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Ocular cicatricial pemphigoid (OCP) is a putative autoimmune, chronic, inflammatory, subepithelial blistering disease affecting mucous membranes.1 It is characterised by linear deposition of immunoglobulins and complement along epithelial basement membranes.1 In this report, we describe a cicatrising conjunctivitis that was clinically and pathologically similar to ocular cicatricial pemphigoid but occurred concurrently with systemic malignancy.
A 69‐year‐old cachectic man with chronic obstructive pulmonary disease presented with painful mouth and skin blisters. He developed a painful red eye 1 week later. Visual acuity was 20/25 on the right and 20/30 on the left. Slit‐lamp examination showed bulbar conjunctival injection and an intense papillary reaction of the palpebral conjunctiva on the right. At examination 10 days later, the patient had new fornix foreshortening and symblepharon formation. A skin biopsy revealed subepidermal bullous dermatosis with eosinophils and neutrophils (fig 1A1A).). Direct immunofluorescence showed linear IgG and C3 staining along the basement membrane (fig 1B1B).). These results were consistent with cicatricial pemphigoid. The patient began aggressive ocular lubrication and oral prednisone 80 mg daily.
During the following week, his ocular symptoms improved dramatically, although he developed bullae of the superior palpebral conjunctiva on the right, and bilateral subepithelial scarring, fornix foreshortening and symblepharon (fig 2A2A).). The patient was hospitalised that same week because of increasing difficulty with oral intake and weight loss. A chest x ray suggested lung cancer (fig 2B2B).). Bronchoalveolar lavage and systemic evaluation confirmed non‐small cell lung carcinoma, most likely squamous cell, stage IIIA. The patient underwent radiation and chemotherapy with cisplatin and etoposide. While on moderate‐dose steroids, he developed new cutaneous bullae. Mycophenolate mofetil was added but had no effect. Alkylating agents were not tried due to profound neutropenia. At a 5‐month follow‐up, visual acuity remained 20/25 in each eye, with clear corneas despite cicatricial entropion and trichiasis of the right lower eyelid.
Previously reported cases have shown a temporal association between cicatricial pemphigoid and systemic solid malignancies, including non‐small cell lung cancer (adenocarcinoma, squamous cell, large cell), gastric adenocarcinoma and endometrial adenocarcinoma.2,3,4,5,6,7,8 In some patients, the pemphigoid responded to chemotherapy or surgical resection of the tumour, suggesting a paraneoplastic process.2,6,8 Antibodies to laminin‐5, a basement membrane protein elaborated by lung and gastrointestinal epithelia, have been found in patients with cicatricial pemphigoid and lung and gastric cancers.2,3,4,6,8 Aberrant synthesis of laminin‐5 by tumour cells, or tumour‐related inflammation and invasion, may expose the antigen to the immune system.2,9 Pemphigus, an autoimmune disease characterised by intraepithelial blisters, can also present as a paraneoplastic process, although it is primarily associated with lymphoreticular malignancies.10
In a similar reported case of paraneoplastic pemphigoid, the diagnosis of squamous cell lung cancer was made 2 months after the patient developed cicatrising conjunctivitis.7 The differential diagnosis for cicatrising conjunctivitis is broad and includes OCP, Stevens Johnsons syndrome, chemical burns, radiation, toxic conjunctivitis, post‐infectious conjunctivitis, graft versus host disease, atopic keratoconjunctivitis and conjunctival squamous cell carcinoma.1 Paraneoplastic pemphigoid should also be considered in the differential diagnosis, and this case illustrates the importance of obtaining a pertinent history and review of systems.
Funding: This research was supported by the That Man May See Foundation.
Competing interests: None.
Informed consent was obtained for publication of the person's details in this report.