Immune checkpoint inhibitor-induced bullous pemphigoid effectively controlled by dupilumab

Mastocytosis encompasses a heterogeneous group of clonal mast cell disorders ranging from cutaneous forms in children to advanced systemic mastocytosis (AdvSM) with significant morbidity. This review covers epidemiology, pathogenesis, diagnosis, and management, integrating World Health Organization (WHO)/International Consensus Classification (ICC) 2022 classification updates, molecular insights, and therapeutic advances. Pediatric cutaneous mastocytosis (CM) usually has a favourable prognosis and often resolves spontaneously, whereas adult-onset disease frequently reflects systemic involvement and warrants bone marrow biopsy. KIT D816V and non-D816V variants serve as key diagnostic and prognostic markers, guiding targeted therapy. Midostaurin and avapritinib have reshaped the treatment of AdvSM, while next-generation tyrosine-kinase inhibitors (TKIs) are in clinical trials. Multidisciplinary care is critical, and knowledge gaps remain in paediatric risk stratification, optimal sequencing of therapies, and disease progression assessment. This review highlights recent advances and future priorities for personalized, evidence-based care.

Authors

Publication Details

Journal
European Journal of Dermatology
Published
2025-12-01
DOI
https://doi.org/10.1684/ejd.2025.5007
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Immune checkpoint inhibitor-induced bullous pemphigoid effectively controlled by dupilumab

Jan-Hendrik Maiwald, Anja Gesierich, Valerie Glutsch, Simon Goller et al.
European Journal of Dermatology
Autoimmune Bullous Skin Diseases
article

Immune checkpoint inhibitor-induced bullous pemphigoid effectively controlled by dupilumab

Jan-Hendrik Maiwald, Anja Gesierich, Valerie Glutsch, Simon Goller, Matthias Goebeler
article en

Abstract

Mastocytosis encompasses a heterogeneous group of clonal mast cell disorders ranging from cutaneous forms in children to advanced systemic mastocytosis (AdvSM) with significant morbidity. This review covers epidemiology, pathogenesis, diagnosis, and management, integrating World Health Organization (WHO)/International Consensus Classification (ICC) 2022 classification updates, molecular insights, and therapeutic advances. Pediatric cutaneous mastocytosis (CM) usually has a favourable prognosis and often resolves spontaneously, whereas adult-onset disease frequently reflects systemic involvement and warrants bone marrow biopsy. KIT D816V and non-D816V variants serve as key diagnostic and prognostic markers, guiding targeted therapy. Midostaurin and avapritinib have reshaped the treatment of AdvSM, while next-generation tyrosine-kinase inhibitors (TKIs) are in clinical trials. Multidisciplinary care is critical, and knowledge gaps remain in paediatric risk stratification, optimal sequencing of therapies, and disease progression assessment. This review highlights recent advances and future priorities for personalized, evidence-based care.

European Journal of DermatologyVol. 35(6)
Zero hunger
Openalex Percentile: Top 26%
Autoimmune Bullous Skin Diseases
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.