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Updated: May 23, 2025
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus
An-Wei Chen1, Li Liu1, Huan Yang1
1Department 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, China.
Insights
Pemphigus in children is a rare autoimmune disease often overlooked. This study highlights its unique pediatric characteristics and effective management strategies for better recognition and treatment.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Pediatrics
Background:
- Pemphigus is a rare, life-threatening autoimmune condition.
- Pediatric pemphigus is often overlooked, necessitating increased awareness.
- Understanding clinical characteristics and management in children is crucial.
Purpose of the Study:
- To raise awareness of pemphigus in the pediatric population.
- To detail the clinical characteristics and management of pediatric pemphigus subtypes.
- To review and discuss improved strategies for assessment and treatment.
Main Methods:
- Retrospective case series review of 3 pediatric patients.
- Involved patients diagnosed with pemphigus vulgaris, foliaceus, and erythematosus.
- Data collected from January 2022 to January 2024.
Main Results:
- All 3 patients experienced delayed diagnosis.
- Diagnosis confirmed by intraepidermal blisters and elevated anti-Dsg1/Dsg3 antibodies.
- Treatment involved systemic corticosteroids with or without steroid-sparing agents.
Conclusions:
- Pemphigus in children presents distinct clinical features.
- One patient with pemphigus vulgaris recurred due to inconsistent treatment.
- Two patients achieved remission; further research into pediatric pemphigus is warranted.
Abstract:
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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