Abstract

The cutaneous amyloidoses represent a heterogeneous group of conditions in which amyloid, a fibrillar material that can result from the degradation of various proteins, is deposited in the skin. In primary cutaneous amyloidosis, the deposits are derived from keratin (macular, lichen, biphasic) or immunoglobulin light chains (nodular). The specific cutaneous lesions of primary systemic amyloidosis are waxy, translucent or purpuric papules, nodules and plaques. Primary systemic amyloidosis is due to a plasma cell dyscrasia while secondary systemic amyloidosis arises from chronic inflammatory conditions such as rheumatoid arthritis or in the setting of chronic infections. Treatment of all forms of amyloidosis is challenging, and although the primary cutaneous forms are not life-threatening, primary systemic amyloidosis can carry a poor prognosis such that early recognition and appropriate management are vital.

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