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Oculomucocutaneous changes as paraneoplastic syndromes

B Kreutzer1, N Stübiger, H J Thiel

  • 1Universitäts-Augenklinik, Abteilung, Tübingen, Germany.

German Journal of Ophthalmology
|May 1, 1996
PubMed
Summary

Severe eye conditions like erythema exsudativum multiforme major and bullous pemphigoid can be paraneoplastic syndromes linked to lymphoma. Ocular complications often have a poor prognosis despite treatment.

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Area of Science:

  • Ophthalmology and Dermatology
  • Oncology
  • Immunopathology

Background:

  • Bullous dermatoses, including erythema exsudativum multiforme major (EEMM) and bullous pemphigoid, can cause severe ocular complications.
  • These conditions may rarely manifest as paraneoplastic syndromes associated with underlying malignancies, particularly non-Hodgkin's lymphoma (NHL).
  • Understanding the immunopathologic mechanisms is crucial for managing these complex cases.

Observation:

  • Case 1: A 69-year-old woman with grade IIIb NHL developed EEMM during treatment, leading to conjunctival necrosis, corneal superinfection, and perforation requiring keratoplasty. She died 6 weeks later.
  • Case 2: A 44-year-old man with grade IVa NHL developed bullous pemphigoid affecting skin, mucous membranes, and eyes. Ocular symptoms showed limited response to immunosuppressants like azathioprine and cyclophosphamide but improved slightly with cyclosporin A.
  • Both patients received treatments including sulfamethoxazole and trimethoprim, raising questions about drug-induced or exacerbated conditions in the context of lymphoma.

Findings:

  • The study highlights the severe ocular manifestations of paraneoplastic bullous dermatoses in lymphoma patients.
  • Therapeutic interventions, including immunosuppressants and antibiotics, showed limited efficacy in preventing irreversible ocular damage such as conjunctival cicatrization and keratinization.
  • The rapid progression and poor response to treatment underscore the challenges in managing these severe oculomucocutaneous syndromes.

Implications:

  • Occult malignancies must be considered and excluded in patients presenting with acute or chronic oculomucocutaneous syndromes.
  • The poor ocular prognosis associated with paraneoplastic bullous dermatoses necessitates early diagnosis and aggressive management strategies.
  • Further research into the immunopathogenesis and effective therapeutic options for these rare paraneoplastic syndromes is warranted.

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