Pathological Findings of Pemphigus Vulgaris Showing Giant Cobblestone-Like Conjunctival Papillae

PURPOSE: To describe the pathophysiological findings of a patient with pemphigus vulgaris (PV) showing giant conjunctival papillae. METHODS: A 64-year-old man who had mucosal-dominant PV with giant conjunctival papillae, resembling those of vernal keratoconjunctivitis (VKC), underwent an ophthalmological workup. The clinical and pathological findings were investigated. RESULTS: Ophthalmic interventions were unable to provide the desired beneficial effects, and multiple excisions were necessary to remove the proliferative conjunctival lesions. Histopathological investigations of the excised tissues demonstrated acantholysis and a subconjunctival infiltration with numerous inflammatory cells such as lymphocytes, plasma cells, and neutrophils. However, in contrast to typical VKC, mast cells and eosinophils were rarely found in the subconjunctival tissues. Direct immunofluorescent staining showed a significant deposition of immunoglobulin G and complement component 3 in the epithelial intercellular substance, consistent with mucosal-dominant PV. Then, the patient was hospitalized because of oral erosion exacerbation and malnutrition. Because of the patient's declining general condition, we administered an increasing dose of a systemic steroid with an intravenous immunoglobulin, after which his ocular lesions and symptoms improved. CONCLUSIONS: The histological conjunctival papilla findings were quite different from those of VKC papillae. If PV causes a lesion in a patient, systemic immunosuppression might be more effective than topical ophthalmic treatment because of overall immunological involvement.