Recurrent tenosynovitis in Sweet's syndrome

A. M. E. Brown, M. G. Davies1 and P. Hickling

Departments of Rheumatology and
1 Dermatology, Derriford Hospital, Plymouth, Devon PL6 8DH, UK

SIR, An uncommon association of inflammatory polyarthritis is acute febrile neutrophilic dermatosis or Sweet's syndrome. This has the four cardinal features of fever, peripheral neutrophilic leucocytosis, painful plaque-forming inflammatory papules and a diffuse dermal neutrophilic infiltrate without vasculitis [1, 2]. There may be ocular, central nervous system, pulmonary, hepatic and renal involvement as well as oro-genital ulceration. Up to 62% of cases [3, 4] have musculo-skeletal manifestations including myalgia, arthralgia and arthritis. Of the cases of arthritis described in the literature [46], there no reports of associated tenosynovitis. The cause of Sweet's syndrome is unknown but it has an increased incidence in a number of conditions, particularly infections, haematological malignancies and solid tumours, inflammatory bowel disease, other autoimmune diseases and with certain drugs [3, 4].

We report a case of recurrent tenosynovitis in Sweet's syndrome, in a middle-aged woman who presented in the city where the first eight cases were originally described by Robert Douglas Sweet in 1964.

A 50-yr-old Caucasian barmaid was referred to the Dermatology Clinic, with a history of two episodes of painful skin lesions with an associated polyarthritis over the preceding 3 months. Each attack was characterized by the sudden onset of myalgia in the upper arms, thighs and calves, followed 24 h later, by arthralgia in her shoulders, knees and ankles with the development of swelling in her wrists and ankles, after 3 days. The rash appeared 1 week after the onset of her symptoms, initially as discrete, erythematous macules over her shoulders, the extensor aspect of her arms, thighs and shins. Blisters gradually formed in the centre of the macules, with fresh lesions continuing to develop for approximately 7 days and the rash lasting about 2 weeks. There were no subcutaneous nodules. The joint symptoms lasted 10 days. Her only regular medication was oestradiol which she had been taking for at least 2 yr. She smoked 20 cigarettes per day. She had had no symptoms suggestive of infection prior to the onset of each attack. Her past medical history was unremarkable apart from a Bilroth II gastrectomy performed at the age of 39 yr, for recurrent peptic ulceration.

On examination during the second attack, she was febrile with a temperature of 38°C. She had a capsulitis of the right shoulder with painful, restricted movements. Over the extensor surface of the left wrist there was a tender swelling, consistent with an extensor tenosynovitis. There was redness, tenderness and swelling over the left tibialis posterior tendon. Fading, erythematous macules were present over the extensor surface of the upper arms and thighs. In addition there were discrete, tender, inflammatory papules and papulovesicles over the upper arms (Fig. 1), thighs and shins. There was no oral or genital mucosal ulceration. She had no eye inflammation. The remainder of the examination was normal.



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FIG. 1. Upper arm showing inflammatory papules and pseudovesicles typical of Sweet's syndrome.

 

Investigations showed a total white cell count of 12.4 x 109/l with a neutrophil count of 9.3 x 109/l. ESR was 38 mm/h. Serological tests for parvovirus, enterovirus and coxsackie virus were negative. ANF, RF, pANCA and cANCA were negative. Full blood count, liver function tests, uric acid, complement levels, immunoglobulins and protein electrophoresis were normal. Bence Jones protein was negative. Urinalysis was normal. No abnormality of the involved joints was demonstrated on plain X-ray. Joint scan showed minimally increased uptake in the right knee and right foot but the appearances were not suggestive of synovitis. Ultrasound scan of the left wrist was in keeping with a hyperaemic tenosynovitis of the extensor tendons (Fig. 2). Fluid was present around the left tibialis posterior tendon. Biopsy of one of the skin lesions was typical of Sweet's syndrome with a mid-dermal dense inflammatory infiltrate consisting of large numbers of polymorphs. There was endothelial swelling but no true vasculitis. The papillary dermis was grossly oedematous with early vesicle formation. Chest X-ray and ultrasound scans of the abdomen and pelvis were normal.



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FIG. 2. Transverse ultrasound scan of left wrist showing synovial thickening of extensor tendon compartment (small arrow), extensor tendon (curved arrow) and lower end of radius (large arrow).

 

Treatment was commenced with dapsone in a dose of 100 mg per day. However, she continued to have further episodes of skin eruptions with myalgia, arthralgia and tenosynovitis approximately every 2 months, her last attack being associated with tenosynovitis of the left tibialis posterior tendon and the flexor tendon of the right index finger. Colchicine was added to the dapsone as the patient was reluctant to take corticosteroids.

