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A Handy Clue: Palmar Fasciitis and Polyarthritis Syndrome

Published:November 20, 2013DOI:https://doi.org/10.1016/j.amjmed.2013.09.021

      Presentation

      Rheumatologic symptoms signaled a deadly underlying disorder. A 48-year-old woman presented with progressive pain and functional impairment of both hands. She was in her usual state of good health until 3 months prior to admission, when she developed pain and stiffness associated with swelling of the proximal and distal interphalangeal joints. Her joint symptoms were more pronounced in the morning, improving over the course of the day. Yet, difficulty flexing and extending all fingers continued, significantly compromising her ability to carry out routine daily activities. Meloxicam was prescribed but offered little relief. Treatment with hydroxychloroquine and a brief prednisone taper followed, and while this transiently helped resolve some of the articular swelling, stiffness, and pain, it did not alleviate the impaired finger dexterity.
      The patient also reported that a 13-pound unintentional weight loss had occurred with the onset of her joint symptoms. She had a poor appetite, dyspepsia, and occasional episodes of facial flushing, but no dysphagia, Raynaud's phenomenon, or prior history of skin or joint disorders. Her past medical history was significant for hypertension since she was 35-years-old. She also had a 30-pack-year history of cigarette smoking. Her family history was notable for lung cancer, colon cancer, and ovarian cancer.

      Assessment

      On the day of admission, the patient presented to the emergency department with a mildly productive cough, rhinorrhea, and lightheadedness. She had a blood pressure of 67/38 mm Hg and a temperature of 101.3° F (38.5° C). Her face and neck were flushed, but she had no malar rash, periorbital heliotrope rash, or lymphadenopathy. Breath sounds were diminished over the left lung base with dullness to percussion. She had diffuse erythema and swelling of the proximal and distal interphalangeal joints of both hands (Figure 1). Flexion contractures limited active and passive finger range-of-motion. Moreover, prominent thickening of the palmar fascia was noted along the thenar eminence, hypothenar eminence, and across the center of the palm, bilaterally (Figure 2). No sclerodactyly, clubbing, digital pitting, telangiectasia, or proximal muscle weakness was evident.
      Figure thumbnail gr1
      Figure 1A dorsal view shows diffuse erythema and swelling of the proximal and distal interphalangeal joints on each hand.
      Figure thumbnail gr2
      Figure 2Digital contractures and thickening of the palmar fascia along the thenar eminence, hypothenar eminence, and across the center of the palm, bilaterally, are evident in this palmar view of the hands.
      Laboratory evaluation demonstrated leukocytosis with 35.2 x 103cells/mm3 but no eosinophilia. The patient's hemoglobin level was 12.6 g/dL, and platelets were 617,000 cells/mm3. Hyponatremia, with a serum sodium level of 128 mEq/L, was present. Renal, liver, and thyroid function tests were normal. Testing with a urine dipstick showed 1+ proteinuria and large-level positivity for leukocyte esterase. Urinalysis identified 6-10 white blood cells/hpf and 0-5 red blood cells/hpf.
      Serology for antinuclear antibodies (ANAs) was positive at a low titer value of 1:80. Tests for rheumatoid factor and anti-cyclic citrullinated peptide (CCP) antibodies were negative.

