Cutaneous necrosis associated with the antiphospholipid syndrome and mycosis fungoides

Abstract

The development of extensive cutaneous necrosis in a patient with tumour-stage mycosis fungoides is described. Skin biopsies showed a lymphomatous infiltrate, and thrombosis of dermal blood vessels. Investigation revealed the presence of anticardiolipin antibodies, a lupus anticoagulant, and low free protein S, which contributed to a prothrombotic state. Antiphospholipid antibodies have been detected in non-Hodgkin's lymphoma, but clinical manifestations are uncommon. Such autoantibodies may be produced by neoplastic lymphoid cells. The frequency with which antiphospholipid antibodies occur in mycosis fungoides is currently unknown.

Dermatologic aspects of antiphospholipid syndrome

“…Anetoderma has been reported in both primary and SLErelated APS. 44,63 Blue toe syndrome, 64 mycosis fungoides, 65 eosinophilic fasciitis, and multiple morphea 66 have been reported in association with LA or aPLs. Adrenal failure secondary to hemorrhagic infarction of the adrenal glands was reported as a melanoderma in APS.…”

Antiphospholipid Antibodies, Malignancies and Paraproteinemias

“…Allegue et al [53] documented a patient with cutaneous T cell lymphoma of the Mycosis fungoides type with an APS. Another patient with Mycosis fungoides who developed cutaneous necrosis and APS was reported by Hill et al [54]. This patient also demonstrated low free protein S levels.…”

Widespread cutaneous necrosis occurring in association with the antiphospholipid syndrome: a report of two cases

“…There are only 12 previously reported cases. 14–22 All of the patients were women. In five; the associated disease was SLE; one was associated with rheumatoid arthritis, another, mycosis fungoides; and a third, human immunodeficiency virus.…”