Mucocutaneous manifestations in systemic amyloidosis

Abstract

It has long been recognized that certain characteristic mucocutaneous symptoms and signs may act as important clues in diagnosing the early stage of systemic amyloidosis. Since the first description of clinical cutaneous lesions of primary amyloidosis by Koenigstein 1 in 1925, the spectrum of mucocutaneous manifestations in this disorder has been well documented. 2–7 There is no truly satisfactory classification of systemic amyloidosis; however, systemic amyloidosis could be simply classified as follows: 1. 1. Primary systemic amyloidosis: usually no evidence of preceding or coexisting disease but paraproteinemia or plasma-cell dyscrasia can be found. 2. 2. Amyloidosis associated with multiple myeloma. 3. 3. Secondary systemic amyloidosis: evidence for coexistence of previous chronic inflammatory or infectious conditions. 4. 4. Heredofamilial amyloidosis. Based on the result of amyloid protein analysis, Glenner 8 has proposed a more detailed clinical-chemical-pathological classification for human amyloidoses. It appears, however, that this classification is overly complex for clinicians, thus its clinical usage seems rather limited. In primary systemic and myeloma-associated amyloidosis, deposits consist mainly of light-chain (AL) amyloid protein produced as a result of plasma-cell dyscrasia. In a large series of 236 cases of systemic amyloidosis, 4 56% were of primary type and 26% were multiple-myeloma patients. During the period 1978–1987, we have found 14 cases of systemic amyloidosis in our hospital; primary systemic amyloidosis with a type of paraprotein and amyloidosis secondary to multiple myeloma occurred in nine cases (64%) and five cases (36%), respectively. Multiple myeloma associated with amyloidosis has been widely studied in medicine. It has been pointed out that amyloidosis develops in 6–15% of patients with myeloma. 9 In our hospital, five cases (4%) out of 159 myeloma patients showed an association with systemic amyloidosis.