Familial primary localized cutaneous amyloidosis

Get in touch with RARE Concierge.

Contact RARE Concierge

Familial primary localized cutaneous amyloidosis

Synonyms: FPLCA

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.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.

Newly diagnosed with
Familial primary localized cutaneous amyloidosis?

Our RARE Concierge Services Guides are available to assist you by providing information, resources and connections as you navigate your rare disease journey.

Get Concierge Help

Advocacy Organizations

Clinical Trials

For a list of clinical trials in this disease area, please click here.