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[Severe eye involvement in pemphigus vulgaris]
H E Baykal1, U Pleyer, K Sönnichsen
1Abteilung I, Allgemeine Augenheilkunde und Poliklinik, Universitäts-Augenklinik Tübingen.
Summary
Pemphigus vulgaris (PV) can cause severe eye problems, including corneal perforation, even with treatment. This rare ocular manifestation requires considering PV in the differential diagnosis of chronic bullous dermatoses.
Area of Science:
- Ophthalmology
- Dermatology
- Immunology
Background:
- Pemphigus vulgaris (PV) is an immune-mediated blistering disease primarily affecting skin and mucous membranes.
- Ocular involvement in PV is uncommon, typically presenting as conjunctivitis without scarring, unlike ocular cicatricial pemphigoid.
- Corneal complications are exceedingly rare in pemphigus vulgaris.
Observation:
- A case report details severe ocular complications in a 56-year-old male patient with PV.
- The patient experienced conjunctival cicatrization, corneal ulceration, and perforation despite immunosuppressive therapy.
- A subsequent penetrating keratoplasty failed due to corneal vascularization.
Findings:
- This case highlights that pemphigus vulgaris can lead to severe, sight-threatening ocular manifestations, including corneal perforation.
- Standard immunosuppressive therapy (corticosteroids and azathioprine) was insufficient to prevent corneal perforation in this patient.
- Ocular cicatricial pemphigoid is a primary differential diagnosis for severe ocular cicatricial disease, but PV must also be considered.
Implications:
- Severe ocular involvement necessitates considering pemphigus vulgaris in the differential diagnosis of chronic bullous dermatoses.
- Early recognition and appropriate management strategies are crucial for patients with PV and ocular complications.
- Further research may be needed to understand and manage rare but severe ocular manifestations of pemphigus vulgaris.