Juvenile bullous pemphigoid: the presentation and follow-up of six cases.

BACKGROUND

Juvenile bullous pemphigoid (JBP) is a very rare autoimmune blistering disease. Up to now, 81 cases have been published.

OBJECTIVES

To describe clinical, histopathological and immunopathological characteristics of our patients with JBP, their response to therapy and clinical course, and to show the rarity of JBP in our population.

STUDY

DESIGN

Retrospective study of all patients with JBP diagnosed from 1983 to the end of 2007. The patients were followed from 6 months to 24 years.

SETTING

An academic, teaching hospital - Pediatric Dermatology Unit of the Institute of Dermatovenereology, Clinical Center of Serbia, Belgrade.

PATIENTS AND

METHODS

Six patients with JBP (4 girls and 2 boys) aged 2 to 17 years. The average age at onset of BP was 10 years. The diagnosis was confirmed by histopathological analysis and direct immunofluorescence test. The patients were treated with systemic and topical corticosteroids, and three patients received dapsone.

RESULTS

The disease control was achieved after 2 weeks to 2.5 months of therapy. The treatment lasted from 2 weeks to 4.5 months, 1.8 months at the average. The period from the beginning of the disease to complete remission ranged from 1.5 month to 5 years, with a mean value of 14 months. In one boy, the parents interrupted the therapy, and the disease remitted spontaneously after 5 years.

CONCLUSION

According to our experience, JBP can spontaneously remit within 5 years. JBP has a relatively indolent course and may be a self-limiting disease. The patients should not be over-treated in order to avoid side-effects of medication.