Paraneoplastic conjunctival cicatrization: two different pathogenic types.
OBJECTIVE
To describe the clinical and immunopathologic features of patients with 2 different types of paraneoplastic conjunctival cicatrization.


METHODS
Retrospective observational case analyses with a review of the literature.


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


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


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


RESULTS
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.


CONCLUSIONS
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.


BACKGROUND
The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Ahuero
Audrey E
AE
Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts 02114, USA.
Jakobiec
Frederick A
FA
Bhat
Pooja
P
Ciralsky
Jessica B
JB
Papaliodis
George N
GN
D001402:B-Lymphocytes / Q000276:immunology; D001706:Biopsy; D002294:Carcinoma, Squamous Cell / Q000276:immunology / Q000473:pathology*; D003176:Complement C3 / Q000032:analysis; D003229:Conjunctival Diseases / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000276:immunology / Q000473:pathology*; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000276:immunology / Q000473:pathology*; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology / Q000473:pathology*; D010392:Pemphigus / Q000276:immunology / Q000473:pathology*; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000276:immunology; D014057:Tomography, X-Ray Computed
D003176:Complement C3; D007074:Immunoglobulin G