Systemic amyloidosis and the skin: a review with special emphasis on clinical features and therapy

Abstract

Summary This report is a review of the literature on systemic amyloidosis and concentrates largely on primary and plasma cell dyscrasia‐related disease. Recent developments in the fields of amyloid structure and pathogenesis arc discussed and patterns of clinical presentation are described. It is emphasized that a combination of the carpal tunnel syndrome, macroglossia and specific mucocutaneous lesions necessitates an intensive search for an underlying plasma cell dyscrasia, and that immunodectrophoresis of serum and concentrated urine is essential to exclude a paraproteinaemia. Other diagnostic measures, including cutaneous histopathology, are considered. Despite the advances in our understanding of the pathogenesis of amyloid, there has been little progress in terms of the success of therapy, and the ultimate prognosis of patients with systemic amyloidosis remains poor.

A Patient with Multiple Myeloma, Amyloidosis and Light-Chain Deposition Disease in Kidneys with a Long Survival

“…Involvement of the wrist ligaments and the skin is usually associated with involvement of the heart muscles, the nerves and the gastro-intestinal tract. Involvement of the skin is one of the commonest manifestations of primary amyloidosis, and the lesions may consist of nodules, vesicles, plaques, nail dystrophy and thickening of the skin similar to that observed in scleroderma [6, 7]. In addition, vascular infiltration may result in purpura, ecchymoses and petechiae [8].…”

Amyloid vascular disease: cord-like thickening of mucocutaneous arteries, intermittent claudication and angina in a case with underlying myelomatosis

“…Cutaneous involvement is frequent in 'primary' and myeloma-associated amyloidosis and is often accompanied by glossomegaly. Cutaneous abnormalities in systemic amyloidosis have been extensively reviewed by Goltz (1952), Rukavina et al (1956), Brownstein & Helwig (1970), Rubinow & Cohen (1978) and Breathnach & Black (1979). Purpuric and ecchymotic lesions on normal or clinically involved skin, and smooth, shiny, waxy amber-coloured papules, nodules and plaques occurring on the fiexural areas are amongst the most characteristic lesions.…”

Nail Changes as the First Sign of Systemic Amyloidosis

“…Histologic evidence of amyloid deposits in the nail fold and nail bed has been described both in primary and my eloma-associated systemic amyloidosis [3,4,[9][10][11].…”