Primary Cutaneous Amyloidosis with Familial Background / 대한피부과학회지

ABSTRACT

Primary localized cutaneous amyloidosis is classified as macular, lichen, and rarely nodular amyloidosis according to clinical manifestation. Most cases are sporadic, but several cases have been reported to be familial with autosomal dominant transmission. Herein, we report a patient with familial primary localized cutaneous amyloidosis suggesting autosomal dominant transmission. A 31-year old woman presented with pruritic brown hyperkeratotic papules on both legs which developed 5 years ago and gradually had spread around the knee. A skin biopsy showed an amorphous eosinophilic material in the papillary dermis that appeared pink with congo red stain. Her mother and older sister have also suffered from similar pruritic brown papules on the legs without any kind of manifestation suggesting the disease is systemic.