Primary localized cutaneous amyloidosis

Definition:

Primary localized cutaneous amyloidosis (PLCA) is a chronic itchy skin disorder associated with amyloid deposits in the superficial dermis. It is a purely cutaneous disease with no association with systemic forms of amyloidosis. Clinically, skin lesions comprise small, flat-top papules (lichen amyloidosis) or brown-gray macules (macular amyloidosis). PLCA is relatively common in South America and Asia, and some cases have an autosomal dominant family history (familial PLCA, FPLCA). The genetic basis of FPLCA involves mutations in the OSMR and IL31RA genes. Both belong to the family of interleukin (IL)-6 family cytokine receptors.

References:

[1]. Akio Tanaka, et al. The molecular skin pathology of familial primary localized cutaneous amyloidosis. Exp Dermatol. 2010 May;19(5):416-23. [Content Brief]

[2]. Chi-Fan Yang, et al. Loss of GPNMB Causes Autosomal-Recessive Amyloidosis Cutis Dyschromica in Humans. Am J Hum Genet. 2018 Feb 1;102(2):219-232. [Content Brief]

[3]. Ken Arita, et al. A novel OSMR mutation in familial primary localized cutaneous amyloidosis in a Japanese family. J Dermatol Sci. 2009 Jul;55(1):64-5. [Content Brief]

Biomedical Dictionary

The Biomedical Dictionary is a comprehensive and professional collection of biological academic terms and subject datas. All explanations are supported by authoritative books or high impact factor literatures, and you can acquire accurate explanations of the biomedical terms you want to know.

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z #

Name
Email *

	Sorry, but the email address you supplied was invalid.