Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition
Open Access
- 1 July 2021
- journal article
- case report
- Published by MDPI AG in Dermatopathology
- Vol. 8 (3), 229-235
- https://doi.org/10.3390/dermatopathology8030028
Abstract
Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, and in a few cases with systemic sclerosis. Herein, we describe three cases of PLCNA occurring in women with a diagnosis of limited cutaneous systemic sclerosis and review the literature on the topic to highlight a stereotypical presentation. Moreover, we support the usefulness of dermoscopy, characterized by a yellow–orange waxy pattern surrounded by telangiectasias, for a rapid and non-invasive diagnostic assessment. Thus, when asymptomatic nodules occur on lower limbs of women affected with limited systemic sclerosis, and dermoscopy identifies yellow–orange blotches, a diagnosis of PLCNA can be considered and further confirmed by histopathology. Monitoring for systemic amyloidosis development is advisable, although the risk of progression is considered very low.This publication has 24 references indexed in Scilit:
- Multiple lesions of primary cutaneous nodular amyloidosis in Sjögren syndromeJournal of the American Academy of Dermatology, 2012
- Characteristic dermoscopic features of primary cutaneous amyloidosis: a study of 35 casesBritish Journal of Dermatology, 2012
- Cutaneous Amyloidosis: A Concept ReviewThe American Journal of Dermatopathology, 2012
- Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamideAustralasian Journal of Dermatology, 2011
- A Plethora of ProteinThe American Journal of Medicine, 2010
- Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndromeInternational Journal of Dermatology, 2010
- Sjögren's syndrome and localized nodular cutaneous amyloidosis: Coincidence or a distinct clinical entity?Arthritis & Rheumatism, 2008
- Papules and Plaques on the Nose—Quiz CaseArchives of Dermatology, 2007
- The Systemic AmyloidosesThe New England Journal of Medicine, 1997
- Nodular localized cutaneous amyloidosis: detection of monoclonality of infiltrating plasma cells by polymerase chain reactionBritish Journal of Dermatology, 1996