Paraneoplastic conjunctival cicatrization: two different pathogenic types

To describe the clinical and immunopathologic features of patients with 2 different types of paraneoplastic conjunctival cicatrization.

Retrospective observational case analyses with a review of the literature.

One patient with paraneoplastic ocular cicatricial pemphigoid (POCP) and 1 patient with paraneoplastic pemphigus (PNP) with ocular involvement.

Critical review of clinical history, diagnostic studies, and immunopathologic results of biopsies in the 2 cases, together with a review of the literature.

Ability to recognize paraneoplastic conjunctival cicatrization and to diagnose the conditions accurately.

The first patient, 46 years of age, presented with conjunctival scarring and symblephara, cough, oral lesions, and chest rash. Concurrently, a diagnosis of pulmonary squamous cell carcinoma was made. Conjunctival biopsy revealed a subepithelial bulla, an inflammatory infiltrate of T and B lymphocytes, and basement membrane zone deposition of immunoglobulin (Ig)-G and C3 consistent with POCP. The second patient, 54 years of age, had a recently diagnosed B-cell chronic lymphocytic leukemia, followed 1 month later with ocular irritation and bilateral extensive symblephara. Extensive oral lesions and skin involvement of the lower half of the body were seen. Skin biopsy disclosed subepidermal bullae and mostly T cells with virtually no B cells in the dermal infiltrate (the patient was being treated with rituximab). Linear subepithelial deposition of IgG and C3 and deposition within the epidermis were consistent with PNP. Further indirect immunofluorescence and immunoprecipitation studies with the patient's serum-derived antibodies established PNP as the definitive diagnosis.

Underlying malignancy is an important consideration in younger patients with puzzling bilateral cicatrizing conjunctivitis, and a paraneoplastic condition can be established from either a conjunctival or a skin biopsy.

The author(s) have no proprietary or commercial interest in any materials discussed in this article.