Literature DB >> 10530350

[Resistant acquired bullous epidermolysis with severe ocular involvement: the success of extracorporeal photochemotherapy].

A Camara1, P A Bécherel, A Bussel, S Lagrange, O Chosidow, P Joly, J C Piette, C Francès.   

Abstract

BACKGROUND: Epidermolysis bullosa aquisista can leave several functional sequelae. 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 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. DISCUSSION: This case demonstrates the efficacy of extracorporeal chemotherapy to be tried in case other treatments failed.

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Year:  1999        PMID: 10530350

Source DB:  PubMed          Journal:  Ann Dermatol Venereol        ISSN: 0151-9638            Impact factor:   0.777


  6 in total

Review 1.  Epidermolysis bullosa acquisita.

Authors:  Rishu Gupta; David T Woodley; Mei Chen
Journal:  Clin Dermatol       Date:  2012 Jan-Feb       Impact factor: 3.541

2.  Guidelines on the use of extracorporeal photopheresis.

Authors:  R Knobler; G Berlin; P Calzavara-Pinton; H Greinix; P Jaksch; L Laroche; J Ludvigsson; P Quaglino; W Reinisch; J Scarisbrick; T Schwarz; P Wolf; P Arenberger; C Assaf; M Bagot; M Barr; A Bohbot; L Bruckner-Tuderman; B Dreno; A Enk; L French; R Gniadecki; H Gollnick; M Hertl; C Jantschitsch; A Jung; U Just; C-D Klemke; U Lippert; T Luger; E Papadavid; H Pehamberger; A Ranki; R Stadler; W Sterry; I H Wolf; M Worm; J Zic; C C Zouboulis; U Hillen
Journal:  J Eur Acad Dermatol Venereol       Date:  2014-01       Impact factor: 6.166

Review 3.  Extracorporeal photopheresis as a therapy for autoimmune diseases.

Authors:  Zoya Kuzmina; David Stroncek; Steven Z Pavletic
Journal:  J Clin Apher       Date:  2014-12-26       Impact factor: 2.821

4.  Clinical presentation, pathogenesis, diagnosis, and treatment of epidermolysis bullosa acquisita.

Authors:  Ralf J Ludwig
Journal:  ISRN Dermatol       Date:  2013-07-15

Review 5.  Epidermolysis Bullosa Acquisita: The 2019 Update.

Authors:  Hiroshi Koga; Catherine Prost-Squarcioni; Hiroaki Iwata; Marcel F Jonkman; Ralf J Ludwig; Katja Bieber
Journal:  Front Med (Lausanne)       Date:  2019-01-10

6.  European dermatology forum: Updated guidelines on the use of extracorporeal photopheresis 2020 - Part 2.

Authors:  R Knobler; P Arenberger; A Arun; C Assaf; M Bagot; G Berlin; A Bohbot; P Calzavara-Pinton; F Child; A Cho; L E French; A R Gennery; R Gniadecki; H P M Gollnick; E Guenova; P Jaksch; C Jantschitsch; C Klemke; J Ludvigsson; E Papadavid; J Scarisbrick; T Schwarz; R Stadler; P Wolf; J Zic; C Zouboulis; A Zuckermann; H Greinix
Journal:  J Eur Acad Dermatol Venereol       Date:  2020-09-22       Impact factor: 9.228

  6 in total

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