Allergic Granulomatosis of the Conjunctiva in Asthmatic Patients: A Limited Form of the Churg-Strauss Syndrome

1. Allergic Granulomatosis of the Conjunctiva in Asthmatic Patients: A Limited Form of the Churg-Strauss Syndrome AltinPani MD, Martin Mayers MD, Pearl S Rosenbaum MD Department of Ophthalmology Bronx-Lebanon Hospital Center Albert Einstein College of Medicine Bronx, NY The authors have no financial interest in the subject matter of this poster.

2. PURPOSE Although rare, ophthalmologic manifestations of the Churg-Strauss syndrome have been described and include conjunctival inflammation, (3, 5, 7, 9), uveoscleritis and papilledema (1), amaurosis fugax, superior oblique palsy, ischemic optic neuropathy (10), branch retinal artery occlusion (2), and orbital inflammatory pseudotumor (8 ). Herein we describe the clinical and pathological entity of allergic granulomatosis (hypersensitivity granuloma, eosinophilic granuloma with flame figures) of the conjunctiva which, in a patient with bronchial asthma, may represent a limited form of the potentially life-threatening Churg-Strauss syndrome (allergic granulomatosis and angiitis).

3. METHODS Clinicopathological case reports of a 56-year-old Hispanic man (Case #1) and a 40-year-old African-American woman (Case#2) who underwent biopsies of superior limbalconjunctival nodules. Case # 2 Case # 1 Case # 2 External photograph of 1-2 mm, tan conjunctival nodules on the superior bulbar conjunctiva of both patients. The lesions are mobile over the sclera.

4. METHODS Both patients had a past medical history of bronchial asthma. The patient in Case #1 had exacerbation of his asthma at presentation and required hospitalization one week later. Both patients underwent excision of their bulbar conjunctival nodules. Histopathology was performed in both cases and electron microscopy was additionally performed in Case #1.

5. RESULTSHistopathology Histopathology of the conjunctival nodules in both cases revealed an intact epithelium and basement membrane. Within the substantia propria there was eosinophilic infiltration with scattered lymphocytes and plasma cells. Several “flame figures” (arrows) were also present.

6. “FLAME FIGURE” HISTOPATHOLOGY Higher power magnifications of the flame figures demonstrate a central mass of amorphous basophilic and eosinophilic material surrounded by palisading histiocytes and scatterd multinucleated giant cells (arrows).

7. Leder esterase staining was positive, showing the reddish granularity of the eosinophilic enzymes within the central zone of the flame figures.

8. RESULTSTransmission Electron Microscopy C C E EG C C C E Original magnification 9,000x Original magnification 30,000x Case # 1: Ultrastructurally, the flame figures demonstrated degranulated eosinophils (E) adjacent to intact bundles of collagen (C) (left). Free eosinophilic granules (EG) coat the collagen (right).

9. CONCLUSIONS Allergic granulomatosis and angiitis (Churg-Strauss syndrome, 1951) is severe, usually fatal, multisystem vasculitis characterized clinically by bronchial asthma, fever and eosinophilia. Histopathologically, a necrotizing vasculitis of small arteries and veins, infiltration of vessels and perivascular tissues by eosinophils, and extravascular granulomas have been noted . Several asthmatic patients with allergic granulomatosis of the prostate who later developed systemic angiitis have been described (4, 6). Histopathology of prostatic biopsies in these cases also disclosed eosinophilic granulomas with flame figures . These cases may initially have represented a pre-vasculitic phase of Churg-Strauss syndrome.

10. CONCLUSIONS Thus, while the conjunctival flame figure may simply represent a transient result of unusual eosinophil activity in response to various inciting stimuli, its presence in a patient with bronchial asthma may warrant close clinical observation for the development of multisystem vasculitis and the full blown clinical features of the Churg-Strauss syndrome.

11. Bibliography • 1) Curry D, Breakey AS, Payne BF. Allergic granulomatous angiitis associated with uveoscleritis and papilledema. Arch Ophthalmology 55: 261-266, 1955. • 2) Dagi LR, Currie J. Branch retinal artery occlusion in the Churg-Strauss syndrome. J Clin Neuro – Ophthalmol 5:237-299, 1985. • 3) Delarbre X, Andre M, Dalens H et al. Ocular manifestations of systemic vasculitis : report of six cases and review of the literature . La Revue De Medicine Interne 22:1039-1048, 2001. • 4) Kiokowa H, Koyama M, Kato H. Int J Urol. Churg-Strauss syndrome presenting with eosinophilic prostatitis. 2006 Jun;13(6):838-40. • 5) Margolis R, Kosmorsky GS, Lowder CY, Schoenfield L.Conjunctival involvement in Churg-Strauss syndrome. Ocul Immunol Inflamm. 2007 Mar-Apr;15(2):113-5.  • 6) Melicow MM. Allergic granulomas of the prostate gland. J Urol 65: 288-296, 1951. • 7) Meisler DM, Stock EL, Wertz RD, et al. Conjuctival inflammation and amyloidosis in allergic granulomatosis and angitiis( Churg-Strauss syndrome). J Am Ophthalmol 91: 216-219, 1981. • 8) Nissim F, Von der Valde J, Czernobilisky B. A limited form of Churg-Strauss syndrome. Ocular and cutaneous manifestations. Arch Pathol Lab Med 106: 305-307, 1982. .

12. Bibliography • 9) Shields CL, Shields JA, Rozanksi TI. Conjuctival involvement in Churg-Strauss syndrome. Am J Ophthalmol 102:601-605, 1986. • 10) Weinstein JM, Chui H, Lane S, et al. Churg-Strauss syndrome allergic granulomatous angitiis: Neuro-ophthalmologic manifestations . Arch Ophthalmol 101: 1217-1220, 1983