Bosser H, Schubert B, Grosshans E
Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg.
Ann Dermatol Venereol. 1993;120(5):369-72.
We report a familial form of rheumatoid nodulosis where the early lesions appeared in the first years of life in a young man and his son. The numerous papular and nodular skin lesions disclosed an ulcerative evolution leaving atrophic scars mainly near the limbs' joints. In both patients the evolution was characterized by the late onset of polyarthralgias and of a non-destructive seronegative polyarthritis; intra-osseous lesions were present in the metatarsal bones in the father. Rheumatoid nodulosis is a rare disease, sometimes considered as a benign variant of rheumatoid arthritis without destructive joint involvement. A serum rheumatoid factor is inconstantly present and the intra-osseous geodes have been shown to be rheumatoid nodules exhibiting the same histological structures of palisading granulomas as the subcutaneous nodules. The main symptom of the disease is the occurrence of multiple nodules followed by the delayed onset of a polyarthritis with a benign course without systemic involvement.
我们报告了一种家族性类风湿结节病,早期病变出现在一名年轻男子及其儿子生命的最初几年。众多丘疹和结节性皮肤病变呈现溃疡性演变,主要在四肢关节附近留下萎缩性瘢痕。两名患者的病情演变均以多关节痛和非破坏性血清阴性多关节炎的迟发为特征;父亲的跖骨出现骨内病变。类风湿结节病是一种罕见疾病,有时被视为类风湿关节炎的良性变体,不累及破坏性关节。血清类风湿因子并非始终存在,骨内圆形病灶已被证明是类风湿结节,其组织学结构与皮下结节的栅栏状肉芽肿相同。该病的主要症状是出现多个结节,随后迟发性多关节炎起病,病程良性,无全身受累。