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Trends Mol Med. 2018 May;24(5):435-448. doi: 10.1016/j.molmed.2018.03.007. Epub 2018 Apr 13.

Lichen Planopilaris and Frontal Fibrosing Alopecia as Model Epithelial Stem Cell Diseases.

Author information

1
The Dermatology Centre, Salford Royal NHS Foundation Trust, Salford, Greater Manchester, UK; Centre for Dermatology Research, University of Manchester, MAHSC and NIHR Manchester Biomedical Research Centre, Manchester, UK.
2
Mediteknia Dermatology Clinic and Universidad Fernando Pessoa Canarias, Las Palmas de Gran Canaria, Spain.
3
Tissue Engineering Group, Instituto Biodonostia, Hospital Universitario Donostia, San Sebastián, Spain.
4
Centre for Dermatology Research, University of Manchester, MAHSC and NIHR Manchester Biomedical Research Centre, Manchester, UK.
5
Department of Zoology, University of Kerala, India.
6
Department of Pathology, Murcia University, University General Hospital of Murcia, Murcia, Spain.
7
Centre for Dermatology Research, University of Manchester, MAHSC and NIHR Manchester Biomedical Research Centre, Manchester, UK; Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA. Electronic address: ralf.paus@manchester.ac.uk.

Abstract

Inflammation-associated, irreversible damage to epithelial stem cells (eSCs) of the hair follicle in their immunologically privileged niche lies at the heart of scarring alopecia, which causes permanent difficult-to-treat hair loss. We propose that the two most common and closely related forms, lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA), provide excellent model diseases for studying the biology and pathology of adult human eSCs in an easily accessible human mini-organ. Emphasising the critical roles for interferon (IFN)-γ and peroxisome proliferator-activated receptor (PPAR)-γ-mediated signalling in immune privilege (IP) collapse and epithelial-mesenchymal transition (EMT) of these eSCs respectively, we argue that these pathways deserve therapeutic targeting in the future management of LPP/FFA and other eSC diseases associated with IP collapse and EMT.

KEYWORDS:

epithelial–mesenchymal transition; hair follicle; immune privilege; interferon-γ; peroxisome proliferator-activated receptor-γ; stem cells

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