      Diagnosis

      The history and examination findings pointed to palmar fasciitis and polyarthritis syndrome, a diagnosis that prompted a search for an occult neoplasm. Computed tomography (CT) of the chest showed a left hilar mass with endobronchial extension and consequent occlusion of the left lower lobe bronchus and postobstructive pneumonia. Bilateral adrenal masses were seen as well, along with lytic lesions affecting both acetabula. Next, a transbronchial fine-needle aspiration biopsy of the left mainstem bronchus revealed a poorly differentiated non-small-cell lung cancer (Figures 3 and 4). The pathology and CT imaging results suggested a stage IV non-small-cell lung cancer with an extensive metastatic burden.
      Figure thumbnail gr3
      Figure 3Abnormal cells were identified during transbronchial fine-needle aspiration biopsy of the left mainstem bronchus. Cells revealed hyperchromatic nuclei with prominent nucleoli, an irregular nuclear membrane, scant cytoplasm, and high nuclei:cytoplasm ratios (Diff-Quick staining, 40x).
      Figure thumbnail gr4
      Figure 4A core biopsy demonstrated a poorly-differentiated carcinoma without glandular formation or keratinization characteristic of adenocarcinoma and squamous cell carcinoma, respectively (hematoxylin and eosin stain, 40x).
      Palmar fasciitis and polyarthritis syndrome is a rare paraneoplastic disorder first reported in 1982 in association with ovarian carcinoma.
      • Medsger T.A.
      • Dixon J.A.
      • Garwood V.F.
      Palmar fasciitis and polyarthritis associated with ovarian carcinoma.
      It has since been associated with malignancies of the lung, pancreas, bladder, kidney, colon, breast, prostate, endometrium, cervix, fallopian tube, stomach, and liver, as well as with hematologic malignancies, including chronic myelogenous leukemia, multiple myeloma, and Hodgkin’s lymphoma.
      • Shiel Jr., W.C.
      • Prete P.E.
      • Jason M.
      • Andrews B.S.
      Palmar fasciitis and arthritis with ovarian and non-ovarian carcinomas. new syndrome.
      • Sheehy C.
      • Ryan J.G.
      • Kelly M.
      • Barry M.
      Palmar fasciitis and polyarthritis syndrome associated with non-small-cell lung carcinoma.
      • McGivern U.
      • McAleese J.
      Palmar fasciitis: A para-neoplastic phenomenon indicating recurrence of non small cell lung cancer—case report and review of the literature.
      • Pfinsgraff J.
      • Buckingham R.B.
      • Killian P.J.
      • et al.
      Palmar fasciitis and arthritis with malignant neoplasms: A paraneoplastic syndrome.
      • Sung Y.K.
      • Park M.H.
      • Yoo D.H.
      Idiopathic palmar fasciitis with polyarthritis syndrome.
      Interestingly, a similar disorder which may present with fasciitis of the palms and feet, distal symmetric polyarthritis, digital flexion contractures, and shoulder contractures, has been described with nonmalignant processes including teratoma, paraovarian cyst, and recent antituberculosis therapy with ethionamide.
      • Fernandez-Nebro A.
      • Fernandez-Ruiz A.
      • Rodriguez M.
      • Haro M.
      • Rodriguez-Andreu J.
      • Gonzalez-Santos P.
      Palmar fasciitis and polyarthritis syndrome is multicausal.
      • Seaman J.M.
      • Goble M.
      • Madsen L.
      • Steigerwald J.C.
      Fasciitis and polyarthritis during antituberculous therapy.
      Palmar fasciitis predominately affects women, with rheumatologic symptoms often triggering the detection of an otherwise occult malignancy, as in our patient's case.
      The differential diagnosis includes scleroderma, rheumatoid arthritis, reflex sympathetic dystrophy, Dupuytren's contracture, diabetic cheiroarthropathy, and eosinophilic fasciitis.
      • Sheehy C.
      • Ryan J.G.
      • Kelly M.
      • Barry M.
      Palmar fasciitis and polyarthritis syndrome associated with non-small-cell lung carcinoma.
      • McGivern U.
      • McAleese J.
      Palmar fasciitis: A para-neoplastic phenomenon indicating recurrence of non small cell lung cancer—case report and review of the literature.
      • Pfinsgraff J.
      • Buckingham R.B.
      • Killian P.J.
      • et al.
      Palmar fasciitis and arthritis with malignant neoplasms: A paraneoplastic syndrome.
      • Sung Y.K.
      • Park M.H.
      • Yoo D.H.
      Idiopathic palmar fasciitis with polyarthritis syndrome.
      • Qureshi A.A.
      • Saavedra A.
