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

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.

Keywords

• Pemphigus vulgaris
• Pemphigus foliaceus
• Pemphigus erythematosus
• Children
• Treatment