Katz D, Berhault J
Clinique du Ter, Ploemeur.
Ann Chir Main Memb Super. 1990;9(3):229-31. doi: 10.1016/s0753-9053(05)80556-3.
Shulman's syndrome or fasciitis with eosinophilia is a disease of unknown origin with unclear relations with scleroderma, characterised by thickening of the fascia superficialis associated with blood eosinophilia. This diagnosis should be considered in a patient presenting with a lesion of the anterior surface of the forearms, which have an "armoured" appearance, associated with claw deformity of the wrist, hand and fingers. The diagnosis is based on cutaneo-muscular biopsy and treatment consists of corticosteroids. The clinical course is generally favourable, although recovery is sometimes incomplete. There is generally no visceral or systemic involvement apart from rare cytopenic forms which must be identified.
舒尔曼综合征或嗜酸性筋膜炎是一种病因不明、与硬皮病关系不清的疾病,其特征为浅筋膜增厚并伴有血液嗜酸性粒细胞增多。对于出现前臂前表面病变、有“铠甲样”外观并伴有手腕、手部和手指爪形畸形的患者,应考虑这一诊断。诊断基于皮肤肌肉活检,治疗包括使用皮质类固醇。尽管恢复有时不完全,但临床病程总体良好。除了必须识别出的罕见血细胞减少形式外,一般无内脏或全身受累情况。