POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.

POEMS syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, POEMS syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that POEMS syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.

[Renal vein thrombosis and constitutional protein S deficiency]. / Thrombose de la veine rénale et déficit constitutionnel en protéine S.

Venous and arterial thrombosis due to a constitutional protein S deficiency is well-known. We report the case of a 36 year-old patient admitted to hospital in 1991 for primary renal vein thrombosis due to a constitutional protein S deficiency of type I. The diagnosis was made by CT scan and angiography. Left nephrectomy, which was made because of doubt with regard to subjacent neoplasm, showed left renal vein thrombosis and multiple renal infarcts. In 1994, after 4 months of discontinuation of oral anticoagulants, the patient presented pulmonary embolism documented by pulmonary scintigraphy and CT scan, partial portal thrombosis and sural thrombophlebitis documented by echography coupled with Doppler. To our knowledge, this is the first reported case of a constitutional protein S deficiency associated with primary renal vein thrombosis.