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Diseases

Genetic and Rare Diseases Information Center (GARD)

Primary cutaneous amyloidosis


Other Names for this Disease
  • Primary localized cutaneous amyloidosis
  • PLCA
  • Lichen amyloidosis familial
  • Amyloidosis familial cutaneous lichen
  • Amyloidosis 9
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Overview

Primary cutaneous amyloidosis is a form of amyloidosis, a group of conditions in which an abnormal protein (called amyloid) builds up in various organs and tissues throughout the body. In primary cutaneous amyloidosis, specifically, this protein accumulates in the skin. There are three main forms of primary cutaneous amyloidosis:[1][2]
  • Lichen amyloidosis - multiple itchy, raised spots which are scaly and red/brown in color. This rash generally affects the shins, thighs, feet and forearms.
  • Macular amyloidosis - mild to severely itchy, flat, dusky-brown or greyish colored spots that may come together to form patches of darkened skin. This rash generally appears on the upper back between the shoulder blades, the chest and less commonly, the arms.
  • Nodular amyloidosis - asymptomatic firm bumps that may be pinkish-brown to red in color. This rash may occur on the trunk, limbs, face, and/or genitals.
The exact underlying cause of primary cutaneous amyloidosis is poorly understood. It is sometimes associated with other conditions such as atopic dermatitis, sarcoidosis, and psoriasis. Although most cases occur sporadically in people with no family history of the condition, there are rare familial forms caused by changes (mutations) in the OSMR or IL31RA gene.[3][4] Treatment is based on the signs and symptoms present in each person and may include surgery to remove amyloid deposits and/or certain medications to relieve itching.[1][2]
Last updated: 3/29/2016

References

  1. Amyloidosis. DermNet NZ. June 2014; http://www.dermnetnz.org/systemic/amyloidosis.html.
  2. Al Yahya RS. Treatment of primary cutaneous amyloidosis with laser: a review of the literature. Lasers Med Sci. March 2016; [Epub ahead of print]:
  3. AMYLOIDOSIS, PRIMARY LOCALIZED CUTANEOUS, 1. OMIM. May 2011; http://www.omim.org/entry/105250.
  4. AMYLOIDOSIS, PRIMARY LOCALIZED CUTANEOUS, 2. OMIM. May 2011; http://www.omim.org/entry/613955?search=cutaneous%20amyloidosis&highlight=cutaneous%20amyloidosi.
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Basic Information

  • Amyloidosis Awareness is an illustrated booklet for patients and physicians developed by Amyloidosis Support Groups Inc. Versions of the booklet are also available in Spanish and Portuguese.
  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
    Lichen Amyloidosis
    Macular Amyloidosis
    Nodular Localized Cutaneous Amyloidosis
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    Primary localized cutaneous amyloidosis 1
    Primary localized cutaneous amyloidosis 2
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Primary cutaneous amyloidosis. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Primary localized cutaneous amyloidosis
  • PLCA
  • Lichen amyloidosis familial
  • Amyloidosis familial cutaneous lichen
  • Amyloidosis 9
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.