Ramírez-Bellver Jose Luis, Macías Elena, Bernárdez Claudia, López-Robles Joaquín, Vegas-Sánchez Maria Del Carmen, Díaz-Recuero Jose Luis, Quiceno Hernán, Requena Luis
Departments of*Dermatology, †Immunology, and ‡Pathology, Fundación Jimenez Diaz, Universidad Autónoma, Madrid, Spain.
Am J Dermatopathol. 2017 Jan;39(1):e3-e7. doi: 10.1097/DAD.0000000000000694.
Paraneoplastic syndromes consist of a group of disorders that are not related to the extension of the primary tumor or its metastases and that might be the first manifestation of a hidden neoplasm. It is a well-known association between dermatomyositis (DM) and cancer, especially gynecological tumors in women and lung cancer in men.
We describe the case of a 67-year-old male who developed muscular weakness and pruritic skin lesions. Skin biopsies were performed and histologic findings were consistent with DM.
Skin biopsy showed interface dermatitis with vacuolar degeneration of the basal layer, dermal mucin deposits, and necrotic keratinocytes in the acrosyringia, a finding that has been previously reported in lupus erythematous but not in DM. Autoimmunity tests showed positivity for antinuclear antibodies and anti-NXP2, a recently described antibody associated with juvenile DM and, more rarely, with paraneoplastic DM.
We present the first case in the literature with histopathologic changes of DM affecting the acrosyringia. Besides, our patient autoimmunity results support the utility of the new myositis-specific autoantibodies and its relation with a clinical phenotype.
副肿瘤综合征由一组与原发性肿瘤的扩展或转移无关的疾病组成，可能是隐匿性肿瘤的首发表现。皮肌炎（DM）与癌症之间存在众所周知的关联，尤其是女性的妇科肿瘤和男性的肺癌。
我们描述了一名67岁男性出现肌肉无力和瘙痒性皮肤病变的病例。进行了皮肤活检，组织学结果与皮肌炎一致。
皮肤活检显示界面性皮炎伴基底层空泡变性、真皮粘蛋白沉积以及顶泌汗腺导管内坏死的角质形成细胞，这一发现先前在红斑狼疮中报道过，但在皮肌炎中未见报道。自身免疫检测显示抗核抗体和抗NXP2呈阳性，抗NXP2是一种最近描述的与青少年皮肌炎相关的抗体，在副肿瘤性皮肌炎中较少见。
我们报道了文献中首例出现影响顶泌汗腺导管的皮肌炎组织病理学改变的病例。此外，我们患者的自身免疫结果支持新型肌炎特异性自身抗体的实用性及其与临床表型的关系。
