Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

Christopher J. Issa; Aubrey C. Hong; Peter A. Lio

doi:10.1155/2023/3863357

Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

Christopher J. Issa
, Aubrey C. Hong
, Peter A. Lio

Research output: Contribution to journal › Review article › peer-review

2 Scopus citations

Abstract

Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These fndings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.

Original language	English
Article number	3863357
Journal	Dermatologic Therapy
Volume	2023
DOIs	https://doi.org/10.1155/2023/3863357
State	Published - 2023
Externally published	Yes

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@article{3ded30af0625464ab209581135444d96,

title = "Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy",

abstract = "Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients{\textquoteright} quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These fndings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab{\textquoteright}s potential in the treatment of refractory pruritus.",

author = "Issa, \{Christopher J.\} and Hong, \{Aubrey C.\} and Lio, \{Peter A.\}",

note = "Publisher Copyright: Copyright {\textcopyright} 2023 Christopher J. Issa et al.",

year = "2023",

doi = "10.1155/2023/3863357",

language = "English",

volume = "2023",

journal = "Dermatologic Therapy",

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T1 - Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa

T2 - A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

AU - Issa, Christopher J.

AU - Hong, Aubrey C.

AU - Lio, Peter A.

PY - 2023

Y1 - 2023

N2 - Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These fndings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.

AB - Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These fndings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.

UR - https://www.scopus.com/pages/publications/85205940787

U2 - 10.1155/2023/3863357

DO - 10.1155/2023/3863357

M3 - Review article

AN - SCOPUS:85205940787

SN - 1396-0296

VL - 2023

JO - Dermatologic Therapy

JF - Dermatologic Therapy

M1 - 3863357

ER -

Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

Abstract

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