Eye
Conjunctiva
Other nonneoplastic
Ocular cicatricial pemphigoid
Author: Deepali Jain, M.D.
Last author update: 1 April 2013
Last staff update: 27 May 2021
Copyright: 2004-2023, PathologyOutlines.com, Inc.
PubMed Search: Ocular cicatricial pemphigoid conjunctiva [title]
Table of Contents
Definition / general | Grossing | Diagnosis | Case reports | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Jain D. Ocular cicatricial pemphigoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeocularcicatricialpemphigoid.html. Accessed September 21st, 2023.
Definition / general
- Also called mucus membrane pemphigoid of conjunctiva
- Mucocutaneous autoimmune disorder
- Bilateral; only rarely unilateral (Cornea 2010;29:1462)
- Usually age 50+ years, more common in women
- 50% of cases involve conjunctiva, usually 10 years or more after onset of cutaneous or other mucosal disease; may occasionally demonstrate purely ocular disease (Ophthalmology 1999;106:355)
- Uncontrolled cases are associated with infection (Cornea 2004;23:819)
- Rarely presents with corneal ulceration (Eur J Ophthalmol 2007;17:121)
Grossing
- Impression cytology may be useful for diagnosis (Arch Soc Esp Oftalmol 2004;79:67)
- Divide sample in two - one for formalin, one in transport media for immunofluorescent studies
Diagnosis
- Compatible clinical picture plus linear deposition of IgA and IgG along conjunctival basal lamina or circulating anti-basement membrane antibodies (Ophthalmology 2004;111:45)
- Autoantibodies targeting α-6 and β-4 integrins (Clin Dermatol 2012;30:34)
- Fragmented and lumpy basement membrane fibrinogen on immunofluroscence (Am J Dermatopathol 2011;33:786)
Case reports
- 62 year old woman with anti-laminin 5 antibodies (Clin Exp Dermatol 2005;30:679)
- 2 case reports associated with Lyell syndrome in 80 and 60 year old patients (Ophthalmology 2012;119:688)
Clinical images
Images hosted on other servers:
Cicatrizing conjunctivitis
Slit lamp photograph showing bullae
Microscopic (histologic) description
- Early - epithelial erosions and bullae
- Late - epithelial and stromal scarring, perivascular lymphocytes and plasma cells
Microscopic (histologic) images
Images hosted on other servers:
Inflamed conjunctiva
Subepidermal bullous dermatosis
Positive stains
- Local overexpression of IL6, IL12, IL17 (Invest Ophthalmol Vis Sci. 2012;53:8112)
Electron microscopy description
- EM has only limited usefulness in diagnosis (Ophthalmology 2006;113:1651)
Differential diagnosis
- Epidermolysis bullosa acquisita (Arch Ophthalmol 2006;124:1615)
- Paraneoplastic syndrome from nonocular carcinoma (Br J Ophthalmol 2007;91:1562)
