Department of Rheumatology, Musgrave Park Hospital, Stockman's Lane, Belfast BT9 7JB and
1 Department of Rheumatology, Altnagelvin Area Hospital, Glenshane Road, Londonderry, UK
SIR, Digital ischaemia is a condition which may present to rheumatologists [1]. It is important to be aware that it can be a rare manifestation of internal malignancy [1, 2]. Improvement of the digital ischaemia may occur following treatment of the underlying disease [3].
A previously well 48-yr-old lady presented with a 6-month history of painful cold fingers, particularly affecting the middle and ring fingers of her left hand. There was no history of Raynaud's syndrome. The symptoms had not improved with aspirin EC 300 mg, Adalat Retard (nifedipine) 10 mg b.d. and amlodipine 5 mg daily. A moderate improvement was noted following five intravenous infusions of Iloprost (prostacyclin) during a vascular surgery assessment.
On examination she was apyrexic. There were ischaemic changes of the middle and ring fingers of the left hand (Fig. 1) but no ischaemic changes in the toes. All peripheral pulses were palpable, with blood pressure 140/85 mmHg in both arms. There were no large vessel bruits or cardiac murmurs. The remainder of the physical examination was unremarkable.
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Immunoglobulins showed elevated IgG (25.3 g/l; normal range 7.016.0), decreased IgM (0.35 g/l; normal range 0.84.7) and IgA was within normal limits (0.55 g/l; normal range 0.53.0). Serum electrophoresis showed a paraprotein band of 23 g/l in the gamma globulin region (IgG1 paraprotein). Urine was negative for Bence Jones protein. Serum calcium was normal and the skeletal survey was unremarkable. Bone marrow aspiration confirmed the diagnosis of multiple myeloma, with plasma cell infiltrate accounting for 25% of the cells. Treatment was commenced with plasmapheresis and six courses of combination chemotherapy (cyclophosphamide, vincristine, adriamycin and prednisolone). At rheumatology review after treatment, the patient had complete resolution of the ischaemic symptoms and signs in the hands (Fig. 2).
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Digital necrosis [4] and other vasculitic syndromes [1, 6, 7] have been reported in association with myeloma but the dramatic response to treatment observed in this case is not typical. The clinical manifestations of digital ischaemia with neoplasia vary from pulp atrophy to frank digital necrosis. Classical Raynaud's phenomenon is not typical but has been reported as an initial manifestation of ovarian carcinoma [8]. The commonest associated malignancy is lung adenocarcinoma. Gastrointestinal tumours, gynaecological tumours, renal cell carcinoma, leukaemia and lymphoma have also been reported in association with digital necrosis [1, 2, 4, 9].
An understanding of the mechanism of digital ischaemia is crucial, since it may dictate therapy. The induction of vasculitis by antibodies to tumour antigens has been suggested as a possible mechanism [3]. Digital ischaemic symptoms have been reported to fade after removal of renal carcinoma and ovarian carcinoma, with recurrence indicating relapse [3, 10]. The improvement in digital ischaemia following treatment of multiple myeloma with plasmapheresis and chemotherapy in this case may be in keeping with the suggestion that tumour antigen contributes to the pathogenesis. However, prednisolone and cyclophosphamide are also effective treatments used in the management of necrotizing vasculitis.
The possibility of underlying malignant disease should be considered in patients presenting with digital ischaemia, and the investigation of such patients should include plasma protein electrophoresis. Treatment of the underlying tumour may be the most effective treatment option in these patients.
Notes
Correspondence to: P. A. Courtney.
References