When these findings of cicatrizing conjunctivitis are discovered, the first diagnostic consideration is whether it is the result of a historical event or is reflective of an active process. Patients should be questioned about previous episodes of severe conjunctivitis, chemical injuries, a history of Stevens-Johnson syndrome (SJS) , or prior chronic use of topical glaucoma medications, particularly pilocarpine [1]. These episodes can result in conjunctival scarring that, in general, is not progressive. A rare but important exception here is an autoimmune-mediated progressive scarring found in some patients with SJS long after resolution of the acute episode [2].

Active, progressive cicatrizing conjunctivitis has many reported etiologies. Of those with autoimmune etiologies, ocular cicatricial pemphigoid (OCP) is likely the most common with an estimated incidence of 1:12,000 to 1:60,000 [3]. Rosacea with MGD and atopic disease are both common entities in general ophthalmic practice, but, in especially severe forms, each can be the primary cause of conjunctival cicatrization [4, 5]. Importantly, even mild to moderate MGD or atopy can combine with OCP to form a more refractory disease.

Several systemic inflammatory syndromes have also been described as rare causes of cicatrizing conjunctivitis. These include (but are not limited to) Sjögren’s syndrome , granulomatosis with polyangiitis (GPA) (formerly Wegener’s granulomatosis ), and sarcoidosis [1, 6–8]. Paraneoplastic cases have also been reported [9]. Trachoma causes chronic conjunctival scarring resulting in a cicatricial entropion that then causes mechanical damage to the ocular surface. Worldwide, it is the most common infectious cause of blindness [10]. While extremely rare in the USA, trachoma should be considered in recent immigrants from endemic areas.

OCP is defined as a slow but relentless progression of conjunctival scarring eventually leading to corneal involvement and blindness [11, 12]. It is a bilateral disease but in some cases can be markedly asymmetric. The four stages of the disease have been described (Table 12.1). It falls under the spectrum of diseases classified as mucous membrane pemphigoid (MMP) [13]. In these disorders antibodies are formed to various glycoproteins in mucous membrane basement membrane zones (BMZ) resulting in chronic inflammation and scarring of mucous membranes. OCP can occur as an isolated condition or in combination with other mucous membrane involvement. The gold standard for diagnosis of OCP remains a conjunctival biopsy demonstrating immunoglobulin or complement deposition at the epithelial BMZ. False negatives are common and do not rule out the condition [14].

Though this patient’s current condition started with a presumed viral conjunctivitis, the conjunctivitis itself was never noted to be especially severe; rather it has simply lingered. Now identified as cicatrizing, a thorough history and review of systems should be performed to look for an occult underlying systemic inflammatory process with focus on involvement of other mucous membranes. Patients will often not mention oral or vaginal lesions without prompting. Special emphasis should be placed on dysphagia and breathing abnormalities as these may signal occult involvement in the esophagus or larynx. Involvement of these areas has been estimated to be 5–15% [15]. Laryngeal strictures are of particular importance as they can be life-threatening if not diagnosed. Though uncommon, OCP has been reported as a manifestation of a para-neoplastic process; however, routine workup for occult malignancy is not indicated (beyond age-appropriate routine screening tests).