Rheumatol Int DOI 10.1007/s00296-009-1017-1
C A S E RE P O RT
Jaccoud’s arthropathy and psoriatic arthritis, a rare association Yumei Wu · Jie Zheng
Received: 12 March 2009 / Accepted: 2 June 2009 © Springer-Verlag 2009
Abstract Jaccoud’s arthropathy is a syndrome of chronic progressive painless deformity of the hands and feet with surprisingly well-preserved functions. Although it is most frequently seen in patients with systemic lupus erythematosus, an association with other diseases has occasionally been described. A patient with long-standing psoriasis arthritis is presented in this report who developed an arthropathy with the clinical and radiologic Wnding indistinguishable from that of the Jaccoud’s arthropathy. In patients with long-standing psoriasis arthritis, the disease may be associated with Jaccoud’s arthropathy. Keywords
Psoriatic arthritis · Jaccoud’s arthropathy
function; it frequently aVects the hands and feet , but rarely the ankles, elbows, shoulders or knees [4, 5]. Ulnar drift with subluxation of the metacarpophalangeal joints is often the Wrst sign, whereas swan neck, boutonniere deformities, and Z deformity may occur at a later stage . Pathological studies have revealed mild synovitis and inXammatory capsular Wbrosis, which might be responsible for the characteristic deformities [3, 7–10]. Radiographic studies generally do not show evidence of bone or cartilage damage . Here, we describe a male patient with longstanding psoriasis arthritis who developed an arthropathy of the clinical and radiologic Wnding indistinguishable from that of the Jaccoud’s arthropathy. This unusual presentation could certainly provide more information to the study of Jaccoud’s arthropathy.
Introduction Jaccoud’s arthropathy was Wrst described in 1896 in patients with long-standing rheumatic fever . Similar articular signs have also been reported in patients with systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, hypocomplementemic vasculitis and systemic necrotizing vasculitis. Isolated cases have been reported in nonautoimmune rheumatic diseases such as keratitis ichthyosis deafness (KID) syndrome, AIDS, mycosis fungoides, angioimmunoblastic lymphadenopathy and Calort syndrome . Jaccoud’s arthropathy is a nonerosive arthropathy, which is characterized by severe deformities with multiple subluxations, little or no pain, and usually well-preserved
Y. Wu · J. Zheng (&) The Department of Dermatology, Ruijin Hospital, Jiao Tong University School of Medicine, Shanghai, China e-mail: [email protected]
Case report A 58-year-old male presented to our clinic in 2007 with a 10-year history of deformity of hands. Thirty years earlier at the age of 28, psoriatic rash developed gradually, involving the scalp, the trunk and extremities with a little itching and the typical character of being worse in winter and better in summer. Physical examination revealed well demarcated, raised, scaly erythematous plaques on scalp, trunk, extremities with Auspitz sign (+). Figure 1 is the pathological manifestations of the skin lesion. He was diagnosed at our Dermatology Department as having psoriasis and was treated with local steroid ointments and UV irradiation. During the last 10 years of follow-up, many episodes of acute synovitis of hands and knees occurred, deformity of hands occurred gradually. Physical examination showed marked bilateral reducible subluxation of the metacarpophalangeal joint with ulnar deviation and was treated with NSAID. Methotrexate was instituted with marked
Fig. 1 Part of metacarpophalangeal and interphalangeal joint dislocation, subluxation no signiWcant joint space narrowing, no osteoporosis
improvement of both the arthritis and the psoriasis rash in 2005. He denied previous joint problems, hypermobility or a family history of rheumatic disorders. Laboratory tests revealed a negative RF, ANA, ENA and HLA B-27. Recent X-ray of the hands showed deformity of joint with no erosive changes (Fig. 2): the metacarpophalangeal joint and the interphalangeal joint of both hands deformities, the bilateral Wrst metacarpophalangeal joint metacarpal Xexion and subluxation; the interphalangeal joint of the thumb dorsiXexion and dislocation; the proximal interphalangeal joint of the right index Wnger dorsiXexion and subluxation, the distal interphalangeal joint metacarpal Xexion and dislocation; the proximal interphalangeal joint of the bilateral middle Wnger varus and subluxation, the distal interphalangeal joint metacarpal Xexion deformities; proximal interphalangeal joint of the bilateral ring Wnger metacarpal Xexion deformity; bilateral Wfth metacarpophalangeal joint dorsiXexion and subluxation, proximal interphalangeal joint metacarpal Xexion and dislocation. There was no obvious joint space narrowing, no osteoporosis, no soft tissue swelling.
Discussion Jaccoud’s arthropathy was Wrst described in 1869 in patients with long-standing rheumatic fever. It is characterized by severe deformities of the hands and feet with multiple subluxations with little or no pain, and usually with well-preserved function. Radiographic studies generally do not show evidence of bone or cartilage damage. Furthermore, this arthropathy has been reported in patients with systemic lupus erythematosus (SLE), hypocomplementemic vasculitis, sarcoidosis and so on, apart from rheumatic fever .
Fig. 2 H&E staining £100: parakeratosis with nuclear dust and neutrophils inWltration, acanthosis; tortuous capillary expansion, erythrocyte extravasation in dermal papilla; the inWltrating mononuclear cells can be seen around blood vessels in superWcial dermal
We formulated the diagnosis of PA (psoriatic arthritis) complicated by Jaccoud’s arthropathy (JA) in this patient. According to the new classiWcation criteria of the PSA (CASPAR)  which was made in 2006, the diagnosis of psoriatic arthritis in our patient was evident since the presence of symptoms of arthritis, the absence of rheumatoid factor in association with psoriasis are usually suYcient grounds for such diagnosis . Although diagnostic criteria for JA have been proposed in the past, they have not been widely accepted. Our diagnosis in this patient was based on the clinical picture of deforming arthritis dominated by articular subluxations without any erosive radiological changes of the joint. Deformities in JA seem to be due to lax joint capsules, tendons and ligaments that cause joint instability . The pathogenesis of JA is uncertain. Many physicians believe ligament laxity and the stretching of joint capsules result from capsular periarticular Wbrosis or synovial vasculitis which were involved in the development of the arthropathy . The pathologic Wndings obtained from joint necropsy in this arthropathy have been reported and the Wbrotic process of the joint capsules is striking . This patient had suVered for a long period from recurrent acute synovitis and tendinitis. A similar mechanism may be hypothesized for this case. There was only one reported case describing JA associated with psoriatic arthritis by Tishler in 1993 . This report does support that psoriatic arthritis may be associated with JA and provide more information to the study of JA. Psoriatic arthritis could be added to the long list of conditions leading to Jaccoud’s arthropathy.
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