原發性肥大性骨關節病合併腎絲球腎炎：一病例報告

Pachydermoperiostosis associated with glomerulonephritis: a case report

肥大性關節病變Hypertrophic osteoarthropathy（HOA）之表現為杵狀指（趾）、及四肢長骨腫大。本病分為原發性和繼發性兩類。原發性肥大性骨關節病Pachydermoperiostosis（PDP）也稱為厚皮骨膜增生症或骨膜增生厚皮症，表徵為皮肥厚、杵狀指（趾）、及四肢長骨骨膜增生，但是並無合併心肺、肝臟、或腸疾病。其他表徵包含關節炎、皮脂漏、毛囊炎、回狀頭皮、肥厚性胃病變、及肢端骨質溶解。一位四十三歲男性有十幾年呈現四肢末端杵狀指、下肢及前臂皮膚增厚、面部特徵變粗及皮脂溢出。近一年因發現雙側膝蓋關節炎及下肢水腫而就醫。皮膚組織切片及肢體放射線發現與 PDP符合。因病患有大量蛋白尿，腎臟切片進而發現系膜增生性腎小球腎炎。右上前臂切片證實為粘液瘤。至今在醫療文獻未發現有PDP合併腎絲球炎及軟組織粘液瘤之報告。因皮膚、骨頭、及腎臟病變有共同相似之致病機轉，我們認為 PDP 與腎絲球炎之關係應為病理致病性有關聯，而非巧合。

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing fingers and periostosis of the long bones, which can be classified as the primary or secondary form. Pachydermoperiostosis (PDP), the primary idiopathic form of HOA, is featured by pachydermia, clubbing fi ngers, and periostosis of long bones, but lack of signs of cardiopulmonary, hepatic or intestinal diseases. Other manifestations include arthritis, seborrhea, folliculitis, cutis verticis gyrata, hypertrophic gastropathy and acroosteolysis. Here we report a 43 year-old male who had clubbing of the terminal phalanges, thickening of leg and forearm skin, progressive coarsening of facial features and seborrhea for more than a decade, came to our clinic due to both knee arthritis and pitting edema of lower limbs for about one year. The histology of skin and extremity x-ray findings were also consistent with the diagnosis of PDP. In addition, owing to heavy proteinuria, renal biopsy was performed and showed mesangial proliferative glomerulonephritis. He also had a myxoma over the right forearm. Described to date, there was no report of PDP associated with glomerulonephritis and soft tissue myxoma in the literature. Because similar pathogenic mechanisms involved in the changes of skin, bone and kidney have been revealed, we suggest that the coexistence of PDP and glomerulonephritis can be pathogenically rather than coincidentally related.