Rheumatology Advance Access originally published online on March 7, 2006
Rheumatology 2006 45(5):640-641; doi:10.1093/rheumatology/kel048
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LETTER TO THE EDITOR |
Management of tenosynovitis in linear scleroderma
Department of Orthopaedics, Bristol Royal Infirmary, Bristol, UK
Correspondence to: D. Hartwright, Department of Orthopaedics, St Thomas’ Hospital, Lambeth Palace Road, London SE1 7EH UK. E-mail: davehartwright{at}yahoo.com
SIR, Scleroderma is a rare connective tissue disease characterized by fibrosis and microvascular occlusion. The hallmark of scleroderma is tight, thickened skin brought about by excessive collagen deposition in the dermal and subdermal layers. Generalized scleroderma (systemic sclerosis) is associated with internal organ involvement, autoantibodies and a poor prognosis [1–4]. Patients with localized scleroderma also carry antibodies but their projected lifespan is not foreshortened because sclerotic involvement is largely confined to the skin and musculoskeletal systems.
Three variants of localized scleroderma exist: morphoea, generalized morphoea and linear scleroderma [4–7].
In linear scleroderma, sclerotic areas occur in a linear, band-like distribution, often crossing joint lines [3, 4]. The lesions follow the embryological lines of Blaschko. Local inflammation and fibrosis may affect the dermis, connective tissue, muscle and bone, giving rise to arthralgias, tenosynovitis, contracture, undergrowth of the limb, and nodulosis. There may be associated Raynaud's phenomenon and carpal tunnel syndrome, but linear scleroderma usually affects the lower limbs of young females [2–4, 6, 7].
Linear scleroderma may cause substantial disability where one or more limbs are severely affected and there is the potential for marked cosmetic morbidity. Medical management may include non-steroidal anti-inflammatory drugs, penicillamine aimed at skin softening, and camouflage creams.
Local and system corticosteroids, methotrexate and interferon may also play a role [8]. Surgical intervention is rarely indicated and the outcome of surgery must be considered uncertain, given the patient's predisposition to excessive local collagen deposition and that taut skin might significantly impair wound healing.
We present the case of a 19-yr-old, left-handed motor mechanic who complained of a 2-yr history of arthralgia involving the fingers of both hands. There was an ill-defined history of Raynaud's phenomenon, but no symptoms of systemic disease. Examination revealed symmetrical, linear scleroderma arising around the mid-scapula region and radiating down the posterior aspect of the arms to the dorsum of the hands (Fig. 1). Anti-nuclear antibody was present at a titre of 1/160 IgG. Rheumatoid factor, ESR, anti-Scl70, anticentromere antibody and complement levels were normal or negative. The diagnosis of linear scleroderma was made.
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Over the next 7 yr he continued to complain of palmar pain and swelling as well as ‘catching’ of his fingers with straightening, especially in the right hand. This interfered with his ability to grip tools and had a significant impact on his ability to work.
Examination revealed nodularity of the flexor tendons in the right hand proximal to the flexor retinaculum and within the palm, with palpable triggering of all digits as the fingers were actively flexed and extended. Similar findings were found in the left hand to a lesser degree. There were no signs or symptoms of carpal tunnel syndrome. Magnetic resonance imaging (MRI) demonstrated marked thickening around all flexor tendons.
The patient was managed with oral D-penicillamine and local corticosteroid injections, but his principal symptoms persisted unchanged. In view of his continuing intrusive symptoms, tenosynovectomy was considered as a means of reducing symptoms. In the absence of any information in the literature concerning the results of surgery in linear scleroderma, the patient was informed of the possibility of poor wound healing, and of recurrence and excessive fibrotic reaction.
Under a general anaesthetic and with tourniquet control, an approach from the distal forearm to the distal palmar crease was made. The median nerve was protected. There was nodular, fibrotic synovial thickening, most prominent around the profundus tendons. The synovial thickening extended from the musculo-tendinous junction to the entrance of the fibrous flexor sheath distally, confirming the findings on MRI.
A complete flexor tendon synovectomy was performed, combined with release of the A1 pulley of the index and middle finger (Fig. 2). Histological examination of the synovium showed minimal synovitis with marked fibrotic and fibrinoid changes, probably due to scleroderma. Postoperatively, the patient made an excellent recovery with a full range of pain-free movements in all digits of the right hand. At review 2 yr later, there was no evidence of recurrence. Symptoms in the left hand were not sufficient to warrant surgery.
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Carpal tunnel triggering is rare and has not been reported in association with linear scleroderma. There is also no literature on the surgical management of tenosynovitis in this condition.
We present a patient with a symmetrical linear scleroderma suffering with bilateral synovitis and triggering of the flexor tendons of the fingers at the entrance to the carpal tunnel. Having been resistant to medical therapy, this was successfully treated with flexor synovectomy and release of selected A1 pulleys.
The surgical treatment of the synovitis in patients suffering with scleroderma causes some concern in view of the risk of postoperative fibrosis resulting in worsening contractures and deformities.
This case demonstrates that good results may be achieved with surgical management and that prolonged medical treatment may be avoided.
The authors have declared no conflicts of interest.
References
- LeRoy EC, Black C, Fleischmajer R et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988;15:202–5.[Web of Science][Medline]
- Dehen L, Roujeau JC, Cosnes A, Revuz J. Internal involvement in localised scleroderma. Medicine 1994;73:241–5.[Medline]
- Falanga V. Localised scleroderma. Med Clin North Am 1989;73:1143–56.[Medline]
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[Abstract/Free Full Text] - Schachter RK. Localised scleroderma. Curr Opin Rheumatol 1990;2:947–55.[Medline]
- Krafchik BR. Localised cutaneous scleroderma. Semin Dermatol 1992;1:65–72.
- Doyle JA, Connolly SM, Winkelmann RK. Cutaneous and subcutaneous inflammatory sclerosis syndromes. Arch Dermatol 1982;118:886–90.
[Abstract/Free Full Text] - Person JR, Su WPD. Subcutaneous morphoea: a clinical study of sixteen cases. Br J Dermatol 1979;100:371–80.[CrossRef][Web of Science][Medline]
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