Primary Localized Cutaneous Amyloidosis Affecting Female Individuals of a Pakistani Pedigree. (May 2019)

• Record Type: Journal Article
• Authors: Bhoyrul, Bevin; Ng, Alicia; Laws, Philip M.; Mathew, Bipin; Shanmugam, Sangeetha
• Is Part Of: American journal of dermatopathology. Volume 41:Number 5(2019)
• Publication Date: 2019-05
• Subjects: primary localized cutaneous amyloidosis -- macular amyloidosis -- amyloidosis cutis dyschromica -- OSMR gene -- IL31RA gene; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000001288
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract : Abstract: Primary localized cutaneous amyloidosis is a group of rare conditions where amyloid deposition is limited to the skin without systemic manifestations. Most cases are sporadic; however, mutations in the oncostatin M receptor ( OSMR ) and interleukin-31 receptor A ( IL31RA ) genes can cause a familial form of the condition in up to 10% of cases. Here, we describe a family in which 8 female individuals are affected by either macular amyloidosis or amyloidosis cutis dyschromica. To the best of our knowledge, a sex-specific expression or the coexistence of 2 different phenotypes of primary localized cutaneous amyloidosis in 1 pedigree has not yet been reported.