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Erythema Nodosum, Idiopathic Granulomatous Mastitis, and Arthritis—ENIGM(A): a New Case Report and Review of the Literature


Idiopathic Granulomatous Mastitis is a chronic, benign, inflammatory disease of the breast, which has rarely been described within an autoimmune inflammatory triad comprehending erythema nodosum and arthritis. These manifestations, which are more frequent in the Turkish ethnic population, occur in premenopausal women in whom clinical lesions of the breast could resemble mammary cancer or abscess. The diagnosis and management of this inflammatory syndrome are limited by the absence of exhaustive literature. Therefore, identification and treatment are largely based on expert opinion and anecdotal data from available case reports and small case studies. The purpose of this paper is to present an exemplificative case and review the current literature in order to clarify the epidemiology, clinical presentation, histology, and differential diagnosis. Moreover, even if there is no consensus in the literature about the treatment, we propose oral corticosteroid as the first-line therapy, with surgical intervention as a backup in the most challenging cases.

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  1. Kessler E, Wolloch Y. Granulomatous mastitis: a lesion clinically simulating carcinoma. Am J Clin Pathol. 1972;58:642–6.

    CAS  Article  Google Scholar 

  2. Çetin K, Sıkar HE, Güllüoğlu BM. Idiopathic granulomatous mastitis with erythema nodosum: is it a variant of clinical presentation indicating treatment resistance? A retrospective cohort study. Breast J. 2020;26:1645–51.

    Article  PubMed  Google Scholar 

  3. Vural S, Ertop P, Ceyhan K, Şanli H. An unusual cause of oligoarthritis and erythema nodosum: idiopathic granulomatous mastitis. Arch Rheumatol. 2017;32:71–5.

    Article  Google Scholar 

  4. Ben Abid F, Abdel Rahman S, Al Soub H. A case report of TB versus idiopathic granulomatous mastitis with erythema nodosum, reactive arthritis, cough, and headache. Aging Male. 2018;6:1–4.

    Google Scholar 

  5. Fruchter R, Castilla C, Ng E, Pomeranz MK, Femia AN. Erythema nodosum in association with idiopathic granulomatous mastitis: a case series and review of the literature. J Eur Acad Dermatol Venereol. 2017;3:e391–3.

    Article  Google Scholar 

  6. Alungal J, Abdulla MC, Narayan R. Idiopathic granulomatous mastitis with erythema nodosum and polyarthritis. Reumatismo. 2016;68:97–9.

    CAS  Article  Google Scholar 

  7. Kageyama R, Ueda H, Hashizume H. A case of granulomatous mastitis, erythema nodosum and oligoarthralgia in a pregnant woman with a high level of serum granulocyte colony-stimulating factor. Eur J Dermatol. 2016;26:205–7.

    CAS  Article  Google Scholar 

  8. Zabetian S, Friedman BJ, McHargue C. A case of idiopathic granulomatous mastitis associated with erythema nodosum, arthritis, and reactive cough. JAAD Case Rep. 2016;2:125–7.

    Article  Google Scholar 

  9. Fahmy J, Halabi-Tawil M, Bagot M, Tournant B, Petit A. Érythème noueux au cours d’une mastite granulomateuse idiopathique [Erythema nodosum during the course of idiopathic granulomatous mastitis]. Ann Dermatol Venereol. 2015;142:46–9.

    CAS  Article  Google Scholar 

  10. Binesh F, Shiryazdi M, Bagher Owlia M, Azimi S. Idiopathic granulomatous mastitis, erythema nodosum and bilateral ankle arthritis in an Iranian woman. BMJ Case Rep. 2013;2013:bcr2012007636.

    PubMed  PubMed Central  Google Scholar 

  11. Nakamura T, Yoshioka K, Miyashita T, Ikeda K, Ogawa Y, Inoue T, et al. Granulomatous mastitis complicated by arthralgia and erythema nodosum successfully treated with prednisolone and methotrexate. Intern Med. 2012;51:2957–60.

