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Cicatricial pemphigoid and erythema multiforme
1Jules Stein Eye Institute, UCLA School of Medicine 90024.
Insights
Cicatricial pemphigoid (CP) and erythema multiforme (EM) are severe bullous diseases affecting the conjunctiva. Treatments range from supportive care to systemic immunosuppression for these chronic or acute inflammatory conditions.
Area of Science:
- Ophthalmology
- Dermatology
- Immunology
Background:
- Cicatricial pemphigoid (CP) and erythema multiforme (EM) are bullous diseases frequently and severely affecting the conjunctiva.
- CP is a chronic, progressive condition in the elderly, causing severe ocular complications like symblepharon and vision loss.
- EM is an acute, self-limited disorder in young individuals, with potential for scarring and vision-impairing sequelae.
Purpose of the Study:
- To differentiate the clinical presentation, pathogenesis, and management of Cicatricial Pemphigoid (CP) and Erythema Multiforme (EM) concerning conjunctival involvement.
- To highlight the unique challenges and severe outcomes associated with these bullous diseases in the ocular tissues.
Main Methods:
- Review of clinical characteristics, histopathology, and immunopathology of CP and EM.
- Analysis of conjunctival manifestations, including symblepharon, entropion, trichiasis, and dry eye.
- Examination of treatment strategies for both conditions, from supportive measures to systemic therapies.
Main Results:
- CP presents with subepithelial blisters and basement membrane deposition, leading to chronic conjunctival shrinkage and vision impairment.
- EM involves subepithelial bullae and perivascular infiltrates, with potential for scarring and ankyloblepharon after acute episodes.
- Both diseases require multifaceted management, including lubrication, antibiotics, surgical correction, and immunosuppression.
Conclusions:
- CP and EM represent the most frequent and severe bullous diseases affecting the conjunctiva, necessitating prompt and appropriate management.
- Understanding the distinct pathophysiologies of CP and EM is crucial for effective treatment and prevention of irreversible ocular damage.
- Ocular involvement in these bullous diseases underscores the importance of a multidisciplinary approach involving ophthalmologists, dermatologists, and immunologists.
Abstract:
Cicatricial pemphigoid (CP) and erythema multiforme (EM) are bullous diseases that involve the skin and mucous membranes including the conjunctiva. Of all the bullous diseases, CP and EM not only involve the conjunctiva most frequently but also cause the most severe conjunctival disease. A chronic, progressive disease, CP is characterized by shrinkage of the conjunctiva, symblepharon, entropion, trichiasis, dry eye, and finally reduced vision from corneal opacification. It is primarily a disease of the elderly that affects more women than men and is characterized by blisters or bullae in a subepithelial location and immunoglobulins and complement bound to the basement membrane zone of skin and mucous membranes including the conjunctiva. Circulating antibodies to the basement membrane zone can be demonstrated occasionally. Treatment includes artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, and systemic immunosuppressive therapy including corticosteroids. An acute, generally self-limited, inflammatory disorder of the skin and mucous membranes, EM occurs primarily in young, healthy individuals. The most frequent precipitating factors are (1) drugs and (2) infections caused by Mycoplasma pneumoniae and herpes simplex. Conjunctival involvement ranges from a mild catarrhal conjunctivitis which terminates without sequelae to membranous conjunctivitis which may heal leaving scarring, symblepharon, and even ankyloblepharon. Histopathologic findings include subepithelial bullae and perivascular mononuclear cell infiltrates. Patients with EM have circulating immune complexes and immunoreactant deposition in the blood vessel walls of the dermis. After the acute episode has subsided, they may require artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, tarsorrhaphy, and mucous membrane grafts.