今为止，仅发表过另一例家族性病例。与聚合性痤疮的风湿病一样，掌跖脓疱病的脊柱炎是胸肋锁骨肥厚症的病因之一。
