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
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.