Abstract：
Fibroblastic rheumatism (FR) is an uncommon disease of the skin, characterized by the presence of non-tender cutaneous nodules accompanied often by other rheumatic manifestations. This condition shows male predominance, no age preference and unpredictable course, resulting frequently in permanent joint damage. A 60-year-old man came to our department with a 4-year history of multiple non-tender nodules and morning stiffness affecting mainly the upper extremities. Clinical examination revealed arthritis of the hands, confirmed by imaging tests. Laboratory exams were unremarkable. A skin nodule biopsy showed a dermal collagenous lesion with myxoid areas composed of spindle and stellate cells. Immunohistochemical staining demonstrated positivity for CD68 and negativity for CD34, S100, EMA and desmine. FR was diagnosed and the patient started methylprednisolone 16 mg/day. Hydroxychloroquine 400 mg/day and methotrexate 15 mg/weekly were further added as steroid-sparing agents with clinical benefit. Clinicians should be aware of this underreported entity, which can rapidly lead to irreversible deformities.
摘要：
纤维母细胞性风湿病（FR）是一种罕见的皮肤病，其特征是存在非压痛的皮肤结节，通常伴有其他风湿病表现。这种情况表明男性占主导地位，没有年龄偏好和不可预测的课程，经常导致永久性关节损伤。一名60岁的男子来到我们部门，有4年的多个非招标结节和晨僵的历史，主要影响上肢。临床检查显示手关节炎，通过影像学检查证实。实验室检查并不引人注目。皮肤结节活检显示真皮胶原性病变，粘液样区域由纺锤体和星状细胞组成。免疫组织化学染色显示CD68阳性，CD34，S100，EMA和去胺阴性。诊断为FR，患者开始使用甲基强的松龙16mg/天。进一步加入400mg/天的羟氯喹和15mg/周的甲氨蝶呤作为具有临床益处的类固醇保护剂。临床医生应该意识到这个被低估的实体，会迅速导致不可逆的畸形。
