Ocular cicatricial pemphigoid - External and Internal Eye
Characterized by chronic conjunctivitis, ocular involvement in CP may lead to conjunctival injection, conjunctival and corneal scarring, and blindness. Patients may initially present with dryness and redness of eyes, eye irritation ("gritty eyes"), excessive tearing, burning, and photophobia. While this disease may begin unilaterally, it will progress to involve both eyes over time if untreated.
In the oral cavity, CP causes painful ulcers and erosions. Involvement of other sites may lead to hoarseness (vocal cords), epistaxis (nasal mucosa), and dysphagia (esophagus). Genital involvement may lead to phimosis and vaginal scarring. See cicatricial pemphigoid for oral mucosa, genital mucosa, and skin manifestations.
This condition develops over weeks to months and results in scarring and irreversible tissue destruction. (While cicatrization is common in the larynx, eye and skin, it is uncommon in the oral mucosa.)
For more information, see OMIM.
L12.1 – Cicatricial pemphigoid
314757003 – Ocular cicatricial pemphigoid
- Cicatricial conjunctivitis
- Pemphigus vulgaris
- Linear IgA disease may be indistinguishable clinically.
- Stevens-Johnson syndrome can affect the eyes, oral mucosa, and skin as well.
- Severe chemical burn
- Pseudo ocular CP from chronic topical medication use (glaucoma medications, antiviral agents)