Sir: A 45-year-old African American man presented with a 10-year history of progressively deteriorating bilateral flexion contracture at the proximal interphalangeal joints of the middle, ring, and small fingers, and inability to fully extend his left elbow. The contracture was associated with arthralgias, initially of the small joints of the hand, but later involving knees, elbow, and ankles. Progressive disease caused loss of fine coordinated finger movements, as fixed flexion contractures developed (Fig. 1). The patient worked as a jet fuel loader in the navy, during which he sustained a significant occupation-related exposure to JP-5 and/or JP-8 jet fuel, including one episode in which he was drenched with fuel.Fig. 1.: Bilateral flexion contractures of the proximal interphalangeal joints of the middle, ring, and small fingers.The patient denied history of swelling at the wrists or knuckles, palmar thickening, tenderness, or sensory loss. Results for rheumatoid factor, anti-SSA, anti-SSB, anti-SM, anti-Jo1, anti-DS DNA, antinuclear antibody, anti–ribonucleic protein, serum and urine mercury were negative. Serum protein electrophoresis was normal. Screening for tumor markers CA 19-9, CA 27.29, CA 125, CA 15-3, CH50, and α-fetoprotein were negative, as was testing for occult blood. Plain films revealed calcification in soft tissues adjacent to the first interphalangeal joint bilaterally. Computed tomographic scan of the abdomen and pelvis was normal. Magnetic resonance imaging scan of the left hand showed erosive changes and synovial proliferation in the entire carpus but no osteophytes. Electromyographic studies revealed mild diffuse demyelization motor neuropathy without evidence of axonal injury. Right median motor study showed normal conduction velocity and distal latency. Ulnar motor studies showed decreased conduction velocity in the right arm and decreased conduction velocity in the distal segment in the left arm. Examination of biopsy specimens of the hand skin revealed fibrosis around neurovascular structures and markedly thickened perineurium. A unique pathologic feature was the presence of triangularly shaped nerve trunks (Fig. 2). It was concluded that this was a previously uncharacterized musculoskeletal disorder. The patient was treated symptomatically using nonsteroidal antiinflammatory drugs and acetaminophen.Fig. 2.: Hematoxylin and eosin staining demonstrates thickened perineurium and triangularly shaped nerve trunks.Our differential diagnosis included Dupuytren disease, scleroderma, immune arthropathies, gadolinium-induced nephrogenic fibrosing dermopathy, palmar fasciitis and polyarthritis syndrome, and hand contracture as a manifestation of a paraneoplastic syndrome. Physical examination revealed soft, supple skin in our patient's hand without nodules or cords, ruling out the diagnosis of Dupuytren disease. Nephrogenic fibrosing dermopathy1 was unlikely given our patient's normal renal function. Palmar fasciitis and polyarthritis syndrome2,3 was unlikely because of a lack of inflammation and fascial involvement. Lack of inflammation on biopsy and negative serum markers ruled out scleroderma. A comprehensive workup revealed no primary tumor in our patient, ruling out paraneoplastic syndrome as a cause of contracture.4 Given a comprehensive negative workup and known occupational exposure to jet fuel (JP5 or JP-8), we conclude that our patient developed a new form of hand contracture secondary to this exposure. Studies in rats investigating effects of JP-8 exposure further support our hypothesis that this syndrome resulted from an occupational exposure. These studies revealed that rear claw contractures developed in rats after moderate exposure to JP-8 for 1 hour per day for 28 days.5 This is the first case reported in humans, and we believe this represents a new clinical syndrome. Christopher R. Spock, B.A. Quratulain Masood, M.B.B.S. Shawn E. Cowper, M.D. Deepak Narayan, M.D. Department of Plastic and Reconstructive Surgery Yale University School of Medicine New Haven, Conn.
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Dec 9, 2009
Kian Eisazadeh Far + 2
Open Access