A Rare Case of Juvenile Pemphigus Vulgaris Treated with Intravenous Immunoglobulin.
- Author:
Sul Hee LEE
1
;
Ho Jung LEE
;
Young Lip PARK
;
Moon Kyun CHO
;
Sang Hoon LEE
Author Information
1. Department of Dermatology, Soonchunhyang University Bucheon Hospital, Bucheon, Korea. shlee@schmc.ac.kr
- Publication Type:Case Report
- Keywords:
Intravenous immunoglobulin;
Juvenile pemphigus vulgaris
- MeSH:
Adolescent;
Adrenal Cortex Hormones;
Adult;
Humans;
Immunoglobulins*;
Immunoglobulins, Intravenous;
Male;
Pemphigus*;
Sepsis
- From:Korean Journal of Dermatology
2019;57(1):36-39
- CountryRepublic of Korea
- Language:English
-
Abstract:
Juvenile pemphigus vulgaris (JPV) is a rare variant of pemphigus vulgaris (PV) occurring in childhood and adolescence that has similar symptoms and the same histological and immunopathological features as classic adult PV. Although rare, advanced cases of JPV can be fatal due to secondary sepsis. Many patients with JPV are misdiagnosed and therefore not properly treated in the early stages of the disease. Although systemic corticosteroids are the therapeutic mainstay, long-term corticosteroid use has various adverse effects. Intravenous immunoglobulin (IVIG) was recently reported to reduce the side effects of corticosteroids. Here, we report a case of JPV in a 14-year-old boy treated with IVIG.
