Familial primary localized cutaneous amyloidosis
A rare primary cutaneous amyloidosis characterized by familial occurrence of lichen and/or macular amyloidosis due to fibrillary degeneration and apoptosis of basal keratinocytes followed by conversion of filamentous masses into amyloid material in the papillary dermis. Patients typically present with a pruritic eruption of grouped hyperkeratotic papules which may coalesce to form hyperkeratotic plaques with a predilection for the lower limbs (lichen amyloidosis) or with hyperpigmented macules sometimes with a reticulate pattern most commonly arising on the back chest or interscapular areas (macular amyloidosis).
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.
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Familial primary localized cutaneous amyloidosis?
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