Resistant epidermolysis bullous acquisita with severe ocular involvement: successful extracorporeal photochemotherapy

Citation
A. Camara et al., Resistant epidermolysis bullous acquisita with severe ocular involvement: successful extracorporeal photochemotherapy, ANN DER VEN, 126(8-9), 1999, pp. 612-615
Citations number
17
Categorie Soggetti
Dermatology
Journal title
ANNALES DE DERMATOLOGIE ET DE VENEREOLOGIE
ISSN journal
01519638 → ACNP
Volume
126
Issue
8-9
Year of publication
1999
Pages
612 - 615
Database
ISI
SICI code
0151-9638(199908/09)126:8-9<612:REBAWS>2.0.ZU;2-G
Abstract
Background. Epidermolysis bullosa aquisista can leave several functional se quelae. The lesions sometimes resist treatment, Case report. We report a case of a 25-year-old young man presenting with a severe epidermolysis bullosa acquisita confirmed by the electronic immunomi croscopy. He had a major ocular involvement with symblepharon and cicatrici al synechial lesions, He was almost blind because of corneal scars. Ail imm unosuppressive treatments had failed: systemic corticoids, cyclosporin, aza thioprine. The introduction of extracorporeal photochemotherapy resulted in the healing of the lesions, after a total of 32 procedures. All treatment are now stopped, and the lesions are purely cicatricial, without any relaps e of the disease since 9 months. Corneal grafts are now under process, to t ry to recover a part of the lost visual acuity. Discussion, This case demonstrates the efficacy of extracorporeal chemother apy to be tried in case other treatments faile.