Long‐term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children

Abstract

Background Autoimmune bullous dermatoses (AIBDs) in children are uncommon, and their long‐term evolution remains unknown. Objective The aim of this retrospective study was to characterize the long‐term prognosis of AIBDs that started during childhood. Methods We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBDs. The long‐term outcome was obtained through a phone call questionnaire. Results Sixty‐three patients were included from January 1993 to December 2015, 34 female and 29 males: 27 Linear immunoglobulin A disease (LAD), 12 bullous pemphigoid (BP), 12 pemphigus, 8 herpetiform dermatitis (DH) and 4 epidermolysis bullosa aquisita (EBA). The mean age was 4.7 years old. Twenty‐five patients were lost during the follow‐up. For the 38 remaining patients, the mean follow‐up duration for all pathologies was 6.6 years. Twenty‐nine of them had at least one relapse. Late relapses were observed in two cases of DH and six cases of pemphigus (7–34 months). The mean treatment duration was 30.6 months with variability according to the AIBDs. Topical corticosteroids were used alone, effectively, for seven patients and in association with other treatment in 19 patients in complete remission. Complete remission was noted in 34/38 children with a follow‐up of 4.4 years (0.08–19.5). The mean duration to complete remission was 30.5 months (6–114 months). Late nasal synechiae were reported in one EBA only. There was no significant associated comorbidity, but an association with a primary immune deficiency (PID) was observed in two cases. Conclusion Childhood AIBDs appear to be of good overall prognosis but a long‐term follow‐up is mandatory, as relapses can be late, except for BP. The use of topical corticosteroids is frequently effective alone or in association. The association with PID leads to think about the possibility of a possible underlying dysimmunity in the child.

Spectrum of autoimmune bullous diseases among children in Kuwait

“…Case 20 15 1998-2012 (15) 1976-2007 (31) 1983-2000 (17) 1991-1994 31981-1997 (16) Place of study Table 2 summarizes and compares our data to that of prior series in the English literature. [4][5][6][7][8][9][10] Similar to the majority of prior series, LABD was the most prevalent AIBD in our cohort, occurred more commonly in male children, and was most often treated with dapsone alone or in combination with prednisolone. [4][5][6][7][8][9][10] We also used high dose IVIG in 4 of our LABD patients with moderate-to-severe disease in whom dapsone was contraindicated.…”

“…[4][5][6][7][8][9][10] Similar to the majority of prior series, LABD was the most prevalent AIBD in our cohort, occurred more commonly in male children, and was most often treated with dapsone alone or in combination with prednisolone. [4][5][6][7][8][9][10] We also used high dose IVIG in 4 of our LABD patients with moderate-to-severe disease in whom dapsone was contraindicated. In our experience, Only one prior series by Weston et al 10 noted a prevalence of DH higher than LABD.…”

Review of autoimmune blistering diseases: the Pemphigoid diseases

“…Long term monitoring is recommended given the potential risk of relapse. 5 With the advent of validated scoring systems, it is possible to objectively and subjectively monitor patients' progress and conduct clinical trials with newer therapeutic agents. [6][7][8][9]…”

“…Autoimmune Blistering Diseases occurring in children can usually be managed with topical steroids with a good prognosis. Long term monitoring is recommended given the potential risk of relapse …”

Erythematous Plaques and Tense Bullae in an Infant

“…Despite its relative rarity, infantile BP has been reported, after chronic bullous disease of childhood, to be the second most common immunobullous disease in infants with more than 80 reported cases [4,5]. There is an unusual predominance of cases of BP before the first year of age, with a median age of diagnoses in infants of four months [6].…”