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Rapidly Progressive Stiffness and Nodularity of Both Hands in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her late 40s presented with rapidly progressive redness and stiffness of both hands, accompanied by 2 months of multiple finger joint and right shoulder pain. Results of autoimmune serology, including antinuclear antibody, C3 and C4, rheumatoid factor, and Scl-70 antibody tests, were negative. Raynaud phenomenon was not seen. There was an absence of nailfold capillary changes and distal digital pitting.

The patient was treated with nonsteroidal anti-inflammatory drugs, systemic steroid therapy, and disease-modifying antirheumatic drugs (ie, hydroxychloroquine), but symptoms persisted. Two weeks after treatment initiation, multiple tender nodules developed over the palms and interphalangeal joints, and flexion contractures continued (Figure, A). Incisional skin biopsy of the palmar nodules (Figure, B and C) and computed tomography (CT) were performed. Results of the CT demonstrated bilateral ovarian tumors with highly suspect peritoneal metastases.

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C. Palmar fasciitis and polyarthritis syndrome

Histopathologic examination revealed increased fibroblasts with fibrosis from dermis to subcutis (Figure, B and C). The fascia was not included in the biopsy specimen.

For the ovarian cancer, the patient received neoadjuvant chemotherapy with carboplatin and paclitaxel at 3-week intervals for 3 cycles. Results of the follow-up CT showed partial regression in the left ovarian tumor and a stationary right ovarian tumor. The patient underwent debulking surgery with bilateral salpingo-oophorectomy. The pathology of the ovarian tumors demonstrated high-grade serous carcinoma. Regarding her hands, the patient reported partial resolution of swelling, pain, and nodularity, but no improvement in flexion contractures.

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Article Information

Corresponding Author: Ren-Feng Liu, MD, Department of Dermatology, Chang Gung Memorial Hospital, No. 222, Maijin Rd, Anle Dist, Keelung City, 20401, Taiwan (hiawathahaha@gmail.com).

Published Online: November 11, 2020. doi:10.1001/jamadermatol.2020.4386

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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