慢性结节性软骨皮炎是一种罕见的外耳非肿瘤性炎症和退行性过程，以真皮中延伸到软骨膜的坏死生物变化为特征。这种情况偶尔在局限性皮肤系统性硬化症患者中有报道，但在合并原发性胆汁性肝硬化的患者中没有报道；这种关联被称为雷诺兹综合征。我们报告了一名70岁的妇女，该妇女在47岁时被诊断为原发性胆汁性肝硬化，在54岁时患有皮肤系统性硬化症，在最后一次诊断后不久，左耳的螺旋边缘出现了疼痛的溃疡结节。因为怀疑是恶性肿瘤而切除了病灶,但组织病理学与慢性螺旋型结节性软骨皮炎一致。虽然这种情况很少见，有必要知道，因为它可能发生在系统性硬化症患者身上，被误认为是肿瘤，如基底细胞癌和鳞状细胞癌，这些患者患皮肤恶性肿瘤的风险增加。
Chondrodermatitis nodularis chronica helicis is a rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to the perichondrium. This condition has been occasionally reported in patients with limited cutaneous systemic sclerosis but not in those with concomitant primary biliary cirrhosis; this association is known as Reynolds syndrome. We report a 70-year-old woman diagnosed with primary biliary cirrhosis at age 47 and with limited cutaneous systemic sclerosis at age 54 who developed a painful ulcerated nodule on the helical rim of the left ear shortly after the last diagnosis. The lesion was excised because of the suspicion of malignancy, but the histopathology was consistent with chondrodermatitis nodularis chronica helicis. Although this condition is infrequent, it is necessary to know, because it may occur in patients with systemic sclerosis and be mistaken for neoplasms, such as basal cell and squamous cell carcinoma, and these patients have an increased risk for the development of skin malignancies.
