Amyloidosis, Primary Localized Cutaneous, 1

Alternative Names

  • PLCA1
  • Amyloidosis, Primary Cutaneous, 1
  • PCA1
  • PCA
  • Lichen Amyloidosis, Familial
  • Amyloidosis IX
  • Amyloidosis, Familial Cutaneous Lichen
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WHO-ICD-10 version:2010

Endocrine, nutritional and metabolic diseases

Metabolic disorders

OMIM Number

105250

Mode of Inheritance

Autosomal dominant

Gene Map Locus

5p13.1

Description

Primary Localized Cutaneous Amyloidosis (PLCA) is a condition characterized by the deposition of amyloid protein plaques in the dermal layer of the skin, without any evidence of such deposits in the internal organs. Three major types of PLCAs are recognized- Lichen, Macular and Nodular PLCA, with the macular form being the most common. The condition is caused by mutations in the OSMR gene, which encodes a receptor for the cytokine Oncostatin M.

Epidemiology in the Arab World

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Other Reports

Lebanon

Kibbi et al, 1992, studied 57 patients with primary localized cutaneous amyloidosis. Two-thirds of patients had macular amyloidosis (MA). Intermediate cases constituting of macular lesions with micropapules and/or lichens were also identified. Histopathologic studies found similar characteristic features in the macules, micropapules, and lichens, that appeared more prominent in the lichens.

Oman

Venkataram et al., 2001, studied the role of friction in the causation of cutaneous amyloidosis in 10 patients, four of whom were of Omani nationality. 

Saudi Arabia

Al-Ratrout and Satti, 1997, conducted a retrospective evaluation of all patients that were clinically diagnosed with cutaneous amyloidosis in a tertiary care hospital between 1987 and 1995. Out of 42 suspected cases, 21 were histologically confirmed to have PLCA.  

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