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Ocular involvement in epidermolysis bullosa acquisita with long-term follow-up
  1. Antoine Rousseau1,2,
  2. Catherine Prost-Squarcioni3,4,
  3. Serge Doan5,6,
  4. Christelle Leroux-Villet3,
  5. Frédéric Caux3,
  6. Than Hoang-Xuan5,6,
  7. Isabelle Cochereau5,6,
  8. Eric Gabison6
  1. 1Ophthalmology, Hopital Bicêtre, Assistance Publique-Hôpitaux de Paris; Université Paris-Sud, Le Kremlin-Bicêtre, France
  2. 2Immunology of viral infections and auto-immune diseases (IMVA) / IDMIT / U1184, CEA, Fontenay-aux-Roses, France
  3. 3Dermatology, Hopital Avicennes, Assistance Publique-Hôpitaux de Paris; Université Paris XIII, Bobigny, France
  4. 4Histology, Hopital Avicenne, Assistance Publique-Hôpitaux de Paris, Université Paris XIII, Bobigny, France
  5. 5Ophthalmology, Hopital Bichat-Claude-Bernard, Assistance Publique-Hôpitaux de Paris, Université Paris VII, Paris, France
  6. 6Cornea, External Disorders and Refractive Surgery, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
  1. Correspondence to Professor Eric Gabison, Cornea, external disorders and refractive surgery, Fondation Ophtalmologique Adolphe de Rothschild, Paris 55659, France; eegabison{at}gmail.com

Abstract

Background/aims To describe the ocular manifestations associated with epidermolysis bullosa acquisita (EBA).

Methods This retrospective study was conducted at a tertiary bullous disease clinic. Consecutive patients were enrolled with biopsy proven diagnosis of EBA, with ocular involvement and a follow-up of at least 36 months. A multidisciplinary team of dermatologists, ENT specialists and ophthalmologists evaluated all patients. Immunological workup included direct (including immune-electron microscopy) and indirect immunofluorescence. Ophthalmological examination included best-corrected visual acuity (BCVA) and slit-lamp examination with grading of conjunctival fibrosis using the Tauber classification.

Results Nine patients (five females, four males) were included. The mean age at diagnosis was 32 years (range 1–52 years). Follow-up ranged from 3 to 18 years (mean 10.7 years). Conjunctival fibrosis was present in all affected eyes and was stage III or greater in 60% of patients. Eight patients (14 eyes) had corneal involvement most frequently associated with trichiasis-associated mechanical irritation or extensive cicatrising conjunctivitis. Corneal lesions developed on three eyes of three patients without eyelid disease or severe fibrosis or any identifiable triggering factor. Eyelids were affected in six patients, with trichiasis being the most common feature (affecting three patients, four eyes). Corneal-related blindness occurred in at least one eye in 44% of the patients.

Conclusion EBA may be associated with devastating ocular manifestations. Most patients develop severe cicatrising conjunctivitis. A subset of patients may present with isolated corneal lesions. Further studies are warranted to assess the effects of systemic treatments on the evolution of ocular manifestations.

  • epidermolysis bullosa acquisita
  • cicatrizing conjunctivitis
  • conjunctival fibrosis
  • mucous membrane pemphigoid
  • linear iga disease

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Footnotes

  • Contributors AR, CPS and EEG participated in drafting the manuscript, study design, data collection and screening and hypothesis generation. SD and CLV participated in literature search, data collection and drafting the manuscript. All authors participated in data analysis, evidence synthesis, revising the manuscript and have given final approval of this version to be published

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval This study was approved by the Ethics Committee of the French Society of Ophthalmology (IRB 00008855 Société Française d’Ophtalmologie IRB#1).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement All data relevant to the study are included in the article or uploaded as supplementary information.

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