Forman M B, Lewin J R, Gear A J, Solomon L
S Afr Med J. 1981 Apr 4;59(15):540-2.
Eosinophilic fasciitis was first described by Shulman in 1974 and is characterized by oedema of the extremities, peripheral eosinophilia, hypergammaglobulinaemia and inflammation of the deep fascia. The condition has not been previously reported in South Africa. We describe 2 cases seen at the Rheumatology Clinic of the Johannesburg Hospital recently. The most striking clinical features were the marked palmar fascial thickening and oedema of the hands and feet. The latter is said to be uncommon in this syndrome and mimicked the early changes of scleroderma. Furthermore, blood eosinophilia may also occur in scleroderma. Our patients illustrated the extremely variable laboratory features in this disorder. In accordance with previous findings in the literature, visceral involvement was absent in our cases. We suggest that in patients without Raynaud's phenomenon who present with scleroderma-like changes of the distal extremities, a diagnosis of eosinophilic fasciitis should be excluded by a full-thickness biopsy.
嗜酸性筋膜炎于1974年由舒尔曼首次描述,其特征为四肢水肿、外周血嗜酸性粒细胞增多、高球蛋白血症以及深筋膜炎症。此前南非尚未有该病的报道。我们描述了最近在约翰内斯堡医院风湿病诊所所见的2例病例。最显著的临床特征是手掌筋膜明显增厚以及手足水肿。据说后者在该综合征中并不常见,且类似硬皮病的早期改变。此外,硬皮病患者也可能出现血液嗜酸性粒细胞增多。我们的患者展示了该疾病极为多样的实验室特征。与文献中先前的发现一致,我们的病例均无内脏受累情况。我们建议,对于无雷诺现象且出现远端肢体硬皮病样改变的患者,应通过全层活检排除嗜酸性筋膜炎的诊断。