Chronic Cicatrizing Conjunctivitis (CCC) may occur as a consequence of several different diseases, including Ocular Cicatricial Pemphigoid  (OCP), Stevens-Johnson Synddrome, Atopic Keratoconjunctivitis, Ocular Rosacea, Scleroderma,Lichen Planus, Linear IgA disease, Pemphigus Vulgaris, Ligneous Conjunctivitis, Sarcoidosis,  and even infection (Trachoma, Adeneovirus 8). Coming to a correct firm diagnosis can be challenging yet crucial for choosing correct therapy. Some causes are more obvious than others. For example, a patient with history of an acute exanthema with rash and oral mucosal and conjunctival inflammation would certainly have SJS as the probable diagnosis. But even with this situation one may find that the initial insult (SJS) to tissue eventuated to a secondary phenomenon of autoimmue attack  on previously hidden conjunctival glycoproteins (eg, beta 4 subunit of alpha 6beta4 integrin), causing a second disease (OCP) to arise.

 

Immunopathology of biopsied conjunctiva  is often the most failsafe path to the correct diagnosis. But here too there are challenges. The details of doing the biopsy correctly, of handling the specimen correctly, of performing the analysis of the specimen correctly are many and tedious. We see all too often instances of a “negative” biopsy for OCP resulting in progression of OCP secondary to delayed diagnosis and correct therapy. I will show you an instance of that which was the beginning of my journey into the world of conjunctival immunopathology.