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Publication Date
2025
Keywords
oregon, psvmc, oregon gme
Disciplines
Dermatology | Medical Education | Oncology
Abstract
Introduction: Systemic sclerosis is a rare autoimmune disorder defined by skin thickening in the distal digits and is frequently accompanied by inflammatory arthritis. There are known mimics of systemic sclerosis, which present with similar sclerosis of the skin. Palmar fasciitis and polyarthritis syndrome (PFPAS) is a rare paraneoplastic syndrome that presents with progressive flexion contractures of the hands, inflammatory fasciitis, fibrosis, and generalized inflammatory arthritis. PFPAS is most associated with ovarian adenocarcinoma, and rarely associated with pancreatic adenocarcinoma. Early recognition of this rare syndrome was critical in identifying an asymptomatic pancreatic adenocarcinoma. Case Description: 58-year-old woman with osteoarthritis of multiple joints and 40 pack-year smoking history presented to her primary care physician with 2 months of joint pain and swelling in her hands and progressive skin thickening to the skin of the palms and pointer fingers. Stiffness and swelling lasted throughout the day, improved minimally with activity, and did not respond to physical therapy or non-steroidal anti-inflammatories. Examination showed thickening of the palmar skin in the hands with multiple tender subcutaneous nodules. There was also thickening of the right-hand pointer finger. Prescribed a methylprednisolone dose pack and referred to rheumatology. Discussion: Differentiating through History and Exam - Unlike systemic sclerosis PFPAS has no association with Raynaud’s phenomenon and scleroderma-specific antibodies are often negative. PFPAS tends to involve the palms. In this case, the fingers were involved prior to the palms, however, some fingers were spared while others proceeded rapidly to contractures, a pattern not consistent with scleroderma. The patient may have no other signs of systemic sclerosis, such as severe heartburn, matted telangiectasias, or calcinosis cutis. History of significant tobacco use increases the risk of various malignancies, including pancreatic malignancies. Response to Steroids Steroids may not improve skin thickening in systemic scleroderma; however, inflammatory joint pain should improve. It is important to consider underlying malignancy in similar cases of inflammatory polyarthritis when there is minimal response to high-dose steroids. Usefulness in Early Recognition - The bulk of the literature describing PFPAS occurs with ovarian adenocarcinoma, with only a few case reports associated with pancreatic adenocarcinoma. Ovarian and pancreatic malignancies are “silent killers,” which remain asymptomatic until late stages. PFPAS may be the first recognizable finding for patients with these malignancies.
Area of Special Interest
Cancer
Specialty/Research Institute
Graduate Medical Education
Specialty/Research Institute
Oncology