Sweet's syndrome forms part of a spectrum of neutrophilic dermatoses which include pyoderma gangrenosum, subcorneal pustular dermatosis and erythema elevatum diutinum [7] and may occur in conjunction with erythema nodosum [8]. It is thought to be a hypersensitivity reaction to a bacterial, viral or tumour antigen. T-lymphocyte activation by cytokines and haemopoietic growth factors may be involved [3]. Studies of neutrophil function have not shown a consistent abnormality [4]. In the context of an acute arthritis, it is frequently confused clinically with a cutaneous vasculitis. Behçet's disease is another important differential diagnosis. Most cases respond to corticosteroids. Other previously reported treatments include indomethacin, naproxen, sulphapyridine, potassium iodide, clofazimine, dapsone, isotretinoin, methotrexate, chlorambucil cyclophosphamide, cyclosporin, interferon {alpha}2 and pulse methylprednisolone [3].

This patient demonstrates many of the manifestations of Sweet's syndrome with a recurrent, self-limiting, painful, blistering rash occurring in a middle-aged female in association with a fever, myalgia and a migratory, asymmetrical, polyarticular, large joint arthritis. The histology of affected skin, a peripheral neutrophilic leucocytosis and an elevated ESR are also typical. However, a significant clinical feature of her episodes has been tenosynovitis at multiple sites. This has not previously been included in descriptions of classical Sweet's syndrome, although tenosynovitis is recognized in the bowel bypass syndrome [9]. Histological findings in this condition are the same as in Sweet's syndrome. Bowel-associated dermatosis–arthritis syndrome has been used to describe the Sweet's syndrome which is found in bowel bypass syndrome and other bowel conditions, including ulcerative colitis, Crohn's disease and following partial gastrectomy [10], including Bilroth II gastrectomy for peptic ulceration [11].

We have not been able to identify any obvious underlying association with this patient's Sweet's syndrome. We considered the possible role of her Billroth II gastrectomy but post-operatively she remained well, with no obvious enteropathy. The onset of her current symptoms occurred 11 yr after her surgery, making any causative link unlikely. The syndrome has been reported in a patient on an oral contraceptive containing ethinyloestradiol [12]. Our patient had been on this preparation for several years prior to the development of the syndrome, again making this an unlikely association. Initial investigations have not unearthed a haematological malignancy or occult carcinoma. It is likely that this patient's Sweet's syndrome is idiopathic.

A review of the current literature, including Medline and Embase searches, has failed to reveal reports of any similar cases.

We report this case to highlight the previously undescribed feature of tenosynovitis occurring in association with an unusual cause of inflammatory arthritis, Sweet's syndrome.

Notes

Correspondence to: A. M. E. Brown, Royal Devon and Exeter Hospital, Barrack Road, Exeter EX2 5DW, UK. Back

References

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  3. Honigsmann H, Cohen PR, Wolff K. Acute febrile neutrophilic dermatosis (Sweet's syndrome). In: Freedberg IM, Eisen AZ, Wolff K et al., eds. Fitzpatrick's dermatology in general medicine, New York: McGraw-Hill,1999:1117–23.
  4. von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol1994;31:535–56.[ISI][Medline]
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  6. Nolla JM, Juanola X, Valverde J, Roig-Escofet D, Pagerols X, Servitge O. Arthritis in acute febrile neutrophilic dermatosis (Sweet's syndrome). Ann Rheum Dis 1990;49:135.
  7. Lear JT, Atherton MT, Byrne JPH. Neutrophilic dermatoses: Pyoderma gangrenosum and Sweet's syndrome. Postgrad Med J1997;73:65–8.[Abstract]
  8. Cohen PR, Holder WR, Rapini RP. Concurrent Sweet's syndrome and erythema nodosum: a report, world literature review and mechanisms of pathogenesis. J Rheumatol1992; 19:814–20.[ISI][Medline]
  9. Ely HP. The bowel bypass syndrome: a response to bacterial peptidoglycans. Am Acad Dermatol1980;2:473–87.[ISI][Medline]
  10. Weedon D. Bowel-associated dermatosis–arthritis syndrome. In: Weedon D, ed. Skin pathology. Edinburgh: Churchill Livingstone,1998:204.
  11. Jorizzo JL, Apisarnthanarax P, Subrt P et al. Bowel-bypass syndrome without bowel bypass. Bowel-associated dermatosis– arthritis syndrome. Arch Intern Med1983; 143:457–61.[Abstract]
  12. Tefany FJ, Georgouras K. A neutrophilic reaction of Sweet's syndrome type associated with the oral contraceptive. Aust J Dermatol1991;32:55–9.
Accepted 7 March 2002





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