Takayasu arteritis (TA) is an inflammatory arteriopathy involving predominantly the aorta and its main branches. The disease evolves in two phases: a first, nonspecific inflammatory stage and a late ‘pulseless’ stage, in which complications related to arterial stenosis and aneurysm formation predominate. In both phases, skin manifestations, such as inflammatory nodules, erythema-nodosum- and pyoderma-gangrenosum-like ulcers, have been described. We report 2 patients with TA, who had cutaneous necrotizing vasculitis as presenting manifestation of the disease. A review of the literature revealed 8 similar cases. TA does not only involve large arteries, but also small blood vessels. The observation that in TA the inflammatory process of the large arteries affects regions of the walls supplied by the vasa vasorum, the anatomy of which bears resemblance to the cutaneous vessel system, suggests that primary involvement of small vessels contributes to the development of the clinicopathological features of TA. Knowledge of the skin manifestations associated with TA remains important for its diagnosis and prompt instauration of life-saving treatment.

Keywords:
Takayasu arteritis, Small-vessel vasculitis, Skin manifestations
1.
Frances C, Boisnic S, Blétry O, Dallot A, Thomas D, Kieffer E, Godeau P: Cutaneous manifestations of Takayasu arteritis: A retrospective study of 80 cases. Dermatologica 1990;181:266–272.
2.
Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, Hoffman GS: Takayasu arteritis. Ann Intern Med 1994;120:919–929.
3.
Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG: Takayasu arteritis: A study of 32 North American patients. Medicine 1985;64:89–99.
4.
Perniciaro CV, Winkelmann RK, Hunder GG: Cutaneous manifestations of Takayasu arteritis: A clinicopathologic correlation. J Am Acad Dermatol 1987;17:998–1005.
5.
Fiessinger JN: Aorto-artérite non spécifique (maladie de Takayasu); in Kahn MF, Peltier AP, Meyer O, Piette JC (eds): Les maladies systémiques. Paris, Médecine-Sciences Flammarion, 1991, pp 713–726.
6.
Ask Upmark E, Fajers CM: Further observations on Takayasu’s syndrome. Acta Med Scand 1956;155:275–291.
7.
Ishikawa K: Natural history and classification of occlusive thromboarteriopathy (Takayasu arteritis). Circulation 1978;57:27–35.
8.
Jennette CJ, Milling DM, Falk RJ: Vasculitis affecting the skin: A review. Arch Dermatol 1994;130:899–906.
9.
Hidano A, Watanabe K: Pyoderma gangraenosum et cardiovasculopathies, en particulier artérite de Takayasu. Ann Dermatol Vénéréol 1981;108:13–21.
10.
Mousa AR, Marafie AA, Dajani AI: Cutaneous necrotizing vasculitis complicating Takayasu’s arteritis with a review of cutaneous manifestations. J Rheumatol 1985;12:607–610.
11.
Cajigas JC, Amigo MC, Pineda C, Herrera R, Sanchez-Torres G, Martinez-Lavin M: Association between Takayasu’s arteritis and cutaneous polyarteritis nodosa. Am J Med 1987;82:382–384.
12.
Leavitt RY, Fauci AS: Polyangiitis overlap syndrome: Classification and prospective clinical experience. Am J Med 1986;81:79–85.
13.
Werfel T, Kuipers JG, Zeidler H, Kapp A, Kiehl P: Cutaneous manifestations of Takayasu arteritis. Acta Derm Venereol 1996;76:496–497.
14.
Nasu T: Pathology of pulseless disease: A systematic study and critical review of twenty-one autopsy cases reported in Japan. Angiology 1963;14:225–242.
15.
Nasu T: Takayasu truncoarteritis. Pulseless disease or aortitis syndrome. Acta Pathol Jpn 1982;32:117–131.
16.
Cid MC, Font C, Coll-Vinent B, Grau JM: Large vessel vasculitides. Curr Opin Rheumatol 1998;10:18–28.
17.
Clarke JA: An X-ray microscopic study of the postnatal development of the vasa vasorum in the human aorta. J Anat 1965;99:877–889.
18.
Heistad DD, Marcus ML: Role of vasa vasorum in nourishment of the aorta. Blood Vessels 1979;16:225–238.
19.
Braverman IM: Ultrastructure and organization of the cutaneous microvasculature in normal and pathologic states. J Invest Dermatol 1989;93:2S–9S.
20.
Clarke JA: The vasa vasorum of normal human lower limb arteries. Acta Anat 1965;61:481–487.
21.
Nakata Y: Vascular lesions due to obstruction of the vasa vasorum. Nature 1966;212:1258–1259.
© 2000 S. Karger AG, Basel
2000
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.