[Resistant acquired bullous epidermolysis with severe ocular involvement: the success of extracorporeal photochemotherapy].
BACKGROUND
Epidermolysis bullosa aquisista can leave several functional sequelae. The lesions sometimes resist treatment.


METHODS
We report a case of a 25-year-old young man presenting with a severe epidermolysis bullosa acquisita confirmed by the electronic immunomicroscopy. He had a major ocular involvement with symblepharon and cicatricial synechial lesions. He was almost blind because of corneal scars. All immunosuppressive treatments had failed: systemic corticoids, cyclosporin, azathioprine. 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 relapse of the disease since 9 months. Corneal grafts are now under process, to try to recover a part of the lost visual acuity.


CONCLUSIONS
This case demonstrates the efficacy of extracorporeal chemotherapy to be tried in case other treatments failed.
Camara
A
A
Service de Dermatologie et Vénéréologie, CHU Donka, Guinée.
Bécherel
P A
PA
Bussel
A
A
Lagrange
S
S
Chosidow
O
O
Joly
P
P
Piette
J C
JC
Francès
C
C
D000328:Adult; D002921:Cicatrix / Q000473:pathology; D003229:Conjunctival Diseases / Q000473:pathology; D003316:Corneal Diseases / Q000473:pathology; D016107:Epidermolysis Bullosa Acquisita / Q000188:drug therapy* / Q000473:pathology; D005128:Eye Diseases / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D016253:Microscopy, Immunoelectron; D017893:Photopheresis; D000267:Tissue Adhesions / Q000473:pathology; D017211:Treatment Failure; D016896:Treatment Outcome; D014945:Wound Healing
D007166:Immunosuppressive Agents