Autoimmunity in Segmental Vitiligo

The autoimmune basis of segmental vitiligo (SV) has only recently been recognized. Systemic autoimmune diseases are less frequently associated compared to non-segmental vitiligo (NSV), but localized skin disorders - in particular linear morphea - have been repeatedly observed in patients with SV. Th...

Full description

Saved in:
Bibliographic Details
Published in:Frontiers in immunology 2020-10, Vol.11, p.568447-568447
Main Authors: Speeckaert, Reinhart, Lambert, Jo, Bulat, Vedrana, Belpaire, Arno, Speeckaert, Marijn, van Geel, Nanja
Format: Article
Language:English
Subjects:
Animals
Autoimmunity
Humans
Immunology
lichen striatus
linear morphea
Mosaicism
segmental vitiligo
Vitiligo - genetics
Vitiligo - immunology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:The autoimmune basis of segmental vitiligo (SV) has only recently been recognized. Systemic autoimmune diseases are less frequently associated compared to non-segmental vitiligo (NSV), but localized skin disorders - in particular linear morphea - have been repeatedly observed in patients with SV. The inflammatory response is documented on a clinical level with cases displaying erythematous borders or a hypochromic stage, on a histopathological level with predominantly CD8 lymphocytes migrating toward the basal layer and by flow cytometry demonstrating the antimelanocyte specificity of these cytotoxic T cells. The increased risk for halo naevi and NSV in these patients further underline the immune-mediated mechanisms of SV. Nonetheless, the localized and unique distribution pattern points to somatic mosaicism. This places SV in a category of similar diseases such as lichen striatus, blaschkitis, linear lupus erythematosus, and linear scleroderma where an immune reaction against genetically mutated skin cells is believed to be the underlying cause. All these disorders are characterized by a young age of onset, a temporary disease activity with spontaneous resolution, limited response to treatment, and often long-term sequelae. Although challenging, genetic research proving this genetic mosaicism could offer crucial insights into the pathogenesis of both segmental and non-segmental vitiligo.
ISSN:1664-3224
1664-3224
DOI:10.3389/fimmu.2020.568447