ABSTRACT

Primary localized cutaneous amyloidosis (PLCA) is characterized by amyloid deposition limited to previously apparently normal skin without systemic organ involvement. The best-recognized local forms of amyloidosis confined to the skin are the more commonly seen macular and lichen subtypes and the rare nodular subtype. The etiopathogenesis of PLCA has not been clearly elucidated. However, many studies have attempted to define the chemical nature of the dermal amyloid deposits. Nonfibrillar oligomeric structures are thought to contribute to the pathogenesis of amyloid diseases supporting the hypothesis of common pathogenetic pathways. Nonfibrillar oligomeric structures are thought to contribute to the pathogenesis of amyloid diseases supporting the hypothesis of common pathogenetic pathways. Even though several treatment alternatives have been tried, the results have been unsatisfactory. As interleukin 31 (IL-31) has been implicated in the pathophysiology of PCLA and other itchy dermatoses, therapies targeting IL-31 receptor might play a role in the management of the disease.