Pemphigoid, Cicatricial
Basics
Description
- Cicatricial pemphigoid (CP) is a rare, chronic, acquired autoimmune subepithelial blistering disorder that mainly affects mucous membranes.
- In descending order of involvement, CP affects the oral cavity (85%), conjunctivae (65%), skin (20%), nasal cavity (15%), anogenital region (20%), pharynx (20%), larynx (10%), and esophagus (5%).
- Clinical hallmark of CP is scarring, which may result in serious functional impairment.
- Synonym(s): Brunsting-Perry CP (1); mucous membrane pemphigoid; oral pemphigoid; desquamative gingivitis; ocular CP; ocular pemphigus (incorrect nomenclature)
Epidemiology
- Average onset at age 60 to 80 years
- Rarely children can be affected, youngest found to be 10 months old (1).
- Female to male ratio of 2:1
- No racial or geographic predilection
Incidence
1/1 million annually
Etiology and Pathophysiology
- Autoantibodies binding to specific epithelial basement membrane components:
- Type XVII collagen (bullous pemphigoid antigen 2), type VII collagen, laminin-5, laminin-6, α-6 and β-4 integrin subunits, 120-kDa undefined epithelial antigen
- Exclusive ocular mucosal involvement: autoantibodies to β-4 subunit of integrin
- Exclusive oral mucosal involvement: autoantibodies to α-6 subunit of integrin
- Mucosal and skin involvement: autoantibodies to BP180
- Association with internal malignancy: autoantibodies to anti–laminin-332 (1)
- Type XVII collagen (bullous pemphigoid antigen 2), type VII collagen, laminin-5, laminin-6, α-6 and β-4 integrin subunits, 120-kDa undefined epithelial antigen
- “Epitope spreading”: Previous inflammatory events may predispose epithelial basement membrane components to autoreactive T cells.
- Several patients with Stevens-Johnson syndrome developed CP.
- Association with Sjögren syndrome and ocular CP
- Mechanism linking the binding of the antibodies to the epithelial basement membrane zone and subsequent scarring process yet to be defined
Genetics
Association with HLA DQB1*0301
General Prevention
None identified
Commonly Associated Conditions
Anti–laminin-332 autoantibody–positive (previously anti–laminin-5/epiligrin) patients have a higher risk of internal malignancy.
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Citation
Domino, Frank J., et al., editors. "Pemphigoid, Cicatricial." 5-Minute Clinical Consult, 33rd ed., Wolters Kluwer, 2025. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid_Cicatricial.
Pemphigoid, Cicatricial. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2025. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid_Cicatricial. Accessed November 4, 2024.
Pemphigoid, Cicatricial. (2025). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (33rd ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid_Cicatricial
Pemphigoid, Cicatricial [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2025. [cited 2024 November 04]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid_Cicatricial.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC
T1 - Pemphigoid, Cicatricial
ID - 816198
ED - Domino,Frank J,
ED - Baldor,Robert A,
ED - Golding,Jeremy,
ED - Stephens,Mark B,
BT - 5-Minute Clinical Consult, Updating
UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid_Cicatricial
PB - Wolters Kluwer
ET - 33
DB - Medicine Central
DP - Unbound Medicine
ER -