A Case of Familial Primary Localized Cutaneous Amyloidosis / 대한피부과학회지

ABSTRACT

Primary localized cutaneous amyloidosis comprises macular, papular and rare nodular amyloidosis. Macular and papular amyloidosis are considered as different manifestations of the same disease process and can occur in the same patient, which is known as biphasic amyloidosis. Although most cases of primary cutaneous amyloidosis occur sporadically, some cases have been reported to have familial incidence with papular, biphasic or rarely macular amyloidosis. We report a case of a family in which a mother and her son have brown hyperkeratotic pruritic papules on both upper and lower extremities. The skin lesions initially began as macules on the shins in the second or third decade, but gradually spread with increasing papules and pigmentation. They had no kind of manifestation suggesting systemic amyloidosis. A skin biopsy specimen disclosed amyloid deposition in papillary dermis.