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- Title
Mechanism underlying pruritus in recessive dystrophic epidermolysis bullosa: Role of interleukin- 31 from mast cells and macrophages.
- Authors
Sang Gyun Lee; Song-Ee Kim; In-Hye Jeong; Sang Eun Lee
- Abstract
Background: Pruritus is a highly burdensome symptom in patients with epidermolysis bullosa, especially recessive dystrophic epidermolysis bullosa (RDEB); however, only a few studies have assessed the molecular pathogenesis of RDEB-associated pruritus. Interleukin (IL)-31 is a key cytokine implicated in pruritus associated with dermatologic diseases such as atopic dermatitis and prurigo nodularis. Objective: To investigate the role and cellular source of IL-31 in RDEB-associated pruritus. Methods: Serum and skin samples were obtained from 11 RDEB patients and 11 healthy controls. Pruritus visual analogue scale scores were determined. Serum levels of IL-31 and thymic stromal lymphopoietin (TSLP) were examined by enzymelinked immunosorbent assay (ELISA). The expression of IL-31 and other pruritus mediators in the skin were examined through immunofluorescence staining, and their correlation with pruritus severity was analysed. Results: Serum IL-31 and TSLP were elevated in RDEB patients. IL-31 expression was increased in RDEB skin and positively correlated with pruritus severity. Most of the IL-31-expressing cells were mast cells, and some were CD206(+) M2-like macrophages. The number of substance P(+) cells was also increased in the patients' skin, and most of them were mast cells. The number of substance P(+) mast cells was correlated with the number of IL-31(+) dermal infiltrates. The number of IL-4Rα-and IL-13-expressing cells and expression of TSLP and periostin increased in RDEB skin, but without a correlation to pruritus score. Conclusion: The increased production of skin IL-31 from mast cells and M2-like macrophages may be the mechanism underlying pruritus in RDEB.
- Subjects
EPIDERMOLYSIS bullosa; MAST cells; ITCHING; THYMIC stromal lymphopoietin; SUBSTANCE P; MACROPHAGES
- Publication
Journal of the European Academy of Dermatology & Venereology, 2024, Vol 38, Issue 5, p895
- ISSN
0926-9959
- Publication type
Article
- DOI
10.1111/jdv.19738