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Ocular cicatricial pemphigoid - External and Internal Eye
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Ocular cicatricial pemphigoid - External and Internal Eye

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Contributors: Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
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Synopsis

Ocular cicatricial pemphigoid : Conjunctival scarring, Entropion, Symblepharon, Conjunctival injection, Eyelash misdirection
Cicatricial pemphigoid (CP), also known as mucous membrane pemphigoid, is a chronic autoimmune blistering disease that affects the oral, genital, and ocular mucous membranes and, less commonly, the skin. CP is almost twice as common in females as it is in males, and it is seen most frequently in older individuals.

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.)

Codes

ICD10CM:
L12.1 – Cicatricial pemphigoid

SNOMEDCT:
314757003 – Ocular cicatricial pemphigoid

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated:01/20/2022
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Ocular cicatricial pemphigoid - External and Internal Eye
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A medical illustration showing key findings of Ocular cicatricial pemphigoid : Conjunctival scarring, Entropion, Symblepharon, Conjunctival injection, Eyelash misdirection
Clinical image of Ocular cicatricial pemphigoid - imageId=2597567. Click to open in gallery. caption: 'Fibrotic scarring of the palpebral to the bulbar conjunctiva (symblepharon).'
Fibrotic scarring of the palpebral to the bulbar conjunctiva (symblepharon).

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