Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha3.
BACKGROUND
The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. Patients with inflammatory EBA have IgG autoantibodies against type VII collagen. Patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5.


METHODS
We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha3. A final diagnosis of EBA was based on the sublamina densa level of blister formation.


CONCLUSIONS
This case illustrates the clinical and immunological overlap between EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.
Jonkman
M F
MF
Department of Dermatology, University of Groningen, The Netherlands. m.f.jonkman@derm.azg.nl
Schuur
J
J
Dijk
F
F
Heeres
K
K
de Jong
M C
MC
van der Meer
J B
JB
Yancey
K B
KB
Pas
H H
HH
D001323:Autoantibodies / Q000032:analysis* / Q000276:immunology; D003094:Collagen / Q000276:immunology*; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D005148:Facial Dermatoses / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis* / Q000276:immunology; D007249:Inflammation; D007797:Laminin / Q000276:immunology*; D008856:Microscopy, Fluorescence; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D009092:Mucous Membrane / Q000276:immunology; D011233:Precipitin Tests; D012867:Skin / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure
D001323:Autoantibodies; D007074:Immunoglobulin G; D007797:Laminin; C095567:laminin alpha 3; D003094:Collagen