      Palmar fasciitis and polyarthritis syndrome in patients with ovarian cancer-a case report and review of the literature.
      Our patient lacked the digital cold sensitivity characteristic of Raynaud's phenomenon, which is almost universally present among patients with systemic sclerosis/scleroderma. Its absence, combined with the low titer of ANAs, diminished the probability of scleroderma. Seronegativity for both rheumatoid factor and anti-CCP antibodies argued against rheumatoid arthritis, as did the prominent involvement of the distal interphalangeal joints.
      Reflex sympathetic dystrophy, characteristically seen in 1 extremity, is marked by vasomotor disturbances and slower progression, and it often follows a traumatic injury or acute medical event. These traits made it a poor fit for the described course. Classically, Dupuytren's contracture presents insidiously with nodules and flexor tendon thickening along the ulnar side of the hand; male predominance also is common. Persons with longstanding and poorly controlled insulin-dependent diabetes mellitus may develop thickening of the digits (diabetic cheiroarthropathy), and palmar flexion contractures, producing a prayer sign, might ensue. Common presenting symptoms of eosinophilic fasciitis are myalgia and forearm thickening that contributes to palmar and finger flexion contractures; peripheral eosinophilia, absent in our patient, is a frequent sign.
      Diagnostically, a skin biopsy among patients with palmar fasciitis might reveal dense connective tissue, inflammatory infiltrate, and immunoglobulin deposition, but these findings are not indispensable when establishing a diagnosis.
      • Shiel Jr., W.C.
      • Prete P.E.
      • Jason M.
      • Andrews B.S.
      Palmar fasciitis and arthritis with ovarian and non-ovarian carcinomas. new syndrome.
      • Sung Y.K.
      • Park M.H.
      • Yoo D.H.
      Idiopathic palmar fasciitis with polyarthritis syndrome.
      • Qureshi A.A.
      • Saavedra A.
      Palmar fasciitis and polyarthritis syndrome in patients with ovarian cancer-a case report and review of the literature.
      Likewise, plain radiographs and magnetic resonance imaging of the hands provide limited additional diagnostic information beyond what the clinical picture affords.
      To date, a definitive pathogenic mechanism for onset of palmar fasciitis and polyarthritis syndrome has not been elucidated. Proposed explanations include the presence of a fibroblast proliferative factor, alterations in hormonal state, and activation of profibrotic factors, such as transforming growth factor-β or connective tissue growth factor.
      • Sung Y.K.
      • Park M.H.
      • Yoo D.H.
      Idiopathic palmar fasciitis with polyarthritis syndrome.
      Generally, the condition has poor prognostic implications, as it is often associated with metastatic malignancies.
      • Shiel Jr., W.C.
      • Prete P.E.
      • Jason M.
      • Andrews B.S.
      Palmar fasciitis and arthritis with ovarian and non-ovarian carcinomas. new syndrome.
      Various case reports have documented heterogeneous responses to treatments, which often involve corticosteroids and chemotherapy targeted at the underlying malignancy.
      • Sung Y.K.
      • Park M.H.
      • Yoo D.H.
      Idiopathic palmar fasciitis with polyarthritis syndrome.
      Although rare, palmar fasciitis and polyarthritis syndrome is important to consider when evaluating a patient with thickened hands, digital and palmar contractures, and a symmetric inflammatory polyarthritis, particularly in the absence of both Raynaud's phenomenon and ANA seropositivity. This combination of features, atypical for more common entities like lupus, scleroderma, and rheumatoid arthritis, should raise suspicion for an underlying malignancy, especially when it occurs in conjunction with weight loss and notable risk factors.

      Management

      During hospitalization, the patient was given intravenous fluids and broad-spectrum antibiotics. As a result, her temperature and blood pressure subsequently normalized. Hydroxychloroquine and prednisone were discontinued. After discharge, she was followed in the oncology clinic, where over the subsequent month, she was started on palliative chemotherapy with cisplatin and pemetrexed for stage IV non-small-cell carcinoma. Her palmar fasciitis and polyarthritis, however, persisted.

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