|IMAGES IN RHEUMATOLOGY
|Year : 2018 | Volume
| Issue : 2 | Page : 129-130
Palmar fasciitis and polyarthritis as a paraneoplastic syndrome
Prasanta Padhan, Swati Mishra
Department of Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India
|Date of Web Publication||24-May-2018|
Dr. Prasanta Padhan
Department of Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar - 751 024, Odisha
Source of Support: None, Conflict of Interest: None
Keywords: Breast carcinoma, palmar fasciitis, polyarthritis
|How to cite this article:|
Padhan P, Mishra S. Palmar fasciitis and polyarthritis as a paraneoplastic syndrome. Indian J Rheumatol 2018;13:129-30
Rheumatologists can be faced with a number of paraneoplastic syndromes such as palmar fasciitis with polyarthritis, remitting seronegative symmetrical synovitis with pitting edema, paraneoplastic polymyalgia rheumatica, hypertrophic osteoarthropathy, dermatomyositis, and paraneoplastic vasculitis. The paraneoplastic syndrome can precede the diagnosis of cancer, occur simultaneously, or as in this case be a sign of disease recurrence.
A 68-year-old female presented to us in February 2016 with complaints of bilateral hands deformities with predominant finger involvement. She reported progressive stiffening and pain in both hands with palmar swelling for 3 months. She had been diagnosed with invasive ductal carcinoma of the right breast in 2006 and was treated with surgery, chemotherapy, and radiation. It was found to be estrogen receptor, progesterone receptor, and HER2/neu negative. During her current visit, she had local recurrence and metastasis to local lymph nodes which was confirmed on biopsy.
On physical examination, the fingers of both the hands were in flexion contracture, darkly pigmented with swelling of the palmar fascia otherwise described as “woody hands” [Figure 1]a. All kinds of movements of both the hands were restricted. There was pain on passive stretching of hand joints. All the laboratory parameters for connective tissue disorders were negative. Hand radiographs revealed erosive arthritis of metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints with marked deformities [Figure 1]b. The patient was treated with a short course of oral steroids and physiotherapy with minimal improvements in hand functions. She was advised for further treatment of metastasis and subsequent follow-up.
|Figure 1: (a) Fingers of both the hands showing flexion contracture, darkly pigmented with swelling of the palmar fascia. (b) Hand radiographs posteroanterior view showing erosions in metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints with marked deformities|
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| Discussion|| |
Our patient has been diagnosed to have paraneoplastic syndrome-associated palmar fasciitis and polyarthritis as of breast carcinoma recurrence. Palmar fasciitis and polyarthritis was first identified as a rare paraneoplastic syndrome in 1982 by Medsger et al. The syndrome is most commonly described with ovarian cancer, although other malignancies including pancreatic, gastric, prostate, lung, breast, and uterine have been reported. They may either precede or occur along with the underlying malignancy. The mechanisms are presumed to involve activation of transforming growth factor beta or connective tissue growth factors resulting in increased fibrotic activities primarily involving the hands, though the exact pathology remains obscured. Differentials include systemic sclerosis, Dupuytren's disease, eosinophilic fasciitis, or a complex regional pain syndrome (CRPS). The absence of Raynaud's phenomenon, telangiectasia, calcinosis, or nailbed vasculopathy with negative autoimmune serology would go against a diagnosis of systemic sclerosis.
Dupuytren's disease would not be associated with an inflammatory polyarthritis and not commonly be as rapidly progressive. CRPS would typically be unilateral in nature, have associated vasomotor features, and often occur following a localized insult. Eosinophilic fasciitis is often accompanied by blood eosinophilia and infrequently affects the hands. The syndrome typically responds poorly to anti-inflammatory and steroids. Antirheumatic drugs, including methotrexate, hydroxychloroquine, sulfasalazine, and antitumor necrosis factor, have been used with disappointing results. Treatment of the underlying malignancy has been effective for the inflammatory symptoms, although the palmar contractures usually persist.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Medsger TA, Dixon JA, Garwood VF. Palmar fasciitis and polyarthritis associated with ovarian carcinoma. Ann Intern Med 1982;96:424-31.
Azar L, Khasnis A. Paraneoplastic rheumatologic syndromes. Curr Opin Rheumatol 2013;25:44-9.
Haroon M, Phelan M. A paraneoplastic case of palmar fasciitis and polyarthritis syndrome. Nat Clin Pract Rheumatol 2008;4:274-7.