J Korean Med Sci.  2006 Dec;21(6):1128-1132. 10.3346/jkms.2006.21.6.1128.

Idiopathic Palmar Fasciitis with Polyarthritis Syndrome

• Sung YK
• Park MH
• Yoo DH

• KMID: 1713134
• DOI: http://doi.org/10.3346/jkms.2006.21.6.1128

Abstract

A 31-yr-old Korean woman was presented with 4-month history of bilateral hand swelling and stiffness. On clinical examination, she had a painful synovitis of both hands, wrists, knees and ankles. The radiologic and histological examinations confirmed it with palmar fasciitis and polyarthritis syndrome (PFPAS). PFPAS is an uncommon disorder characterized by progressive flexion contractures of both hands, inflammatory fasciitiis, fibrosis, and a generalized inflammatory arthritis. Although most reported cases of PFPAS have been associated with various malignancies, our patient have not been associated with malignancy during 24 months follow up period from her first symptom onset. Her symptoms were improved with moderate dose of corticosteroid and she is currently taking prednisone 5 mg daily without any evidence for internal malignancy. We present here in a young Korean patient with idiopathic PFPAS who was successfully treated with administration of corticosteroid.

Keyword

Fasciitis; Arthritis; Malignancy

MeSH Terms

Synovitis/*diagnosis
Syndrome
Humans
Hand/*pathology
Female
Fasciitis/*diagnosis
Arthritis/*diagnosis
Adult

Figure

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  Fig. 1 Hands of the patient. It shows the digital flexion contractures and the thickened palmar fascia. The MCP, and PIP joints were tender, and swollen.

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  Fig. 2 T1WI gadolinium enhanced MRI of the left forearm. It shows diffuse enhancements in the fascia, extensor and flexor tendons and some muscles.

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  Fig. 3 Biopsy finding. The fascia is thickened and fibrotic with perivascular infiltration of mononuclear cells. The underlying skeletal muscle shows well preserved myocytes, mild interstitial edema and marked interstitial infiltration mainly of lymphocytes. There is no evidence of perifascicular atrophy, necrosis, granuloma or leukocytoclastic vasculitis (H&E stain ×40).

Reference

1. Medsger TA, Dixon JA, Garwood VF. Palmar fasciitis and polyarthritis associated with ovarian carcinoma. Ann Intern Med. 1982. 96:424–431.

2. Baron M. Palmar fasciitis, polyarthritis, and carcinoma. Ann Intern Med. 1982. 97:616.

3. Pfinsgraff J, Buckingham RB, Killian PJ, Keister SR, Brereton WF, Weinblatt ME, George DL, Arnett FC. Palmar fasciitis and arthritis with malignant neoplasms: a paraneoplastic syndrome. Semin Arthritis Rheum. 1986. 16:118–125.

4. Enomoto M, Takemura H, Suzuki M, Yuhara T, Akama T, Yamane K, Sumida T. Palmar fasciitis and polyarthritis associated with gastric carcinoma: complete resolution after total gastrectomy. Intern Med. 2000. 39:754–757.

5. Laszlo KS, Falanga V, Kerdel FA. Idiopathic palmar fasciitis. Int J Dermatol. 1995. 34:658–660.

6. Seaman JM, Goble M, Madsen L, Steigerwald JC. Fasciitis and polyarthritis during antituberculous therapy. Arthritis Rheum. 1985. 28:1179–1184.

7. Ihn H. Pathogenesis of fibrosis: role of TGF-beta and CTGF. Curr Opin Rheumatol. 2002. 14:681–685.

8. Baer AN, Phillips RM Jr. Pancreatic carcinoma and palmar fasciitis. Ann Intern Med. 1983. 99:411–412.

9. Shiel WC Jr, Prete PE, Jason M, Andrews BS. Palmar fasciitis and arthritis with ovarian and non-ovarian carcinomas. New syndrome. Am J Med. 1985. 79:640–644.

10. Valverde-Garcia J, Juanola-Roura X, Ruiz-Martin JM, Nolla-Sole JM, Rodriguez-Moreno J, Roig-Escofet D. Paraneoplastic palmar fasciitis-polyarthritis syndrome associated with breast cancer. J Rheumatol. 1987. 14:1207–1209.

11. Caron P, Lassoued S, Thibaut I, Fournie B, Fournie A. Thyroid plasmacytoma with dermatomyositis and palmar fasciitis. J Rheumatol. 1989. 16:997–999.