    Article  Google Scholar 

  12. Salesi M, Karimifar M, Salimi F, Mahzouni P. A case of granulomatous mastitis with erythema nodosum and arthritis. Rheumatol Int. 2011;3:1093–5.

    Article  Google Scholar 

  13. Olfatbakhsh A, Beheshtian T, Djavid GE. Granulomatous mastitis, erythema nodosum, and oligoarthritis in a pregnant woman. Breast J. 2008;14:588–90.

    Article  Google Scholar 

  14. Adams DH, Hubscher SG, Scott DG. Granulomatous mastitis--a rare cause of erythema nodosum. Postgrad Med J. 1987;63:581–2.

    CAS  Article  Google Scholar 

  15. Akbulut S, Yilmaz D, Bakir S. Methotrexate in the management of idiopathic granulomatous mastitis: review of 108 published cases and report of four cases. Breast J. 2011;17:661–8.

    CAS  Article  Google Scholar 

  16. Jacquin-Porretaz C, Devalland C, Delapparent T, Nardin C, Dupond AS. Mastite granulomateuse idiopathique associée à un érythème noueux [Idiopathic granulomatous mastitis associated with erythema nodosum]. Ann Dermatol Venereol. 2019;146:571–6.

    CAS  Article  Google Scholar 

  17. Gümüş M, Akkurt ZM, Gümüş H. Is erythema nodosum coexisting with lesions of the breast a suggestive sign for idiopathic granulomatous mastitis? Turk J Surg. 2018;34:71–3.

    PubMed  PubMed Central  Google Scholar 

  18. Akın M, Karabacak H, Esendağlı G, Yavuz A, Gültekin S, Dikmen K, et al. Coexistence of idiopathic granulomatous mastitis and erythemanodosum: successful treatment with corticosteroids. Turk J Med Sci. 2017;47:1590–2.

    Article  Google Scholar 

  19. Kalaycı TÖ, Koruyucu MB, Apaydın M, Etit D, Varer M. Idiopathic granulomatous mastitis associated with erythema nodosum. Balkan Med J. 2016;33:228–31.

    Article  Google Scholar 

  20. Atak T, Sagiroglu J, Eren T, Ali Özemir I, Alimoglu O. Strategies to treat idiopathic granulomatous mastitis: retrospective analysis of 40 patients. Breast Dis. 2015;35:19–24.

    CAS  Article  Google Scholar 

  21. Lucas R, Gussman D, Polis RL, Rattigan MI, Matulewicz TJ. Idiopathic granulomatous mastitis with erythema nodosum simulating breast abscess in pregnancy: a case report. Obstet Med. 2014;7:37–9.

    Article  Google Scholar 

  22. Yaghan R, Hamouri S, Ayoub NM, Yaghan L, Mazahreh T. A proposal of a clinically based classification for idiopathic granulomatous mastitis. Asian Pac J Cancer Prev. 2019;2:929–34.

    Article  Google Scholar 

  23. Chowaniec M, Starba A, Wiland P. Erythema nodosum - review of the literature. Reumatologia. 2016;54:79–82.

    Article  Google Scholar 

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We are indebted to the patient who participated in this report.

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Conceptualization, Edoardo Cammarata; methodology, Paola Savoia; investigation and data curation, Edoardo Cammarata and Paola Savoia; writing–original draft preparation, Edoardo Cammarata; supervision, Paola Savoia. All authors express consent to publication.

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Correspondence to Edoardo Cammarata.

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Cammarata, E., Savoia, P. Erythema Nodosum, Idiopathic Granulomatous Mastitis, and Arthritis—ENIGM(A): a New Case Report and Review of the Literature. SN Compr. Clin. Med. 3, 1295–1300 (2021).

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  • Erythema nodosum
  • Idiopathic granulomatous mastitis
  • Granulomatous chronic diseases
  • Non-caseating granulomas