Journal article
Childhood epidermolysis bullosa acquisita.
- Inauen P Dermatological Clinic, University of Berne, Switzerland.
- Hunziker T
- Gerber H
- Bruckner-Tuderman L
- Braathen LR
- 1994-12-01
Published in:
- The British journal of dermatology. - 1994
Autoantibodies
Basement Membrane
Child
Dapsone
Drug Therapy, Combination
Epidermolysis Bullosa Acquisita
Fluorescent Antibody Technique
Humans
Immunoglobulin G
Male
Mouth Mucosa
Mucous Membrane
Prednisolone
English
We report a 6-year-old boy suffering from acquired epidermolysis bullosa, who presented with extensive lesions of the mucous membranes and disseminated, herpetiform and 'cluster of jewels'-like vesicles and bullae arising on erythematous plaques. Direct immunofluorescence showed linear deposits of IgG and C3 at the epidermal basement membrane zone. Indirect immunofluorescence demonstrated circulating autoantibodies (titre 1:128-1:256) directed at the blister floor of human NaCl-split skin, and reacting specifically with collagen VII of the anchoring fibrils, as demonstrated by immunoblotting. The disease was controlled with a combination of dapsone and prednisone. This case demonstrates the importance of modern immunological techniques in classifying childhood autoimmune bullous diseases, as precise diagnosis is important in determining an appropriate therapeutic regimen.
- Language
-
- English
- Open access status
- closed
- Identifiers
-
- DOI 10.1111/j.1365-2133.1994.tb08598.x
- PMID 7857848
- Persistent URL
- https://sonar.ch/global/documents/260421
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