Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.

No abstract

No abstract

A 26-year-old woman had a chronic vesiculopustular and ulcerating skin disease associated with fever and arthritis. Cutaneous biopsy specimens showed an extensive infiltration of the dermis and epidermis by neutrophils. Direct immunofluorescence (IF) revealed linear subcorneal IgA deposits. Indirect IF showed IgA antibodies reactive with the subcorneal zone of normal epidermis. The disease responded to dapsone therapy. The association between neutrophilic dermatoses, including pyoderma gangrenosum, subcorneal pustular dermatosis, and related entities, and IgA involvement, either IgA gammopathies and/or intraepidermal IgA deposits, is emphasized. Intraepidermal IgA deposits are possibly involved in the pathogenesis of our patient's condition and of other cases of unusual neutrophilic dermatoses.

No abstract