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가족성 원발성 피부 유전분증 1예
A Case of Familial Primary Localized Cutaneous Amyloidosis
안희태(Hee Tae An),한광호(Gwang Ho Han),조광현(Gwang Hyun Jo)
UCI I410-ECN-0102-2009-510-004771792

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.

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