Childhood epidermolysis bullosa acquisita. Detection in a 5-year-old girl

Epidermolysis Bullosa Acquisita in Childhood

“…Histopathologic findings in our case showed a subepidermal blister, papillary inflammation, and a predominance of neutrophils suggestive of LAD or dermatitis herpetiformis; however, direct immunofluorescence of the perilesional skin showed BMZ deposits with anti-IgG and anti-C3 deposits, features of either BP, EBA, or BSLE (21,22). DIF findings reported in the 30 cases of EBA in children revealed deposits at the BMZ with anti-IgG and anti-C3 conjugates in 24 cases, anti-IgG and anti-IgA in five cases, and anti-IgM in six cases (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20).…”

“…Epidermolysis bullosa acquisita (EBA) is a relatively uncommon disease, with an incidence of two new cases per 10 million per year in Europe (19). EBA in childhood is rare; the literature reports 30 cases to date (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20).…”

“…Epidermolysis bullosa acquisita (EBA) is a blistering disease in which autoantibodies directed to type VII collagen, a major component of the anchoring fibrils, lead to blister formation at the basement membrane zone (1)(2)(3). The occurrence of EBA in childhood is considered to be rare; our review of the literature describes 30 cases of childhood EBA (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). EBA is believed to have a better prognosis in children than in adults (8, 10,11,15,16,18).…”

Epidermolysis bullosa acquisita in childhood

“…[3][4][5][6][7][8] Epidermolysis bullosa acquisita in childhood 39 The age of onset ranges from 3 months to 12 years. Of 13 cases, there were nine girls and four boys.…”

“…Of 13 cases, there were nine girls and four boys. [3][4][5][6][7][8][9] Delay in diagnosis and treatment has been a problem because of incorrect assessment. Clinical subdivisions of epidermolysis bullosa acquisita have been based on adult patterns, although children seem to have more inflammatory disease.…”