From the Guidelines
Ocular Pemphigus vulgaris is a rare autoimmune blistering disease that can affect the eyes, causing significant ocular surface inflammation and scarring, and its characteristics include chronic progression, subepithelial fibrosis, and potential for severe vision loss if left untreated 1.
Characteristics of Ocular Pemphigus Vulgaris
The disease is characterized by:
- Chronic progression with frequent remissions and exacerbations of disease activity
- Subepithelial fibrosis and keratinization of the conjunctiva
- Conjunctival scarring, symblepharon, entropion, trichiasis, and corneal ulcers or perforation
- Potential for severe vision loss if left untreated
Clinical Signs and Associated Factors
Clinical signs of Ocular Pemphigus vulgaris include:
- Bilateral, often asymmetric, bulbar conjunctival injection, papillary conjunctivitis, and conjunctival subepithelial fibrosis
- Keratinization, conjunctival scarring, and progressive conjunctival shrinkage
- Associated factors include unknown genetic predisposition, more common in women, and onset generally over age 60
Natural History and Potential Sequelae
The natural history of Ocular Pemphigus vulgaris is characterized by:
- Onset with goblet cell loss and mucin deficiency
- Progressive chronic course, sometimes with remissions and exacerbations
- Potential sequelae include conjunctival scarring and shrinkage, ankyloblepharon, symblepharon, trichiasis, corneal scarring, neovascularization, ulceration, and perforation
Treatment and Management
Treatment of Ocular Pemphigus vulgaris typically involves a combination of systemic and topical therapies, including:
- First-line systemic treatment with oral prednisone and steroid-sparing immunosuppressants such as azathioprine, mycophenolate mofetil, or rituximab
- Topical management with preservative-free artificial tears, topical corticosteroids, and antibiotic ointments
- Regular monitoring by both an ophthalmologist and dermatologist, with eye exams every 1-3 months during active disease 1.
From the Research
Characteristics of Ocular Pemphigus Vulgaris
- Ocular involvement in Pemphigus Vulgaris (PV) is rare, with the most common ophthalmologic involvement being conjunctivitis 2, 3
- Severe conjunctivitis may be the dominating clinical manifestation in ocular pemphigus vulgaris, implying a need for distinct severity criteria and therapeutic standards 2
- Corneal involvement is very rare, but can occur, leading to complications such as corneal ulceration and perforation 3
- Ocular cicatrization, corneal vascularization, and photophobia are also possible symptoms of ocular pemphigus vulgaris 2, 3
Treatment of Ocular Pemphigus Vulgaris
- Treatment of ocular pemphigus vulgaris typically involves a combination of systemic corticosteroids and immunosuppressive agents, such as cyclophosphamide or azathioprine 2, 3, 4, 5, 6
- The use of prednisone and cyclophosphamide has been shown to be effective in achieving clinical remission in patients with pemphigus vulgaris, including those with ocular involvement 2, 5
- Other treatment options, such as rituximab, may also be considered in severe or refractory cases 4