Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus

Pemphigus is a rare and life-threatening autoimmune disease that is easily ignored in children. This study aimed to attract attention to pemphigus in the paediatric population for better understanding and recognition of the clinical characteristics and management in children’s different subtypes of...

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Main Authors: An-Wei Chen, Li Liu, Huan Yang, Xiao-Yan Luo, Juan Xiang
Format: Article
Language:English
Published: Medical Journals Sweden 2025-03-01
Series:Acta Dermato-Venereologica
Subjects:
Online Access:https://medicaljournalssweden.se/actadv/article/view/42776
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author An-Wei Chen
Li Liu
Huan Yang
Xiao-Yan Luo
Juan Xiang
author_facet An-Wei Chen
Li Liu
Huan Yang
Xiao-Yan Luo
Juan Xiang
author_sort An-Wei Chen
collection DOAJ
description Pemphigus is a rare and life-threatening autoimmune disease that is easily ignored in children. This study aimed to attract attention to pemphigus in the paediatric population for better understanding and recognition of the clinical characteristics and management in children’s different subtypes of pemphigus. The case series involved a retrospective record review of 3 patients who were referred to a department of dermatology from January 2022 to January 2024. All patients experienced a delayed diagnosis, but were eventually definitively diagnosed with pemphigus vulgaris, pemphigus foliaceus, and pemphigus erythematosus, because they all have intraepidermal blisters and elevated serum antibodies against Dsg1/Dsg3. In all 3 cases, systemic corticosteroids were used in combination with or without steroid-sparing adjuvants. The patient with pemphigus vulgaris experienced a recurrence due to discontinuous treatment; the other 2 patients achieved mostly remission after treatment, with the length of follow-up ranging from 5 to 12 months. Pemphigus in children has its own clinical characteristics and similar literature concerning improved strategies for clinical assessment and treatments are reviewed and discussed.
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publisher Medical Journals Sweden
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series Acta Dermato-Venereologica
spelling doaj-art-fd7b926599984b32a5644ad7df60fa7b2025-08-20T02:58:39ZengMedical Journals SwedenActa Dermato-Venereologica0001-55551651-20572025-03-0110510.2340/actadv.v105.42776Clinical Features, Diagnosis and Treatment of Different Types of Paediatric PemphigusAn-Wei Chen0Li Liu1Huan Yang2Xiao-Yan Luo3Juan Xiang4Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, ChinaDepartment of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, ChinaDepartment of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, ChinaDepartment of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, ChinaDepartment of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, ChinaPemphigus is a rare and life-threatening autoimmune disease that is easily ignored in children. This study aimed to attract attention to pemphigus in the paediatric population for better understanding and recognition of the clinical characteristics and management in children’s different subtypes of pemphigus. The case series involved a retrospective record review of 3 patients who were referred to a department of dermatology from January 2022 to January 2024. All patients experienced a delayed diagnosis, but were eventually definitively diagnosed with pemphigus vulgaris, pemphigus foliaceus, and pemphigus erythematosus, because they all have intraepidermal blisters and elevated serum antibodies against Dsg1/Dsg3. In all 3 cases, systemic corticosteroids were used in combination with or without steroid-sparing adjuvants. The patient with pemphigus vulgaris experienced a recurrence due to discontinuous treatment; the other 2 patients achieved mostly remission after treatment, with the length of follow-up ranging from 5 to 12 months. Pemphigus in children has its own clinical characteristics and similar literature concerning improved strategies for clinical assessment and treatments are reviewed and discussed. https://medicaljournalssweden.se/actadv/article/view/42776Pemphigus vulgarisPemphigus foliaceusPemphigus erythematosusChildrenTreatment
spellingShingle An-Wei Chen
Li Liu
Huan Yang
Xiao-Yan Luo
Juan Xiang
Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
Acta Dermato-Venereologica
Pemphigus vulgaris
Pemphigus foliaceus
Pemphigus erythematosus
Children
Treatment
title Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
title_full Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
title_fullStr Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
title_full_unstemmed Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
title_short Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
title_sort clinical features diagnosis and treatment of different types of paediatric pemphigus
topic Pemphigus vulgaris
Pemphigus foliaceus
Pemphigus erythematosus
Children
Treatment
url https://medicaljournalssweden.se/actadv/article/view/42776
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