Ocular Cicatricial Pemphigoid: Manifestations and Management
Overview
Pulmonary Medicine
Authors
Affiliations
Ocular cicatricial pemphigoid (OCP) is a chronic, progressive cicatrizing inflammatory disease of presumed autoimmune etiology affecting the mucous membranes and skin. It has sight-threatening ocular manifestations and potentially life-threatening extraocular manifestations. The ocular signs include: chronic cicatricial conjunctivitis, progressive conjunctival fibrosis, and corneal epitheliopathy. Systemic immunomodulatory therapy is the treatment of choice for controlling disease activity and limiting progression, given the systemic nature of the disease and the poor efficacy of current local or topical therapies. Systemic cyclophosphamide with short-term adjunctive high-dose prednisolone is the preferred treatment for severe and/or rapidly progressing OCP. Oral low-dose weekly methotrexate is a useful first-line treatment for mild-to-moderate OCP. The management of OCP requires a multidisciplinary approach to optimize the care of these patients.
Ghanbari H, Rahimi M, Momeni A, Aminizade M, Nozarian Z, Moghtader A Graefes Arch Clin Exp Ophthalmol. 2025; .
PMID: 39909902 DOI: 10.1007/s00417-025-06756-2.
Role of immune cells in the ocular manifestations of pemphigoid diseases.
Bose T Ther Adv Ophthalmol. 2019; 11:2515841419868128.
PMID: 31448360 PMC: 6688137. DOI: 10.1177/2515841419868128.
The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on.
Dart J Eye (Lond). 2017; 31(2):301-332.
PMID: 28106896 PMC: 5306467. DOI: 10.1038/eye.2016.284.
Aldehyde dehydrogenase inhibition blocks mucosal fibrosis in human and mouse ocular scarring.
Ahadome S, Abraham D, Rayapureddi S, Saw V, Saban D, Calder V JCI Insight. 2016; 1(12):e87001.
PMID: 27699226 PMC: 5033866. DOI: 10.1172/jci.insight.87001.
Long Q, Zuo Y, Yang X, Gao T, Liu J, Li Y Int J Ophthalmol. 2016; 9(5):730-7.
PMID: 27275431 PMC: 4886873. DOI: 10.18240/ijo.2016.05.17.