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Childhood, neonatal, and stillborn pemphigus vulgaris
1Department of Dermatology, Karolinska University Hospital, Faculty of Odontology, Karolinska Institute, Stockholm, Sweden.
Insights
Pemphigus vulgaris can occur in children, newborns, and stillborn infants, often due to maternal antibodies. Early diagnosis and reporting are crucial for understanding childhood pemphigus vulgaris treatment and outcomes.
Area of Science:
- Dermatology
- Pediatrics
- Immunology
Background:
- Pemphigus vulgaris, a rare autoimmune blistering disease, can manifest in pediatric populations, including neonatal and stillborn cases.
- Review of literature identified 46 childhood, 9 neonatal, and 3 stillborn cases of pemphigus vulgaris.
Purpose of the Study:
- To review and analyze reported cases of pemphigus vulgaris in childhood, neonatal, and stillborn infants.
- To highlight the importance of physician awareness for early diagnosis and timely treatment of pediatric pemphigus vulgaris.
Main Methods:
- Systematic literature review of pemphigus vulgaris cases in pediatric populations.
- Analysis of case demographics, age of onset, treatment modalities, and outcomes.
Main Results:
- Childhood pemphigus vulgaris typically presents around age 12, with similar sex distribution. Corticosteroids and azathioprine are common treatments, with a low reported fatality rate but unknown long-term prognosis.
- Neonatal cases show an excellent prognosis, and both neonatal and stillborn cases are likely due to transplacental antibody transmission. Maternal factors and immunosuppressive treatments may influence fetal survival.
- Stillborn cases occurred in the third trimester; the link between maternal antibody titers and fetal mortality remains unclear.
Conclusions:
- Physician awareness is critical for early diagnosis and to prevent treatment delays in childhood pemphigus vulgaris.
- Further case reports with long-term follow-up are needed to establish optimal treatment strategies for pediatric pemphigus vulgaris.
- The potential for fetal disease development should be considered in pregnant women with a history of pemphigus vulgaris or those with at-risk family members.
Background:
Childhood, neonatal, and stillborn cases of pemphigus vulgaris were reviewed.
Methods:
From an examination of the pemphigus vulgaris literature, 46 childhood cases, nine neonatal cases, and three stillborn cases were found and investigated.
Results:
In the childhood cases, the ratio between the sexes is approximately the same. The mean age of onset is 12 years, with only 11 children being 10 years of age or younger. Some children were treated with adjuvants to corticosteroids, most of them with azathioprine. While only one childhood case has been reported as fatal, the long-term prognosis and the relationship of early treatment and outcome are unknown. Neonatal prognosis, however, is excellent. Both neonatal and stillborn cases are probably the result of transplacental transmission of maternal antibodies. The connection between maternal antibody titer and fetal mortality is unknown. All stillborn cases reviewed died during the eight month of gestation. Immunosuppressive treatment probably affects fetal survival. In women with an active disease, who have had the disease, or who are monozygotic siblings to such patients, the possibility of the fetus developing the disease must be considered.
Conclusions:
It is essential that physicians be aware of the existence of childhood pemphigus vulgaris in order to make an early diagnosis and to avoid treatment delay. More childhood case reports are needed to obtain better information on optimal treatment, and authors should be encouraged to report the follow-up of their cases.