Fibroblastic rheumatism: a Scandinavian case report.

CASE REPORT A 55 year old Danish woman was referred to our department in July 2000 with a 2K year history of pain in the proximal interphalangeal (PIP) joints, knees, and ankles. The pain worsened over night and upon exercise. On examination, the right knee and the second PIP joint on the left hand were tender and swollen. The other PIP joints and both wrists were tender. On both hands there were several pink, 3–10 mm, tender and mobile skin nodules (fig 1), and a 20 mm nodule under the left foot. All laboratory investigations were normal, including erythrocyte sedimentation rate, C reactive protein, haemoglobin, white blood cell count, platelets, and differential count. IgM rheumatoid factor, antinuclear antibodies and antineutrophil cytoplasmic antibodies were not found. An x ray examination of the hands demonstrated osteoarthritis of the distal interphalangeal joints. Two skin nodules were removed and examined by light microscopy (fig 2): they showed areas with densely packed uniform spindle cells surrounded by bundles of thickened collagen fibres and vascular granulation tissue. The biopsy specimens were without inflammatory cells, histiocytes, giant cells, granulomas, or foreign bodies. Special staining did not disclose any elastic fibres. Immunochemical staining of paraffin sections for myofibroblasts was positive for vimentin, but negative for desmin and a-smooth muscle actin. Treatment with celecoxib and physical therapy was started but had no effect on the symptoms. On follow up in 2003, the patient had severe, intermittent arthralgia and she was unable to work. She had a nodular thickening of the palmar aponeurosis and a slight contracture of the fingers on both hands. x Ray findings of her hands had not changed.