title: Familial primary cutaneous amyloidosis.
abstract: Primary cutaneous amyloidosis is uncommon in Europeans. A British family is described in which an extremely rare variant was inherited as an autosomal dominant. The subtlety of physical signs in this family contrasted with the severity of the associated pruritus. Transepidermal elimination of amyloid was a characteristic histological feature.
mesh_terms: D000293:Adolescent; D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D011537:Pruritus / Q000209:etiology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications / Q000235:genetics* / Q000473:pathology