Childhood, neonatal, and stillborn pemphigus vulgaris

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.