Anadure R K, Shankar S, Mohimen Aneesh, Pemmaraju Arpitha, Kalita Jitumani
Senior Advisor (Neurology), Command Hospital (Air Force), Bengaluru, India.
Professor & Head, Department of Internal Medicine, Armed Forces Medical College, Pune 411040, India.
Med J Armed Forces India. 2022 Sep;78(Suppl 1):S273-S276. doi: 10.1016/j.mjafi.2019.12.006. Epub 2020 Mar 12.
Shulman's disease (eosinophilic fasciitis) is a very rare autoimmune disorder with an unknown etiopathogenesis. During the initial period of disease, it usually causes limb and trunk edema followed by collagenous thickening of the subcutaneous fascia. Eosinophilia is a predominant laboratory finding during the initial phase of the disease and less prominent in the later phases. Patients may also present with arthritis, myositis, peripheral neuropathy, and rarely pleuropericarditis. Here, we are reporting a case of eosinophilic fasciitis presenting with vague constitutional symptoms, fever, and peripheral blood eosinophilia followed by rapidly evolving skin tightening with joint contractures and muscle stiffness, which misled the treating team toward Scleroderma and its overlap syndromes. The diagnosis was finally clinched by a full-thickness skin biopsy along with underlying fascia and muscle tissue from an effected area, with a gratifying treatment response to standard immune suppression.
舒尔曼病（嗜酸性筋膜炎）是一种非常罕见的自身免疫性疾病，其发病机制不明。在疾病初期，它通常会导致肢体和躯干水肿，随后皮下筋膜出现胶原性增厚。嗜酸性粒细胞增多是疾病初期的主要实验室检查发现，在后期则不太明显。患者还可能出现关节炎、肌炎、周围神经病变，很少出现胸膜心包炎。在此，我们报告一例嗜酸性筋膜炎病例，该病例最初表现为模糊的全身症状、发热和外周血嗜酸性粒细胞增多，随后迅速出现皮肤紧绷伴关节挛缩和肌肉僵硬，这使治疗团队误诊为硬皮病及其重叠综合征。最终通过对受累部位进行全层皮肤活检以及取其下方的筋膜和肌肉组织确诊，对标准免疫抑制治疗反应良好。
