Discussion of Diagnosis
Paraneoplastic syndrome can have rheumatic manifestations; such rheumatic symptoms either mimic the features of other rheumatic disorders or produce distinctive features. The association between malignant disease and rheumatic symptoms has been described for some time, and such paraneoplastic syndromes can affect a variety of organ systems. Palmar fasciitis and polyarthritis syndrome is an uncommon paraneoplastic presentation that was first described in 1982 by Medsger and colleagues, who reported the association of palmar fasciitis and polyarthritis syndrome with ovarian carcinoma, and described these symptoms as a variant of reflex sympathetic dystrophy (now known as complex regional pain syndrome). More than 40 cases have since been reported, and the majority of patients are female. Although originally described in association with ovarian carcinoma, some case reports link this form of arthritis with colon, pancreas, lung, cervix and hematolymphatic malignancies.[6,7]
The rheumatic symptoms usually precede the occurrence or detection of a tumor, but can also occur simultaneously with, or follow, the diagnosis of neoplasm. By definition, a paraneoplastic syndrome is not produced by the primary tumor itself, or by its metastases, and it is not caused by compression or treatment of the tumor. The underlying mechanism for palmar fasciitis and polyarthritis syndrome has not yet been defined, but it is likely to have an association with fibrogenic and connective tissue growth factors and autoimmune reactions, since immunoglobulin deposits have been found in the fascial tissues.[4,5]
The literature describes the clinical picture of palmar fasciitis and polyarthritis syndrome as presenting with pain and diffuse synovitis of the hands (usually at the MCP and PIP joints), and rapid progression of palmar fasciitis with flexion contractures in the hands and limbs. Although adhesive capsulitis is also common, and symptoms often involve multiple joints, plantar fasciitis rarely manifests. Laboratory investigations, such as tests for acute phase reactants and the presence of autoantibodies, generally provide unremarkable results. Palmar fasciitis and polyarthritis syndrome is a nonerosive form of arthopathy, and, except for some periarticular demineralization, X-rays of joints usually show no abnormalities.
Palmar fasciitis and polyarthritis syndrome can mimic other conditions, including scleroderma, complex regional pain syndrome (reflex sympathetic dystrophy), Dupuytren's disease and eosinophilic fasciitis, and there are also case reports that contain tentative diagnoses of clinical rheumatoid arthritis ( Box 1 ). The absence of Raynaud's syndrome, the lack of specific autoantibodies and the rapid progression of clinical features help exclude scleroderma; the synovitis and indurated swelling of digits, and the rapid progression of clinical features of the hand, make Dupuytren's disease an unlikely diagnosis. In contrast to complex regional pain syndrome, palmar fasciitis and polyarthritis syndrome almost always presents with bilateral inflammatory arthritis and fasciitis that is not localized to a particular limb, and usually has a more severe presentation.
In the current patient, no evidence of persistent malignant disease has been detected using conventional screening methods (PET scanning was unavailable). The paraneoplastic nature of the patient's disorder is, therefore, hypothetical. The case is regarded as a paraneoplastic presentation, however, since the history of extensive malignancy was diagnosed before presentation of rheumatic symptoms and peripancreatic lymph nodes were positive during tumor resection. Moreover, pancreatic carcinomas tend to develop early subclinical metastases, and parapancreatic lymph node morphology, determined on CT, is not a useful predictor of malignancy.
Most case reports have described the association of the clinical spectrum of palmar fasciitis and polyarthritis syndrome with different malignancies; however, three previous case studies reported palmar fasciitis and polyarthritis syndrome that was not associated with detectable neoplasm. Seaman et al. reported seven patients who developed palmar fasciitis while receiving an antituberculosis drug (ethionamide); the palmar fasciitis resolved when the treatment was discontinued. Laszlo et al. described a 75-year-old woman who developed palmar fasciitis and polyarthritis syndrome; this syndrome was described as idiopathic, and the duration of follow-up was only 12 months. Finally, Sung and colleagues reported a case of a 31-year-old Korean woman; her palmar fasciitis and polyarthritis syndrome was not found to be associated with malignancy during the 24-month follow-up period from the onset of her first symptoms.
Nat Clin Pract Rheumatol. 2008;4(5):274-277. © 2008
Nature Publishing Group
Cite this: A Paraneoplastic Case of Palmar Fasciitis and Polyarthritis Syndrome - Medscape - May 01, 2008.