12. Willemse PH, Mulder NH, van de Tempel HJ, Aalders JG, Sleijfer DT. Palmar fasciitis and arthritis in a patient with an extraovarian adenocarcinoma of the coelomic epithelium. Ann Rheum Dis. 1991. 50:53–54.

13. Van den Bergh L, Vanneste SB, Knockaert DC. Palmar fasciitis and arthritis associated with cancer of the prostate. Acta Clin Belg. 1991. 46:106–110.

14. Strobel ES, Lacour M, Peter HH. Palmar fascial thickening and contractures of fingers resembling arthritis-a paraneoplastic symptom? Rheumatol Int. 1992. 12:79–80.

15. Leslie BM. Palmar fasciitis and polyarthritis associated with a malignant neoplasm: a paraneoplastic syndrome. Orthopedics. 1992. 15:1436–1439.

16. Vinker S, Dgani R, Lifschitz-Mercer B, Sthoeger ZM, Green L. Palmar fasciitis and polyarthritis associated with ovarian carcinoma in a young patient. A case report and review of the literature. Clin Rheumatol. 1996. 15:495–497.

17. Dhote R, Beuzeboc P, Permal S, Poiraudeau S, Revel M, Christoforov B. Paraneoplastic palmar fasciitis and achalasia-like esophageal disorder in a patient with breast cancer. Rev Rhum Engl Ed. 1997. 64:350–352.

18. Saxman SB, Seitz D. Breast cancer associated with palmar fasciitis and arthritis. J Clin Oncol. 1997. 15:3515–3516.

19. Wright GD, Doherty M. Unusual and memorable. Paraneoplastic palmar fasciitis and arthritis syndrome. Ann Rheum Dis. 1997. 56:626.

20. Grados F, Houvenagel E, Cayrolle G, Bellony R, Fardellone P, Sebert JL. Two new cancer locations accompanied with palmar fasciitis and polyarthritis. Rev Rhum Engl Ed. 1998. 65:212–214.

21. Eekhoff EM, van der Lubbe PA, Breedveld FC. Flexion contractures associated with a malignant neoplasm: 'A paraneoplastic syndrome?'. Clin Rheumatol. 1998. 17:157–159.

22. Matteson EL. Groove sign in paraneoplastic palmar fasciitis. J Rheumatol. 1998. 25:2043–2045.

23. Kase H, Aoki Y, Sugaya S, Takakuwa K, Tanaka K. Palmar fasciitis and polyarthritis associated with squamous cell carcinoma of the cervix. Int J Gynecol Cancer. 2000. 10:507–509.

24. Roman S, Tebib J, Scoazec JY, Menard Y, Paliard P, Dumortier J. Palmar fasciitis and paraneoplastic polyarthritis associated with hepatocellular carcinoma. Gastroenterol Clin Biol. 2001. 25:203–204.

25. Docquier Ch, Majois F, Mitine C. Palmar fasciitis and arthritis: association with endometrial adenocarcinoma. Clin Rheumatol. 2002. 21:63–65.

26. Virik K, Lynch KP, Harper P. Gastroesophageal cancer, palmar fasciitis and a matrix metalloproteinase inhibitor. Intern Med J. 2002. 32:50–51.

27. Rammeh N, Elleuch M, Meddeb N, Sahli H, Cheour E, Sellami S. Palmar-plantar fasciitis and multiple myeloma. Tunis Med. 2003. 81:889–893.

28. Martorell EA, Murray PM, Peterson JJ, Menke DM, Calamia KT. Palmar fasciitis and arthritis syndrome associated with metastatic ovarian carcinoma: a report of four cases. J Hand Surg. 2004. 29:654–660.

29. Denschlag D, Riener E, Vaith P, Tempfer C, Keck C. Palmar fasciitis and polyarthritis as a paraneoplastic syndrome associated with tubal carcinoma: a case report. Ann Rheum Dis. 2004. 63:1177–1178.

30. Giannakopoulos ChK, Kyriakidou GK, Toufexi GE. Palmar fasciitis and polyarthritis associated with secondary ovarian carcinoma. Case report. Eur J Gynaecol Oncol. 2005. 26:339–341.

31. Kozin F. Reflex sympathetic dystrophy syndrome: a review. Clin Exp Rheumatol. 1992. 10:401–409.

32. Rayan GM. Clinical presentation and types of Dupuytren's disease. Hand Clin. 1999. 15:87–96.

33. Doyle JA, Ginsburg WW. Eosinophilic fasciitis. Med Clin North Am. 1989. 73:1157–1166.