Epidermolysis bullosa acquisita: diagnosis by fluorescence overlay antigen mapping and clinical response to high-dose intravenous immunoglobulin.
Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.
Campos
M
M
Department of Dermatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain. miniacampos@gmail.com
Silvente
C
C
Lecona
Manuel
M
Suárez
R
R
Lázaro
P
P
D000906:Antibodies / Q000032:analysis; D001485:Basement Membrane / Q000276:immunology; D004334:Drug Administration Schedule; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D019085:Fluorescent Antibody Technique, Direct / Q000379:methods; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000276:immunology; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000276:immunology; D008875:Middle Aged; D016896:Treatment Outcome
D000906:Antibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors