Palmar fasciitis and polyarthritis syndrome (PFPAS) is a rare paraneoplastic dermatoarthropathy characterized by the sudden onset of multifocal joint pain, erythema, and palmar fascial thickening and nodularity, predominantly affecting the hands. PFPAS most commonly affects women in the fifth to seventh decades and is almost always associated with an underlying malignancy, most commonly ovarian cancer, followed by pancreatic, gastrointestinal, breast, and lung cancers. Although the exact etiology is unknown, the production of growth factors involved in fibroblastic proliferation by the malignancy, such as TGF-beta, has been reported.
The condition can rapidly progress to a debilitating arthropathy with irreversible flexion contractures of the hands within months. Cutaneous and rheumatologic manifestations typically precede the onset of systemic signs of malignancy by about 6 and 9 months respectively. If onset of PFPAS occurs after a diagnosis of underlying malignancy has been established, it may be a sign of disease progression. Rare cases of idiopathic PFPAS in the absence of internal malignancy have been reported.
Palmar fasciitis and polyarthritis syndrome
Alerts and Notices
Synopsis
Codes
ICD10CM:
M13.849 – Other specified arthritis, unspecified hand
M72.0 – Palmar fascial fibromatosis
SNOMEDCT:
49783001 – Paraneoplastic syndrome
M13.849 – Other specified arthritis, unspecified hand
M72.0 – Palmar fascial fibromatosis
SNOMEDCT:
49783001 – Paraneoplastic syndrome
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Differential Diagnosis & Pitfalls
- Dupuytren contracture – Typically, more indolent in onset, occurring over years. Has a higher prevalence in Caucasian men, as opposed to women predominantly affected in cases of PFPAS. Dupuytren contracture is associated with a history of alcoholism, diabetes, or smoking, as opposed to an underlying malignancy.
- Scleroderma – Nonspecific inflammatory markers and more specific serologic markers (ie, anti-Scl-70, anticentromere) are typically elevated in scleroderma. Additional clinical features including Raynaud phenomenon, more diffuse cutaneous involvement, sclerodactyly, calcinosis cutis, and nail changes can be seen in scleroderma and are typically absent in PFPAS. Additionally, fibroblastic proliferation would be absent on histopathology in scleroderma.
- Rheumatoid arthritis (RA) – Although RA also predominantly involves the MCP and PIP joints, concomitant palmar fasciitis would not be seen, and serology is often positive for rheumatoid factor (RF) and/or anti-cyclic citrullinated peptide (anti-CCP) antibodies. Subcutaneous nodules in RA are characteristically overlying bony prominences. Histopathologic findings of palisading granulomas surrounding well-demarcated areas of fibrinoid necrosis and degenerated collagen are characteristic.
- Fibroblastic rheumatism (FR) – FR is also characterized by sudden onset of cutaneous nodules and symmetric polyarthritis, with histopathologic evidence of dermal fibrosis with prominent fibroblastic proliferation. However, FR can be differentiated from PFPAS based on histopathologic evidence of loss of dermal elastin fibrils, which can be elucidated via Verhoeff-Van Gieson stain.
- Fibromatosis – Can also be associated with palmar fascial thickening, arthropathy, and flexion contractures. Fibroblasts arranged in long fascicles embedded within a dense collagenous stroma with prominent vascular spaces and periadnexal involvement would be seen on histology.
- Diabetic cheiroarthropathy
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References
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Last Reviewed:08/30/2021
Last Updated:10/20/2021
Last Updated:10/20/2021