Abstract
To present three rare mimics of primary angiitis of the central nervous system (PACNS). We describe 3 patients with rare diseases that can mimic PACNS at clinical presentation and neuroimaging. We describe the clinical course of these patients and also present a review of the literature regarding these three diagnoses. All 3 patients presented with neurological symptoms and had abnormal findings on neuroimaging suggestive of PACNS. After detailed history, careful review of systems, thorough laboratory workup and consideration of lack of a response to immunosuppressive therapy, PACNS was ruled out with identification of an alternative diagnosis. PACNS is a rare disease and a diagnostic challenge with many differentials. A thorough investigation and awareness of unusual disorders is critical in avoiding misdiagnosis.
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References
Alrawi A, Trobe JD, Blaivas M, Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53(4):858–860
Birnbaum J, Hellmann DB (2009) Primary angiitis of the central nervous system. Arch Neurol 66(6):704–709
Miller DV, Salvarani C, Hunder GG, Brown RD, Parisi JE, Christianson TJ et al (2009) Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 33(1):35–43
Hajj-Ali RA, Calabrese LH (2009) Central nervous system vasculitis. Curr Opin Rheumatol 21(1):10–18
Calabrese LH, Duna GF, Lie JT (1997) Vasculitis in the central nervous system. Arthritis Rheum 40(7):1189–1201
Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 67(1):20–39
Lie JT (1992) Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 23(2):164–171
Degos R (1979) Malignant atrophic papulosis. Br J Dermatol 100(1):21–35
Scheinfeld N (2007) Malignant atrophic papulosis. Clin Exp Dermatol 32(5):483–487
Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S (1996) Skin lesion with a fatal neurologic outcome (Degos’ disease). Neurology 46(3):636–640
Chung HY, Trendell-Smith NJ, Yeung CK, Mok MY (2009) Degos’ disease: a rare condition simulating rheumatic diseases. Clin Rheumatol 28(7):861–863
Kanekura T, Uchino Y, Kanzaki T (2003) A case of malignant atrophic papulosis successfully treated with nicotine patches. Br J Dermatol 149(3):660–662
Zhu KJ, Zhou Q, Lin AH, Lu ZM, Cheng H (2007) The use of intravenous immunoglobulin in cutaneous and recurrent perforating intestinal Degos disease (malignant atrophic papulosis). Br J Dermatol 157(1):206–207
Timmermans M, Carr J (2004) Neurosyphilis in the modern era. J Neurol Neurosurg Psychiatry 75(12):1727–1730
Kakumani PL, Hajj-Ali RA (2009) A forgotten cause of central nervous system vasculitis. J Rheumatol 36(3):655
Hart G (1986) Syphilis tests in diagnostic and therapeutic decision making. Ann Intern Med 104(3):368–376
Marra CM (2009) Update on neurosyphilis. Curr Infect Dis Rep 11(2):127–134
Richards A, Van den Maagdenberg Arn MJM, Jen J et al (2007) C-terminal truncations in human 3'-5' exonuclease TREX1 cause autosominal dominant retinal vasculopathy with cerebral leukodystrophy. Nature 39:1068–1070
Terwindt GM, Haan J et al (1998) Clinical and genetic analysis of a large Dutch family with autosomal dominant vascular retinopathy, migraine and Raynaud's phenomenon. Brain 121:303–316
Ophoff RA, DeYoung J et al (2001) Hereditary vascular retinopathy, cerebroretinal vasculopathy, and hereditary endotheliopathy with retinopathy, nephropathy and stroke map to a single locus on chromosome 3p21.1-p21.3. Am J Hum Genet. 69:447–453
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Shahane, A., Khasnis, A. & Hajj Ali, R. Three unusual mimics of primary angiitis of the central nervous system. Rheumatol Int 32, 737–742 (2012). https://doi.org/10.1007/s00296-010-1679-8
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DOI: https://doi.org/10.1007/s00296-010-1679-8