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Pemphigoid, Cicatricial

Pemphigoid, Cicatricial is a topic covered in the 5-Minute Clinical Consult.

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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)
  • “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

Stephens, Mark B., et al., editors. "Pemphigoid, Cicatricial." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid__Cicatricial.
Pemphigoid, Cicatricial. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid__Cicatricial. Accessed March 26, 2019.
Pemphigoid, Cicatricial. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816198/all/Pemphigoid__Cicatricial
Pemphigoid, Cicatricial [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 26]. 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 - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, 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 - 27 DB - Medicine Central DP - Unbound Medicine ER -