Case Report: Soft-tissue Involvement in Systemic Lupus Erythematosus

British Journal of Rheumatology 1997;36:703–704 CASE REPORT

SOFT-TISSUE INVOLVEMENT IN SYSTEMIC LUPUS ERYTHEMATOSUS E. M. MCDERMOTT and R. J. POWELL Clinical Immunology Unit, Queen’s Medical Centre, University Hospital, Nottingham NG7 2UH

titre 1:50 (rat liver), anti-dsDNA (by ELISA) 54 IU/ml (q50 positive) and negative rheumatoid factor. Following initial treatment with hydroxychloroquine and then azathioprine, she was controlled on weekly methothrexate. Each episode of swelling starts with a feeling of tightness across the skin of the anterior surface of the forearm and involves a single arm each time. The forearm then becomes painful with marked local swelling and an overlying warm, tender, erythematous patch of skin. She feels that exercise may precipitate an attack and ice applied locally relieves the symptoms. The swellings develop at irregular intervals, about eight times a year, each lasting for less than a week. The frequency and severity of the episodes have remained constant over the last 2 yr and do not appear to parallel her lupus activity. Examination when symptomatic reveals marked pitting oedema and minimal erythema (see Fig. 1). C1q, C3, C4 and C1 esterase inhibitor levels are normal. Magnetic resonance imaging (using STIR sequence to reduce signalling from fat) demonstrates oedema in the s.c. tissues, particularly at the anterior aspect of the forearm which appears to extend into the intermuscular

S lupus erythematosus (SLE) is a multi-system inflammatory disease. Soft-tissue manifestations are common and include calcification, arthritis, tenosynovitis and myositis. We report a 41-yr-old woman with unusual soft-tissue swellings not previously described in SLE. CASE REPORT A 41-yr-old woman with SLE presented with a 2 yr history of episodic painful swellings of the lower forearms. She had a 15 yr history of arthritis affecting the hands, wrists, knees and feet, recurrent pleurisy, associated lymphopenia (0.66 × 109/l), thrombocytopenia (79 × 109/l), a positive ANA IgG Submitted 29 July 1996; revised version accepted 14 November 1996. Correspondence to: E. M. McDermott, Clinical Immunology Unit, Queen’s Medical Centre, University Hospital, Nottingham NG7 2UH.

F. 1.—Photograph of the left forearm showing thumb imprint demonstrating marked pitting oedema.

= 1997 British Society for Rheumatology 703

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F. 2.—Left forearm MRI, transverse section, STIR sequence (suppresses signalling from fat) demonstrating oedema in the subcutaneous tissues particularly at the anterior aspect of the forearm extending into the intermuscular septa.

septa; the appearance of the muscles and bones is normal (see Fig. 2). An intra-lesional biopsy revealed a mild superficial dermal perivascular infiltrate, but no vasculitis.

DISCUSSION Intermittent s.c. swelling has not been reported before in SLE. There have been several reports of soft-tissue pneumococcal infections in SLE [1], but the history of spontaneous resolution of symptoms does not suggest an infective cause in this case. Similar swellings are frequently seen in attacks of Familial Hibernian Fever (FHF), a rare autosomal dominant periodic fever syndrome characterized by episodic localized myalgia and abdominal pain which responds

dramatically to prednisolone [2]. In the majority of cases of FHF, the episodic localized myalgia is associated with tender, warm, erythematous soft-tissue swellings which may occur at any site. When a limb is affected, the lesion may migrate distally over several days during the course of an attack (personal observation). Histological findings reveal a non-specific perivascular inflammatory infiltrate. In Familial Mediterranean Fever (FMF), an autosomal recessive periodic fever found particularly in Sephardic Jews, Arabs and Armenians, a similar ‘erysipelas-like’ lesion occurs usually in association with arthritis of the ankle joint [3]. The lesions occur rarely (Q5%) and only appear below the knee, in contrast with FHF where the lesions are common (q90%) and may occur at any site. In FMF, the lesions are unresponsive to steroids, but inflammatory attacks are reduced on prophylactic colchicine therapy. Histology reveals a predominant polymorphonuclear infiltrate with pronounced dermal oedema [4]. These soft-tissue lesions are accepted as manifestations of the systemic inflammatory processes in both FHF and FMF. Although the occurrence of the swellings in this case does not correlate with lupus activity, it is likely that the soft-tissue inflammatory lesion is also a manifestation of SLE. R 1. Crowe H, Lawlor M, Quintiliani R. Pneumococcal soft tissue infections in patients with systemic lupus erythematosus. Clin Infect Dis 1995;20:1571. 2. Williamson L, Hull D, Mehta R, Reeves W, Robinson B, Toghill P. Familial Hibernian Fever. Q J Med 1982;204:469–80. 3. Majeed H, Quabzrd Z, Hijazi Z, Farwana S, Harshani F. The cutaneous manifestations in children with Familial Mediterranean Fever (recurrent hereditary polyserositis): a six-year study. Q J Med 1990;75:607–16. 4. Aziz E, Fisher B. Cutaneous manifestations of Familial Mediterranean Fever. Arch Dermatol 1976;112:364–6.