Anti-p200 pemphigoid: diagnosis and treatment of a case presenting as an inflammatory subepidermal blistering disease.
Anti-p200 pemphigoid is a recently defined subepidermal immunobullous disease. It is characterized by the binding of circulating IgG autoantibodies to the dermal side of 1 M NaCl split skin and by reactivity of these autoantibodies to a unique 200-kd antigen on immunoblot of dermal extract. On immunoelectron microscopic examination, these autoantibodies deposit at the lamina lucida-lamina densa interface. We describe the clinical, histologic, and immunopathologic features in a patient with anti-p200 pemphigoid, as well as his favorable response to treatment with systemic glucocorticosteroids and dapsone.
Egan
Conleth A
CA
Dermatology Branch, Division of Clinical Sciences, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
Yee
Carole
C
Zillikens
Detlef
D
Yancey
Kim B
KB
D001323:Autoantibodies / Q000032:analysis / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000628:therapy; D001707:Biopsy, Needle; D003622:Dapsone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D016896:Treatment Outcome
D001323:Autoantibodies; D005938:Glucocorticoids; D003622:Dapsone