Spectrum of Clinical Responses to Therapies in Infantile Bullous Pemphigoid

Taquin, H.; Chiavérini, C.; Lacour, Jean‐Philippe

doi:10.1111/pde.12779

Cited by 10 publications

(10 citation statements)

References 16 publications

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“…All our BP were in complete remission rapidly within an average of 7.25 months (6–9 months). Rarely few cases of literature reported relapses after discontinuation of treatment, with the longest duration of disease of 3 years after diagnosis. For pemphigus, relapses were observed in all our patients with a mean delay of 8.75 months and these relapses could be late (up to 34 months).…”

Section: Discussionmentioning

confidence: 99%

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

Welfringer‐Morin

Bekel

Bellon

et al. 2019

Acad Dermatol Venereol

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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.

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Section: Discussionmentioning

confidence: 99%

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

Welfringer‐Morin

Bekel

Bellon

et al. 2019

Acad Dermatol Venereol

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“…2 It affects mainly elderly individuals, during the 8 th decade of life, without gender predilection, with rare case reports of BP in children and adolescents. 3 , 4…”

Section: Epidemiologymentioning

confidence: 99%

Bullous pemphigoid

Miyamoto

Santi

Aoki

et al. 2019

An. Bras. Dermatol.

174

181

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Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.

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“…Treatment with oral prednisone was effective in achieving disease control in two out of three patients; the third one was treated with a combination of systemic corticosteroids and Tacrolimus and subsequently with intravenous immunoglobulin and plasmapheresis, due to a complex underlying autoimmune disease. BP is an uncommon autoimmune skin disorder in infancy, although recently some cases have been reported after vaccinations or viral infections [ 21 , 22 ]. In most cases it shows prominent palmoplantar involvement and responds well to systemic steroid therapy, even if recognizing it promptly is important to establish appropriate treatment and prevent infectious complications which may be common and severe.…”

Section: Discussionmentioning

confidence: 99%

Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger

et al. 2017

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BackgroundBullous pemphigoid (BP) is a blistering skin disorder infrequent in infancy and rarely reported in medical literature.Case PresentationHere we describe three cases of BP which were referred to our department in the last 15 years. Two of them developed an eruption of bullous lesions just a few days after vaccination for diphtheria, tetanus, pertussis, poliomyelitis, hepatitis B and Haemophilus influenzae B. The third patient developed the same blistering lesions shortly after herpetic stomatitis. In all three cases, clinical diagnosis was confirmed by histological examination which showed subepidermal bullae with a dermal inflammatory infiltrate, and direct immunofluorescence of perilesional skin showed linear IgG and C3 deposits along the basement membrane zone. Immunoblot assay was positive for BP antigen 180. Treatment with oral prednisone was instituted and the lesions resolved in two out of three patients; the third one was treated with an immunosuppressive agent (tacrolimus) and corticosteroid and subsequently with intravenous immunoglobulin and plasmapheresis, due to an underlying complex autoimmune disease.ConclusionAlthough the mechanism of induction of BP is still unclear, the close relationship between trigger events (immunization or viral infection) and onset of the disease arises a possible association.

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Spectrum of Clinical Responses to Therapies in Infantile Bullous Pemphigoid

Cited by 10 publications

References 16 publications

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

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

Bullous pemphigoid

Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger

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