烧伤后莫菲斯病与嗜酸性筋膜炎重叠的严重病例

H. Sami, Faria Sami, Shahzad Ahmed Sami, A. Nashwan
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摘要

背景。全身性斑秃是一种罕见的纤维性皮肤病,会从红斑、紫斑发展为硬化斑。另一种不常见的免疫介导结缔组织疾病嗜酸性粒细胞筋膜炎(EF)也会导致硬化和皮肤木质化。这两种疾病同时存在的情况极为罕见,而且预后较差。我们的病例报告是首例将烧伤作为嗜酸性粒细胞性筋膜炎和全身性斑秃重叠的诱发因素的报告。病例介绍。一名 36 岁的男性患者在一次汽油爆炸烧伤后不久,即出现四肢皮肤迅速增厚、触痛性水肿和皮肤挛缩。检查结果显示,患者有外周嗜酸性粒细胞增多、高丙种球蛋白血症和 C 反应蛋白升高。皮肤活检显示硬皮样变和皮下纤维隔硬化增厚,伴有基质粘蛋白、真皮血管周围弥漫性淋巴浆细胞浸润,其中有嗜酸性粒细胞,CD34表达降低,XIIIa因子升高。随后,他被诊断为全身性斑秃和嗜酸性粒细胞性筋膜炎重叠。使用类固醇、甲氨蝶呤、霉酚酸酯和三苯氧胺醋酸内注射剂后,患者的病情仅得到有限改善。结论伴有EF的全身性斑秃表明存在一定程度的抗药性,预后较差,生活质量较低。烧伤可能是这种重叠综合征的诱发因素。有必要及时识别高危人群并采取积极的治疗措施。
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A Severe Case of Overlap of Morphea and Eosinophilic Fasciitis after Burn Injuries
Background. Generalized morphea is a rare fibrosing skin illness that progresses from erythematous, violet-colored skin patches to sclerotic plaques. Another uncommon immune-mediated connective tissue disease called eosinophilic fasciitis (EF) evolves to cause sclerosis and woody skin induration. The coexistence of the two is extremely rare and has a poorer prognosis. Our case report is one of the first to report burn injuries as a trigger factor for EF and generalized morphea overlap. Case Presentation. A 36-year-old man presented with acute onset of rapidly progressing skin thickening, tender edema, and skin contractures involving all extremities, shortly after enduring burn injuries from a gasoline explosion. Workup was remarkable for peripheral eosinophilia, hypergammaglobulinemia, and elevated C-reactive protein. Skin biopsy demonstrated sclerodermoid changes and sclerotic thickening of subcutaneous fibrous septa associated with stromal mucin, dermal perivascular, diffuse lymphoplasmacytic infiltrate with eosinophils, decreased CD34 expression, and increased factor XIIIa. He was subsequently diagnosed with an overlap of generalized morphea and eosinophilic fasciitis. The patient had only limited improvement with steroids, methotrexate, mycophenolate mofetil, and intralesional triamcinolone acetonide injections. Conclusion. Generalized morphea with concomitant EF indicates some degree of therapeutic resistance and poor prognosis with a low quality of life. Burn injuries can be a trigger factor for this overlap syndrome. Prompt identification of at-risk individuals and initiating aggressive management are necessary.
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