Necrobiosis lipoidica (NL) is an idiopathic chronic granulomatous skin condition. There is currently no standardized effective treatment of NL. Ulceration occurs in up to 35% of cases. Treatment of ulcerative lesions is challenging and often unsuccessful. On the basis of the implication of the tumor necrosis factor α (TNF-α) on the formation of granulomas, since 2003 anti-TNF-α agents have been employed in cases of NL refractory to other therapeutic agents. We report a 50-year-old white woman with treatment-resistant chronic ulcerative NL of both shins successfully treated with subcutaneous etanercept. A review of the published literature suggests that biological agents (etanercept and infliximab) should be considered as a therapeutic alternative mainly in ulcerative NL unresponsive to prior conventional regimens. The dose and duration of treatment with these agents is not defined, therefore it is required to report management of these patients in order to develop an optimal therapeutic strategy.

Keywords:
Necrobiosis lipoidica, ulcerated, Etanercept, Infliximab, Biologicals
1.
Hammami H, Youssef S, Jaber K, Dhaoui MR, Doss N: Perforating necrobiosis lipoidica in a girl with type 1 diabetes mellitus: a new case reported. Dermatol Online J 2008;14:11.
2.
Pestoni C, Ferreirós MM, de la Torre C, Toribio J: Two girls with necrobiosis lipoidica and type I diabetes mellitus with transfollicular elimination in one girl. Pediatr Dermatol 2003;20:211–214.
3.
Elmholdt TR, Sørensen HB, Fogh K: A severe case of ulcerating necrobiosis lipoidica. Acta Derm Venereol 2008;88:177–178.
4.
Stanway A, Rademaker M, Newman P: Healing of severe ulcerative necrobiosis lipoidica with cyclosporin. Australas J Dermatol 2004;45:119–122.
5.
Clayton TH, Harrison PV: Successful treatment of chronic ulcerated necrobiosis lipoidica with 0.1% topical tacrolimus ointment. Br J Dermatol 2005;152:581–582.
6.
Darvay A, Acland KM, Russell-Jones R: Persistent ulcerated necrobiosis lipoidica responding to treatment with cyclosporin. Br J Dermatol 1999;141:725–727.
7.
Reinhard G, Lohmann F, Uerlich M, Bauer R, Bieber T: Successful treatment of ulcerated necrobiosis lipoidica with mycophenolate mofetil. Acta Derm Venereol 2000;80:312–313.
8.
Noz KC, Korstanje MJ, Vermeer BJ: Ulcerating necrobiosis lipoidica effectively treated with pentoxifylline. Clin Exp Dermatol 1993;18:78–79.
9.
Kreuter A, Knierim C, Stücker M, Pawlak F, Rotterdam S, Altmeyer P, Gambichler T: Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study. Br J Dermatol 2005;153:802–807.
10.
Benedix F, Geyer A, Lichte V, Metzler G, Röcken M, Strölin A: Response of ulcerated necrobiosis lipoidica to clofazimine. Acta Derm Venereol 2009;89:651–652.
11.
Durupt F, Dalle S, Debarbieux S, Balme B, Ronger S, Thomas L: Successful treatment of necrobiosis lipoidica with antimalarial agents. Arch Dermatol 2008;144:118–119.
12.
Narbutt J, Torzecka JD, Sysa-Jedrzejowska A, Zalewska A: Long-term results of topical PUVA in necrobiosis lipoidica. Clin Exp Dermatol 2006;31:65–67.
13.
Beattie PE, Dawe RS, Ibbotson SH, Ferguson J: UVA1 phototherapy for treatment of necrobiosis lipoidica. Clin Exp Dermatol 2006;31:235–238.
14.
Berking C, Hegyi J, Arenberger P, Ruzicka T, Jemec GB: Photodynamic therapy of necrobiosis lipoidica: a multicenter study of 18 patients. Dermatology 2009;218:136–139.
15.
Pasternack FR, Fox LP, Engler DE: Silicone granulomas treated with etanercept. Arch Dermatol 2005;141:13–15.
16.
Shupack J, Siu K: Resolving granuloma annulare with etanercept. Arch Dermatol 2006;142:394–395.
17.
Haley H, Cantrell W, Smith K: Infliximab therapy for sarcoidosis (lupus pernio). Br J Dermatol 2004;150:146–149.
18.
España A, Sánchez-Yus E, Serna MJ, Redondo P, Robledo A, Quintanilla E: Chronic balanitis with palisading granuloma: an atypical genital localization of necrobiosis lipoidica responsive to pentoxifylline. Dermatology 1994;188:222–225.
19.
Drosou A, Kirsner RS, Welsh E, Sullivan TP, Kerdel FA: Use of infliximab, an anti-tumor necrosis alpha antibody, for inflammatory dermatoses. J Cutan Med Surg 2003;7:382–386.
20.
Kolde G, Muche JM, Schulze P, Fischer P, Lichey J: Infliximab: a promising new treatment option for ulcerated necrobiosis lipoidica. Dermatology 2003;206:180–181.
21.
Cummins DL, Hiatt KM, Mimouni D, Vander Kolk CA, Cohen BA, Nousari CH: Generalized necrobiosis lipoidica treated with a combination of split-thickness autografting and immunomodulatory therapy. Int J Dermatol 2004;43:852–854.
22.
Zeichner JA, Stern DW, Lebwohl M: Treatment of necrobiosis lipoidica with the tumor necrosis factor antagonist etanercept. J Am Acad Dermatol 2006;54:S120–S121.
23.
Hu SW, Bevona C, Winterfield L, Qureshi AA, Li VW: Treatment of refractory ulcerative necrobiosis lipoidica diabeticorum with infliximab: report of a case. Arch Dermatol 2009;145:437–439.
24.
Zhang KS, Quan LT, Hsu S: Treatment of necrobiosis lipoidica with etanercept and adalimumab. Dermatol Online J 2009;15:12.
25.
Santos-Juanes J, Galache C, Curto JR, Carrasco MP, Ribas A, Sánchez del Río J: Squamous cell carcinoma arising in long-standing necrobiosis lipoidica. J Eur Acad Dermatol Venereol 2004;18:199–200.
26.
Smith KJ, Skelton HG: Rapid onset of cutaneous squamous cell carcinomas in patients with rheumatoid arthritis after starting tumor necrosis factor alpha receptor IgG1-Fc fusion complex therapy. J Am Acad Dermatol 2001;45:953–956.
27.
Toussirot E, Pertuiset E, Kantelip B, Wendling D: Sarcoidosis occuring during anti-TNF-alpha treatment for inflammatory rheumatic diseases: report of two cases. Clin Exp Rheumatol 2008;26:471–475.
28.
Voulgari PV, Markatseli TE, Exarchou SA, Zioga A, Drosos AA: Granuloma annulare induced by anti-tumour necrosis factor therapy. Ann Rheum Dis 2008;67:567–570.
© 2010 S. Karger AG, Basel
2010
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.