Bielsa Isabel, Ariza Aurelio
Department of Dermatology, Hospital Germans Trias i Pujol, Autonomous University of Barcelona, Badalona, Barcelona, Spain.
Semin Cutan Med Surg. 2007 Jun;26(2):90-5. doi: 10.1016/j.sder.2007.02.005.
Deep morphea encompasses a variety of clinical entities in which inflammation and sclerosis are found in the deep dermis, panniculus, fascia, or superficial muscle. Morphea profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are included in this group, but overlapping of the extent and depth of cutaneous involvement in these various conditions precludes their distinction on the sole basis of clinical or even histologic examination. Furthermore, the limits between morphea profunda and generalized morphea, which usually are classified outside this group, are not clear. Histologically, all these disorders show similar inflammatory and sclerotic findings, the primary difference being the depth of these changes. Associated clinical findings, including arthralgias, arthritis, contractures, or carpal tunnel syndrome, are frequent. Although visceral complications are uncommon, pulmonary, esophageal, and even cardiac abnormalities have been reported. Eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate may be present with disease activity. Laboratory studies may demonstrate autoantibody production. Treatment is nonstandardized but UVA irradiation and antiinflammatory or immunosuppressive drugs (mainly antimalarial agents and corticosteroids) may be beneficial.
深部硬皮病包括多种临床类型，其炎症和硬化见于真皮深层、皮下组织、筋膜或浅表肌肉。深部硬斑病、嗜酸性筋膜炎和儿童致残性全硬化性硬皮病均属此类，但这些不同疾病在皮肤受累范围和深度上存在重叠，使得仅根据临床甚至组织学检查难以区分它们。此外，深部硬斑病与通常不归入此类的泛发性硬皮病之间的界限并不明确。组织学上，所有这些疾病都有相似的炎症和硬化表现，主要区别在于这些改变的深度。常见的相关临床表现包括关节痛、关节炎、挛缩或腕管综合征。虽然内脏并发症不常见，但已有肺部、食管甚至心脏异常的报道。疾病活动期可能出现嗜酸性粒细胞增多、高球蛋白血症和红细胞沉降率升高。实验室检查可能显示自身抗体产生。治疗尚无标准化方案，但紫外线A照射以及抗炎或免疫抑制药物（主要是抗疟药和皮质类固醇）可能有益。
