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.
A randomized, double-blind, phase III, multicentre study to evaluate the safety and efficacy of BF-200 ALA (Ameluz â) vs. placebo in the field-directed treatment of mild-tomoderate actinic keratosis with photodynamic therapy (PDT) when using the BF-RhodoLED â lamp.
To our knowledge, this is the first nationwide study to characterize and determine the prevalence of HF-BCC patients. Similar to the more typical patients with 1-2 BCCs, HF-BCC patients have an increasing number of BCCs with increased age. 2 Our data suggest that the HF-BCC population develops BCCs continuously; therefore, most HF-BCC patients should have been captured during the catchment period and the limited-duration prevalence is a good estimate of total prevalence. This HF-BCC prevalence is more than 15-fold higher than the highest population prevalence reported for Gorlin syndrome patients (3.2/ 100 000), and thus any inadvertent inclusion of Gorlin syndrome patients in this study would have a relatively small effect on the estimated frequency. 8 This study shows a high prevalence and consistent burden of disease for HF-BCC patients, warranting the need for alternate methods of care for and monitoring of this patient population. Further research into this population and potential risk factors would be beneficial for physicians to develop diagnostic and treatment guidelines for those at highest risk.
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