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Book

Cicatricial Pemphigoid

Leila Tolaymat  1 , Hasnain A. Syed  2 , Matthew R. Hall  3
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
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Book

Cicatricial Pemphigoid

Leila Tolaymat et al.
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Excerpt

Cicatricial pemphigoid represents a rare, chronic autoimmune blistering disorder characterized by the potential to cause scarring. The disease may affect the skin alone, the mucous membranes alone, or both. When confined to the mucous membranes, the condition is often termed mucous membrane pemphigoid. In cases limited to the ocular surfaces, the term ocular pemphigoid is commonly used.

The likelihood of scarring depends largely on the anatomical site of disease involvement. Diagnosing cicatricial pemphigoid can present significant challenges, particularly in the early stages, when clinical features may be nonspecific. Given the potential for serious complications, eg, vision loss and airway obstruction, early recognition and prompt initiation of aggressive therapy may help prevent irreversible damage and improve long-term outcomes.

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Conflict of interest statement

Disclosure: Leila Tolaymat declares no relevant financial relationships with ineligible companies.

Disclosure: Hasnain Syed declares no relevant financial relationships with ineligible companies.

Disclosure: Matthew Hall declares no relevant financial relationships with ineligible companies.

References

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    1. Schonberg S, Stokkermans TJ. StatPearls [Internet] StatPearls Publishing; Treasure Island (FL): 2023. Jul 31, Ocular Pemphigoid.
    1. Balding SD, Prost C, Diaz LA, Bernard P, Bedane C, Aberdam D, Giudice GJ. Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. J Invest Dermatol. 1996 Jan;106(1):141-6. - PubMed
    1. Domloge-Hultsch N, Gammon WR, Briggaman RA, Gil SG, Carter WG, Yancey KB. Epiligrin, the major human keratinocyte integrin ligand, is a target in both an acquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest. 1992 Oct;90(4):1628-33. - PMC - PubMed
    1. Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmed AR. Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization. Proc Natl Acad Sci U S A. 1996 Dec 10;93(25):14714-9. - PMC - PubMed

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