Fibroblastic Rheumatism: A Report of Three Cases
SA Jurado, GKG Alvin, D Kress, MJJ Jamora, SD Billings. Cleveland Clinic, Cleveland, OH; Skin and Cancer Foundation, Inc., Manila, Philippines
Background: Fibroblastic rheumatism is a rare dermatoarthropathy characterized by the sudden onset of cutaneous nodules, flexion contractures and polyarthritis that may progress to destructive arthropathy. The cutaneous manifestations can present concurrently, precede or follow development of arthropathy. Histopathology, in the correct clinical context, confirms the diagnosis.
Design: Cases coded as fibroblastic rheumatism were retrieved from institutional and consultation files (SDB). Charts and biopsies were reviewed. In selected cases, elastic stains and immunostains for SMA, S100, CD34, desmin and EMA were performed.
Results: Three cases were identified. A 6-year-old Caucasian boy presented with a several month history of arthralgias and cutaneous nodules on the scalp and extremities that developed approximately six weeks after an upper respiratory tract infection. A 15-year old Filipino boy presented with cutaneous nodules on the hands and developed flexion contractures; he subsequently developed nodules on the elbows, feet and face. A 60-year-old Puerto Rican man presented with tender cutaneous nodules on the scalp, face and extremities. He had a history of inflammatory arthritis 2.5 years prior involving the wrists, fingers, elbows, shoulders, knees and ankles. In the pediatric cases, the biopsies demonstrated fascicles of bland spindled cells within the dermis that resembled early scar or fibromatosis. In the adult case the lesions were less cellular with more randomly arranged fibroblasts in a densely collagenous stroma. The nuclei were bland without hyperchromasia or mitotic activity. Adnexal structures in the dermis were relatively spared. Elastic fibers were absent in the fibroblastic proliferation (2 of 2). Uninvolved portions of the dermis had normal appearing elastic fibers. SMA stains highlighted the lesional cells in a myofibroblastic pattern in 1 of 2 cases. No immunoreactivity was seen for S100 (n=2), CD34 (n=1), desmin (n=1) or EMA (n=1). The overlying epidermis was unremarkable. The 6-year-old boy has responded to methotrexate. Methotrexate was contraindicated in the adult and refused by the 15-year-old.
Conclusions: Fibrobolastic rheumatism is a rare dermatoarthropathy characterized by multiple scar-like or fibromatosis-like nodules and arthralgias. Loss of elastic fibers in the fibroblastic proliferation is characteristic. Presentation as multiple lesions is an important clue to the diagnosis. Correlation with clinical history is critical to avoid misdiagnosis as other entities such as fibromatosis or scar.
Wednesday, March 24, 2010 1:00 PM
Poster Session VI # 106, Wednesday Afternoon