Chronic bullous dermatosis of childhood-clinical and immunological features seen in African patients

Aboobaker, Jamila; Wojnarowska, Fenella; Bhogal, B.; Black, M.M.

doi:10.1111/j.1365-2230.1991.tb00336.x

Cited by 52 publications

(34 citation statements)

References 8 publications

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“…89% of our population. For LAD, the prognosis was good, in accordance with the literature, while in adult disease, only one‐third of patients had a complete remission . In our study, all the evaluated patients, except one, were in complete remission in <2 years, and only four patients were still under treatment.…”

Section: Discussionsupporting

confidence: 90%

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: Discussionsupporting

confidence: 90%

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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“…Although clinical trials are lacking, the treatment of choice is dapsone with or without systemic corticosteroids. 80,81 For a review of dosing, side effects, and monitoring of dapsone, please refer to the previous section, which describes the use of dapsone in the treatment of BP. Other reported treatments include colchicine, 82–86 trimethoprin-sulfamethoxazole, 87 tacrolimus ointment, 88 mycophenolate, 89 immunoadsorption, 90 and IVIg.…”

Section: Linear Iga Diseasementioning

confidence: 99%

Treatment of subepidermal immunobullous diseases

Culton

Díaz

2012

Clinics in Dermatology

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The subepidermal immunobullous diseases are a group of autoimmune blistering disorders of the skin and mucous membranes that share the common features of autoantibody deposition and blister formation at the dermal-epidermal junction or basement membrane. This group includes bullous pemphigoid, linear IgA disease, dermatitis herpetiformis, and epidermolysis bullosa acquisita, among others. Although these disorders share some common features, each disease is unique in its clinical presentation, histopathology, and immunofluorescence patterns, which allows for accurate diagnosis and disease-specific treatment strategy. Treatment of these disorders is complex and requires expert knowledge of disease pathogenesis. We review common treatment approaches for each of these disorders.

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“…[4] The prevalence in children has rarely been reported but its incidence is reported to be high in South Africa, North Africa, and Asia. [25–7] Taking into account the previous reported series, our series accounting 31 cases of LAD in 32 years is to our knowledge among the highest frequencies ever reported. [67] The reason of the difference between the frequencies of two series needs to be determined.…”

Section: Discussionmentioning

confidence: 99%

“…These differences were also reported by other authors from African and Asian countries. [56] Molecular and genetic studies may be helpful to prιcis the existence of specificities of this disease in African countries.…”

Section: Discussionmentioning

confidence: 99%

Linear IGA bullous dermatosis in Tunisian children : 31 cases

Kharfi¹,

Khaled²,

Karaa³

et al. 2011

Indian J Dermatol

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Background:Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa.Aim:We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children.Materials and Methods:We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population.Results:Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal–epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients.Conclusion:This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.

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Chronic bullous dermatosis of childhood-clinical and immunological features seen in African patients

Cited by 52 publications

References 8 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

Treatment of subepidermal immunobullous diseases

Linear IGA bullous dermatosis in Tunisian children : 31 cases

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