Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal Wegener granulomatosis.
OBJECTIVE
To describe a case of chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and Wegener granulomatosis.


METHODS
Observational case report. A retrospective study.


RESULTS
An 80-year-old man presented with chronic cicatrizing conjunctivitis, peripheral corneal thinning, and Wegener granulomatosis, which were diagnosed by his referring physician based on clinical (recurrent epistaxis, sinus congestion) and histopathologic features of nasal mucosa (granulomatous inflammation, vasculitis). A conjunctival biopsy performed by us disclosed features of active Wegener granulomatosis and ocular cicatricial pemphigoid, which indicate lack of control of both diseases with methotrexate treatment. The patient died of pulmonary complications from Wegener granulomatosis 1 week after our evaluation.


CONCLUSIONS
Ocular cicatricial pemphigoid and Wegener granulomatosis are both potentially fatal autoimmune diseases. Ocular involvement in Wegener granulomatosis indicates poor control of the underlying systemic condition and is a marker for active vasculitis, which indicates the need for treatment with cyclophosphamide.
Miserocchi
E
E
Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA 02114, USA.
Waheed
N K
NK
Baltatzis
S
S
Foster
C S
CS
D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002908:Chronic Disease; D002921:Cicatrix / Q000473:pathology*; D003228:Conjunctiva / Q000473:pathology; D003231:Conjunctivitis / Q000175:diagnosis / Q000209:etiology*; D017809:Fatal Outcome; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000175:diagnosis