In:
Journal of the European Academy of Dermatology and Venereology, Wiley, Vol. 33, No. 6 ( 2019-06), p. 1158-1163
Abstract:
Autoimmune bullous dermatoses ( AIBD s) 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 AIBD s that started during childhood. Methods We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBD s. 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 AIBD s. 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 AIBD s 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.
Type of Medium:
Online Resource
ISSN:
0926-9959
,
1468-3083
DOI:
10.1111/jdv.2019.33.issue-6
Language:
English
Publisher:
Wiley
Publication Date:
2019
detail.hit.zdb_id:
2022088-1
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