Case Report Churg–Strauss Syndrome with Concomitant Occurrence of Ischemic Stroke and Relapsing Purpura Koji Tanaka, MD,* Masatoshi Koga, MD,† Hatsue Ishibashi-Ueda, MD,‡ Chiho Matsumoto, MD,x and Kazunori Toyoda, MD* A 77-year-old woman suffering from chronic bronchial asthma and chronic atrial fibrillation who had had a previous ischemic stroke presented to our emergency unit with gait disturbance. She had new-onset truncal ataxia, right hemiparesis, and right sensory disturbance related to the previous stroke. Her lower legs were slightly swollen and had a reddened appearance. Her medical history included mitral valve replacement because of severe mitral valve regurgitation. Her white blood cell count was 8600/mL, mainly consisting of eosinophils (4480/mL; 52.1%). Serum nonspecific immunoglobulin E was elevated to 1600 IU/mL (normal range ,170 IU/mL). She was taking warfarin for secondary stroke prevention, and on admission her prothrombin time international normalized ratio was 3.06. Diffusion-weighted magnetic resonance imaging revealed a fresh infarct in the right cerebellum. No stenosis or occlusion was shown in the cervicocephalic arteries on magnetic resonance angiography or carotid ultrasound. No emboligenic diseases, except for atrial fibrillation, were identified. On day 3, an extensive itchy, purpuric rash appeared on her lower limbs. The rash remitted and recurred spontaneously for several weeks. A skin biopsy specimen of the purpuric lesions revealed massive eosinophilic infiltration of the dermis and eosinophilic vasculitis involving small vessels. We diagnosed the patient with Churg–Strauss syndrome (CSS). Skin lesions and eosinophilia disappeared after oral corticosteroid therapy. In this case, cerebellar infarction occurred with purpuric rash despite well-controlled anticoagulation. Patients with CSS may suffer from ischemic stroke when the condition of CSS deteriorates. Key Words: Churg–Strauss syndrome—ischemic stroke—purpura. Ó 2012 by National Stroke Association Case Report From the *Department of Cerebrovascular Medicine; †Division of Stroke Care Unit; ‡Department of Pathology, National Cerebral and Cardiovascular Center, Suita; and xDepartment of Dermatology, Minoh City Hospital, Minoh, Japan. Received November 2, 2011; revision received November 23, 2011; accepted November 29, 2011. Address correspondence to Masatoshi Koga, MD, Division of Stroke Care Unit, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. E-mail: koga@hsp.ncvc.go.jp. 1052-3057/$ - see front matter Ó 2012 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2011.11.010 A 77-year-old woman suffering from chronic bronchial asthma and chronic atrial fibrillation, with previous cardioembolic ischemic stroke, presented to our emergency unit with gait disturbance. She had new-onset truncal ataxia in addition to right hemiparesis and right sensory disturbance because of the previous stroke. Her lower legs were slightly swollen and had a reddened appearance. Her medical history included mitral valve replacement because of severe mitral valve regurgitation. Her white blood cell count was 8600/mL, mainly consisting of eosinophils (4480/mL; 52.1%). Serum nonspecific immunoglobulin E was elevated to 1600 IU/mL (normal Journal of Stroke and Cerebrovascular Diseases, Vol. 21, No. 8 (November), 2012: pp 911.e9-911.e10 911.e9 K. TANAKA ET AL. 911.e10 Figure 1. (A) Diffusion-weighted magnetic resonance image revealing a hyperintense region in the right hemisphere of the cerebellum (arrow). (B) Macroscopic photograph of the purpura on the left lower leg. (C) A skin biopsy specimen revealed extensive eosinophilic infiltration and hemorrhage (arrow) in the dermis. (Hematoxylin and eosin stain; scale bar 5 100 mm.) (D) Eosinophildominant necrotizing vasculitis (circle) of small vessels in the dermis with massive extravasation of eosinophils and red blood cells. (Hematoxylin and eosin stain; scale bar 5 100 mm.) range ,170 IU/mL). Perinuclear antineutrophil cytoplasmic antibody was negative. She was already taking warfarin for secondary stroke prevention and her prothrombin time international normalized ratio was 3.06 on admission, with a D-dimer of 1.8 mg/mL (normal range ,1.0 mg/ml). Diffusion-weighted magnetic resonance imaging (MRI) revealed a fresh infarct in the right cerebellum (Fig 1A). Cerebral microbleeds were not detected on T2-weighted MRI. No stenosis or occlusion was shown in the cervicocephalic arteries on magnetic resonance angiography and carotid ultrasound. No emboligenic diseases, except for atrial fibrillation, were identified. On day 3, an extensive itchy, purpuric rash appeared on her lower limbs (Fig 1B). The rash remitted and recurred spontaneously for several weeks. Skin biopsy speciemens of the purpuric lesions revealed massive eosinophilic infiltration of the dermis and eosinophilic vasculitis involving small vessels (Figs 1C and 1D). We diagnosed the patient with Churg–Strauss syndrome (CSS). Skin lesions and eosinophilia disappeared after oral corticosteroid therapy. Discussion CSS is a rare, small-sized vessel systemic necrotizing vasculitis that occurs in patients having asthma or an allergic history.1 Ischemic stroke is uncommon in patients with CSS.2-7 Concomitant extravascular eosinophils in the dermis being compatible with CSS classification8 has not been pathologically proven in reported cases. In this case, cerebellar infarction occurred with purpuric rash despite well-controlled anticoagulation. Her stroke may have been caused by small-vessel arteritis of the central nervous system9 or hypercoagulable state because of CSS.10 Patients with CSS may suffer from ischemic stroke when the condition of CSS deteriorates. In patients with concomitant occurrence of ischemic stroke, eosinophilia, and extensive purpura, CSS should be considered in the differential diagnosis. Acknowledgment: This report was supported in part by grants-in-aid from the Ministry of Health, Labour and Welfare of Japan (H23-Junkanki-Ippan-010). References 1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301. 2. Ghaeni L, Siebert E, Ostendorf F, et al. Multiple cerebral infarctions in a patient with Churg–Strauss syndrome. J Neurol 2010;257:678-680. 3. Dinç A, Soy M, Pay S, et al. A case of Churg–Strauss syndrome presenting with cortical blindness. Clin Rheumatol 2000;19:318-320. 4. Kok J, Bosseray A, Brion JP, et al. Chorea in a child with Churg–Strauss syndrome. Stroke 1993;24:1263-1264. 5. Kang DW, Kim DE, Yoon BW, et al. Delayed diagnosis: Recurrent cerebral infarction associated with Churg– Strauss syndrome. Cerebrovasc Dis 2001;12:280-281. 6. Bhagirath KM, Paulson K, Ahmadie R, et al. Clinical utility of cardiac magnetic resonance imaging in Churg– Strauss syndrome: Case report and review of the literature. Rheumatol Int 2009;29:445-449. 7. Sonneville R, Lagrange M, Guidoux C, et al. The association of cardiac involvement and ischemic stroke in Churg–Strauss syndrome. Rev Neruol 2006;162:229-232. 8. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg–Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-1100. 9. Chang Y, Kargas SA, Goates JJ, et al. Intraventricular and subarachnoid hemorrhage resulting from necrotizing vasculitis of the choroid plexus in a patient with Churg– Strauss syndrome. Clin Neuropathol 1993;12:84-87. 10. Ishii T, Koide O, Hosoda Y, et al. Hypereosinophilic multiple thrombosis. A proposal of a new designation of disseminated eosinophilic ‘‘collagen disease’’. Angiology 1977;28:361-375.