Departments of Dermatology,
1 Anatomo-Pathology and
2 Rheumatology, CHRU Caen, Avenue Côte de Nacre, 14000 Caen, France
SIR, We report a case of a leg ulceration caused by cholesterol embolism, with an aspect of a vascular ulcer, improved by colchicine and corticosteroids.
A 71-yr-old woman, with a rheumatoid arthritis stabilized with methotrexate and salazopyrine, was hospitalized for a 2 yr history of an ulceration of the right leg. The ulcer was 12 cm in diameter, fibrinous, painful and with inflammatory margins, but no livedo, nodule or purpura. She had palpable pulses. Duplex ultrasonography showed an incompetence of the sapheno-femoral junction and an haemodynamically significant superficial femoral arterial stenosis in the Hunter area. Standard biology, proteinuria, antithrombin III, protein C, S, homocystein, factor V Leiden and cryoglobulin were normal or negative. IgM rheumatoid factors were positive (208 IU; normal value <10 IU; ELISA) as well as a anti-ß2GP1 IgG antibody (27 AU). Erythrocyte sedimentation rate (ESR) was 26 mm/h and C reactive protein (CRP) 40 mg/l. A biopsy of the ulcer revealed a lymphocytic infiltrate in the dermis without vasculitis and capillary thrombosis. Treatment with vacuum-assisted closure followed by a skin graft failed and the margins of the ulcer became necrotic. A bilateral femoral arteriogram showed the right femoral stenosis. A percutaneous transluminal angioplasty was realized, but the ulcer enlarged and was nearly circumferential. The positivity of anti-ß2GP1 antibody was confirmed (31 AU), so the patient received an anticoagulant therapy to treat a possible thrombosis, but the ulcer enlarged. After a few months, she was hospitalized for asthenia, weight loss and increase of the pain of the ulcer. The ulceration was enlarged with the tendon tibialis anterior denuded (Fig. 1A). ESR was 132 mm/h and CRP 152 mg/l. A biopsy of the ulcer showed cholesterol clefts within the lumen of dermal blood vessels (Fig. 2). Anticoagulation was stopped and colchicine 2 mg/day was started. The ulcer improved. Because of a serious diarrhoea, colchicine was discontinued and switched for prednisolone 40 mg/day. When the gastrointestinal side-effects diminished, colchicine was started again and amount of prednisolone was lowered. Healing of the ulcer was dramatic, with granulation tissue covering the tendon and epithelialization of the edges. Four months later, three-quarters of the ulcer was healed with colchicine 2 mg/day and prednisolone 10 mg/day (Fig. 1B).
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Potential precipitating factors are identified in one-third of cases, and are: anticoagulant, angioplasty, vascular surgery, catheterization and fibrinolysis. In our case, the ulcer's aggravation after angioplasty and anticoagulant therapy was caused by cholesterol embolism. Initially the patient's ulceration could be secondary to arteritis and/or to cholesterol crystal embolism. However, all the features are in favour of cholesterol embolism. There is no argument for vasculitis provoked by rheumatoid arthritis. Moreover, the patient was on methotrexate, which seems to be efficacious in these cases of vasculitis [4, 5]. Our patient did not have the manifestations of Felty's syndrome in which leg ulcers are frequent, and did not have the continued rapid expansion of a pyoderma gangrenosum.
With respect to therapy, the vascular surgery of the atheromatous plaque is theoretically ideal. However, a large prospective study [6] failed to show any benefit of surgical treatment over medical therapy. Some authors have suggested that the withdrawal of anticoagulants is common and that corticosteroids may be effective in treating cholesterol embolism [7], particularly an intractable leg ulceration caused by cutaneous cholesterol embolism dramatically healed within 3 weeks treatment with prednisolone [8]. Dahlberg et al. [9] had a rapid resolution of cutaneous peripheral embolization in two patients treated with corticosteroids. Cholesterol embolism probably perpetuates the ischaemic process. Therefore, corticosteroid could reduce this local or general inflammatory response [9]. Colchicine also seems to be effective, limiting the chemotactic and phagocytic activity of polymorphonuclear lymphocytes [10]. If colchicine is not sufficient to control ischaemia or systemic inflammation, it seems logical to treat them with corticosteroids in the same way as systemic small vessels vasculitis [7].
Notes
Correspondence to: L. Verneuil. E-mail: laurence.verneuil{at}bichat.inserm.fr
References