Department of Hematology and Cell Therapy, University Hospital of Tours, 2 Boulevard Tonnellé, 37044, Tours Cedex 9, France.
Department of Medical Imaging, University Hospital of Tours, Tours, France.
J Med Case Rep. 2021 Mar 15;15(1):135. doi: 10.1186/s13256-021-02735-3.
Shulman's disease, or eosinophilic fasciitis (EF), is a rare autoimmune disease, characterized by sclerodermic skin lesions with progressive induration and thickening of the soft tissues. Chronic graft-versus-host-disease (GVHD) presenting as EF is a very rare manifestation of cutaneous GVHD.
We report an unusual case of EF in a 46-year-old Caucasian male patient who had received an allogenic hematopoietic stem cell transplantation in the context of relapsed/refractory multiple myeloma. The diagnosis was challenging, with the patient presenting hepatic dysfunction, normal eosinophils count, and incomplete clinical signs. Magnetic resonance imaging (MRI) and skin biopsy confirmed the diagnosis of EF. Early initiation of specific treatment with corticosteroids and prednisolone achieved complete response.
In practice, incomplete signs in this rare complication should lead to MRI as it is a major tool to guide decision-making based on the skin biopsy, allowing a rapid diagnosis and the initiation of treatment without delay.
舒尔曼病，又称嗜酸性筋膜炎（EF），是一种罕见的自身免疫性疾病，其特征为硬皮病样皮肤损害，伴有软组织进行性硬化和增厚。慢性移植物抗宿主病（GVHD）表现为 EF 是皮肤 GVHD 的一种非常罕见的表现。
我们报告了一例不典型嗜酸性筋膜炎病例，患者为 46 岁白人男性，患有复发性/难治性多发性骨髓瘤，接受了同种异体造血干细胞移植。该诊断具有挑战性，患者表现为肝功能障碍、正常嗜酸性粒细胞计数和不完全的临床体征。磁共振成像（MRI）和皮肤活检证实了 EF 的诊断。早期开始使用皮质类固醇和泼尼松龙进行特异性治疗实现了完全缓解。
在实践中，这种罕见并发症的不典型体征应提示进行 MRI，因为它是一种主要的工具，可以根据皮肤活检来指导决策，从而快速诊断并及时开始治疗。
