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Published on: February 8, 2019
Fibroblastic rheumatism: an addition to fibromatosis
Lanlan Ji1, Yan Geng, Yanjie Hao
1Department of Rheumatology and Clinical Immunology, Beijing University First Hospital, No. 8 Xishiku Street West District, 100034 Beijing, China.
Abstract:
Fibroblastic rheumatism (FR) is a rare rheumatologic entity of unknown etiology. It is characterized by symmetrical polyarthritis associated with multiple cutaneous nodules. Bone erosion can occur as the disease progresses and destructive arthropathy is not rare. We report on an 18-year-old man with FR who presented a 6-year history of cutaneous nodules localized at para-articular sites with only minimal oligoarthralgia on exertion. There was no visceral involvement, and all the routine and immunological tests were normal. The diagnosis of FR was confirmed by histological examination of a nodule, which composed of myofibroblastic proliferation and thickened collagen fibers. Most skin lesions resolved after treated with IFN-α, however there was sequelae of permanent disability due to the progressive bone erosion despite weekly methotrexate treatment.
Insights
Fibroblastic rheumatism (FR) is a rare condition causing joint pain and skin nodules. This case highlights progressive bone erosion and disability despite treatment, emphasizing the need for further research.
Area of Science:
- Rheumatology
- Dermatology
- Histopathology
Background:
- Fibroblastic rheumatism (FR) is a rare rheumatologic disorder of unknown cause.
- It typically presents with symmetrical polyarthritis and cutaneous nodules.
- Progressive bone erosion and destructive arthropathy can occur.
Observation:
- An 18-year-old male presented with a 6-year history of para-articular cutaneous nodules and minimal exertional oligoarthralgia.
- Routine and immunological tests were normal, with no visceral involvement.
- Histological examination confirmed myofibroblastic proliferation and thickened collagen fibers in a nodule.
Findings:
- The patient received Interferon-alpha (IFN-α), which resolved most skin lesions.
- Despite weekly methotrexate treatment, progressive bone erosion led to permanent disability.
- This case illustrates a severe outcome of fibroblastic rheumatism.
Implications:
- Further research into the etiology and optimal treatment of fibroblastic rheumatism is warranted.
- Early diagnosis and aggressive management may be crucial to prevent long-term joint damage.
- This case underscores the potential for significant morbidity in FR, even with standard therapies.
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