Palmar fasciitis and polyarthritis syndrome M72.-

Palmar fasciitis with polyarthritis syndrome, PFPAS for short, is a rare (paraneoplastic?) disease (Celen H et al. 2021) characterized by diffuse inflammation of the palmar fascia, the tendon sheaths and the joints of the fingers and wrists, which rapidly leads to a flexion contracture of the hands (woody hands). The clinical course is analogous to Dupuytren's disease.1

The cause is unknown.

A paraneoplastic constellation is frequently associated with this syndrome (Celen H et al. 2021).

Palmar fasciitis with polyarthritis syndrome was originally associated with ovarian carcinoma (> 50% of patients). However, it has now also been associated with several other malignancies, e.g. adenocarcinoma of the lung and non-small cell carcinoma of the lung (Okumura H et al. 2022; Manger B et al. 2014; Sheehy C et al. 2007).

Case report: 77-year-old man with metastatic adenocarcinoma of the lungs receiving chemotherapy developed rapidly increasing symmetric flexion contractures associated with palmar fascial thickening and arthritis in both hands and shoulders.

Therapeutically, salazosulfapyridine was not effective. Significant improvement of inflammatory symptoms was obtained with 15 mg/day prednisolone.

1. Celen H et al. (2021) Palmar fasciitis and polyarthritis: an uncommon but remarkable paraneoplastic syndrome. Clin Rheumatol 40: 2507-2508.
2. Manger B et al. (2014) Palmar fasciitis and polyarthritis syndrome-Systematic literature review of 100 cases. Seminars in Arthritis and Rheumatism 44: 105-111
3. Okumura H et al. (2022) Palmar Fasciitis and Polyarthritis Syndrome Associated with Lung Adenocarcinoma. Intern Med 61:2221-2225.
4. Sheehy C et al. (2007) Palmar fasciitis and polyarthritis syndrome associated with non-small-cell lung carcinoma. Clin Rheumatol26:1951-1953.
5. van Marcke C et al. (2017) Palmar fasciitis and polyarthritis, a rare paraneoplastic syndrome related to ovarian cancer. Clin Exp Dermatol 42:328-330.