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

Abstract

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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Acknowledgments

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). https://doi.org/10.1007/s42399-021-00869-5

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Keywords

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