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Background: Granulomatosis with polyangiitis (GPA), a systemic antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, is characterized by inflammation of the small arteries, arterioles, and capillaries classically manifesting with glomerulonephritis and necrotizing granulomatous lesions of the upper and lower respiratory tract. With an incidence of approximately 12 cases per one million individuals per year it is an uncommon diagnosis that typically presents as frequent pulmonary and sinus infections; however, if left without definitive treatment progresses to more severe manifestations specifically hemoptysis and hematuria. Case Description: This case report highlights a 15-year-old woman who had both classic and non-classic findings making the diagnosis challenging. Specifically, her age of presentation, improvement with anti-microbials, and coronary dilation were not classic. Additionally, her lab work was negative for the cytoplasmic subset antineutrophil cytoplasmic autoantibody (c-ANCA), but positive for serum anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) which further delayed the ultimate diagnosis as this is typically c-ANCA positive. Conclusions: Other systemic vasculitides, such as mucocutaneous lymph node disease, are associated with cardiac pathology necessitating further medical management and follow-up to prevent increased morbidity and mortality. Knowing this, we conclude that further evaluation for cardiac pathology would be prudent as part of the initial workup of patients with a diagnosis of GPA. Included is a brief review of available literature on GPA to emphasize the typical presentation, lab findings, and importance of early diagnosis.
RESUMO
PURPOSE: To report a temporal profile of Purpureocillium lilacinum keratitis (PLK) secondary to immune dysfunction induced by the combination of reactivation of herpes zoster dermatitis and recent influenza vaccination that suggests a possible association, including successful medical management. METHODS: A 64-year-old contact lens wearer presented with left eye keratitis days after receiving an influenza vaccination and subsequent development of herpes zoster lesions on the flank. Patient was initially treated for bacterial keratitis with fortified antibiotics and oral valacyclovir for her concurrent zoster. Pharmacotherapy was changed to topical voriconazole after cultures were positive for Purpureocillium lilacinum. Topography and anterior segment OCT demonstrated scarring at multiple levels within the cornea with irregular astigmatism. A literature review was conducted to identify mechanisms that demonstrate a temporal link between influenza vaccination, herpes zoster reactivation, and fungal keratitis. RESULTS: After the conclusion of topical therapy, the central corneal infiltrate regressed and a partial light-blocking anterior stromal scar remained. Best corrected visual acuity improved from 20/400 to 20/25. CONCLUSION: Transient systemic immune dysregulation, secondary to influenza vaccination and reactivation of systemic herpetic disease, compounded by contact lens wear, may create a favorable environment for opportunistic fungal keratitis. This case highlights the importance of adequately assessing and treating for existing comorbidities in the successful treatment of mycotic keratitis.
