Gallais V, Crickx B, Belaich S
Service de Dermatologie, Hôpital Bichat, Paris.
Ann Dermatol Venereol. 1996;123(11):722-6.
Prognosis in dermatomyositis is severe, partly due to the development of cancer. The aim of this study was to identify factors predicting cancer development and assess factors predicting reduced survival rate.
A retrospective analysis of 32 cases of dermatomyositis diagnosed on the basis of the Bohan and Peter criteria was performed. Diagnosis was certain in 7 cases, probable in 13 and possible in 5. There were thus 7 cases of pure cutaneous dermatomyositis. Clinical and laboratory data were compared between patients with and without cancer and between deceased and surviving patients.
Overall mortality was 37.5% at 4 years, confirming the gravity of dermatomyositis. Malignancy developed in 9 patients (28.1%) leading to death in all cases, within 18 months in 8. Amyopathic dermatomyositis was observed in 2 of these patients. Necrotic skin ulcerations (p < 0.01) and pruritus (p < 0.05) were significant predictive factors for the development of cancer. Poor prognosis factors were malignancy (p < 0.001), necrotic skin ulcerations (p < 0.01), and pruritus (p < 0.05).
Prognosis is poor in certain sub-groups of patients with dermatomyositis. Such patients can be identified on the basis of skin lesions, notable necrotic ulcerations.
皮肌炎的预后严重，部分原因是癌症的发生。本研究的目的是确定预测癌症发生的因素，并评估预测生存率降低的因素。
对32例根据博汉和彼得标准诊断的皮肌炎病例进行回顾性分析。确诊7例，可能13例，疑似5例。因此，有7例为单纯皮肤型皮肌炎。对有癌和无癌患者以及死亡和存活患者的临床和实验室数据进行了比较。
4年时总死亡率为37.5%，证实了皮肌炎的严重性。9例患者（28.1%）发生恶性肿瘤，所有病例均导致死亡，其中8例在18个月内死亡。其中2例患者出现无肌病性皮肌炎。坏死性皮肤溃疡（p<0.01）和瘙痒（p<0.05）是癌症发生的重要预测因素。预后不良因素为恶性肿瘤（p<0.001）、坏死性皮肤溃疡（p<0.01）和瘙痒（p<0.05）。
某些皮肌炎患者亚组的预后较差。这类患者可根据皮肤病变，尤其是坏死性溃疡来识别。
