Acquired bullous diseases of childhood: re-evaluation of diagnosis by indirect immunofluorescence examination on 1 M NaCl split skin and immunoblotting

Abstract

Acquired autoimmune bullous diseases of childhood are rare, and can be difficult to distinguish clinically. We have studied 12 children, with an initial diagnosis of bullous pemphigoid (BP) in eight patients, cicatricial pemphigoid (CP) in one, chronic bullous disease of childhood (CBDC) in one, and epidermolysis bullosa acquisita (EBA) in two. All patients had positive indirect immunofluorescence (IIF) of the BMZ with IgG. Using 1 M NaCl split skin, six patients showed epidermal binding of IgG, with additional IgA in three cases, and in five patients IgG antibodies bound a dermal protein. Immunoblotting studies revealed an antibody to type VII collagen (EBA antigen) in three patients who had a dermal pattern on IIF. Six sera reacted with an epidermal protein of 180 and/or 220 kDa, characteristic of BP and CP. One of the three IgA-positive sera detected 220- and 180-kDa epidermal proteins using anti-IgA antibody. Following these studies the diagnosis was changed in three of the children. The diagnosis of CBDC was changed to either BP or EBA because of the presence of circulating IgG autoantibodies. In two children with an initial diagnosis of BP the diagnosis was changed to EBA. We conclude that the clinical picture in bullous disorders of childhood shows considerable overlap, and is often misleading. Additional circulating IgA autoantibodies seem to be more common in BP than has been recognized previously. Indirect immunofluorescence investigation on 1 M NaCl split skin may be helpful in differentiating between BP and EBA, but does not replace immunoblotting studies. EBA is apparently more common in children than in adults. No difference was found between the children with BP and EBA with regard to the duration of disease. The long-term outlook is good, although the course may be protracted.

Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects

“…Vulvar involvement in BP is described in 9% of adult patients and 40% of children [53][54][55]. Vulvar BP, instead, is exclusively localized and confined to the vulva, with self-limited, non-scarring lesions.…”

Infantile bullous pemphigoid treated with intravenous immunoglobulin therapy

“…Corticosteroid therapy was effective in 8 cases, 2-4,11,13-16 6 cases were successfully treated with a combination of corticosteroid and dapsone, 17-21 and 4 cases with a combination of corticosteroid and sulfapyridine. 1,12,22,23 Two cases were cured with sulfapyridine alone. 24,25 Other drugs used in combination with systemic corticosteroids include erythromycin, nicotinamide, or mycophenolate mofetil.…”

Interventions for Mucous Membrane Pemphigoid/Cicatricial Pemphigoid and Epidermolysis Bullosa Acquisita

“…Most children were treated with systemic corticosteroids and/or dapsone. 41,[48][49][50] In children, EBA seems to remit within a few years, and it is not possible to judge if this is due to treatment or represents a spontaneous remission.…”

“…9 We identified 11 articles on treatment in EBA involving 2 or more patients, detailing results in 20 adults and 11 children. 10,[41][42][43][44][45][46][47][48][49][50] The adult patients were treated with various medications including systemic corticosteroids, immunosuppressants, dapsone, colchicine, and intravenous immunoglobulins; it is not possible to draw any conclusion regarding the superiority of any of these treatments. Most children were treated with systemic corticosteroids and/or dapsone.…”