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
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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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Last Reviewed:08/30/2021
Last Updated:08/30/2021
Last Updated:08/30/2021
Palmar fasciitis and polyarthritis syndrome