Coexistence of idiopathic granulomatous mastitis and erythemanodosum: successful treatment with corticosteroids

Abstract

Background/aim: Idiopathic granulomatous mastitis (IGM) is a rare, chronic inflammatory disease of the breast. Erythema nodosum (EN) is a rare extramammary manifestation of IGM. The purpose of this study is to determine the clinical and demographic characteristics of 11 IGM and EN patients and to evaluate the efficacy of methylprednisolone treatment. Materials and methods:In our series, ten patients had EN bilaterally, whereas one patient had a lesion of the right pretibial area. The mean age of the patients was 35.5 years (range: 29-45 years). IGM and EN were diagnosed by the necessary serological, microbiological, radiological, and histopathological examination. After diagnosis, methylprednisolone was started in the first week at 0.8 mg/kg daily for treatment. The weekly dose was tapered to 0.1 mg/kg daily over 8 weeks. Results:We started with the treatment of methylprednisolone, and in all our cases the initial response was excellent. In 2 weeks the IGM symptoms had markedly declined, while signs of EN disappeared completely. Patients were followed for an average of 60 months after treatment. None of the 11 patients had recurrence. Conclusion:We herein report a rare series considering IGM cases complicated by EN. Few such cases have been reported in the literature. We advocate for an initial trial of methylprednisolone treatment, which proved to be very successful in our patients.

A Proposal of a Clinically Based Classification for Idiopathic Granulomatous Mastitis

“…Among the patients in this study group, EN affected patients with patterns B and C Table 2. However, in other reports, EN has been described in patients presenting with a clinical picture similar to the other patterns described in this study (Akin et al, 2017;Bani-Hani et al, 2004).We propose adding a lowercase e to the main pattern of IGM when extra-mammary findings are present Figure 4. Many investigators would recommend using an immunosuppressive treatment when EN is present (Atak et al, 2016;Akin et al, 2017).…”

“…A growing evidence suggesting that the degree of inflammation, the presence of pus, and the presence of extra-mammary findings are indicative of the severity of IGM (Akin et al, 2017;Yılmaz et al, 2018) is in harmony with the potential clinical usefulness of our proposed classification. The observation that such patients will benefit from the early introduction of systemic steroids was supported by a recent study (Uysal et al, 2018).…”

“…Since its first classical description 45 years ago as a painless breast lump that mimics carcinoma (Kessler and Wolloch, 1972), the clinical spectrum of IGM has become wider. Additional local inflammatory signs, with occasional extra-mammary associations, were described (Prasad et al, 2017;Atak et al, 2016;Akin et al, 2017). These variable presentations, in the absence of unified recommendations for surgical and immunosuppressive treatment, and the high rate of local recurrence of IGM added extra challenges for the managing surgeon.…”

Mastitis in Autoimmune Diseases: Review of the Literature, Diagnostic Pathway, and Pathophysiological Key Players

“…Nevertheless, the "idiopathic" nature of IdGM is unclear in some reported cases. For example, extramammary symptoms frequently include arthralgia and erythema nodosum [151,155,156]. In addition, some patients have detectable circulating autoantibodies, such as Rheumatoid Factor, ANA, or anti-DNA antibodies [144,157], suggesting a role of autoimmunity in the pathophysiology of IdGM [144].…”

“…Topical steroids also appear to be effective in these patients [162]. Other smaller studies support the efficacy of immunosuppressive treatment on the course of this disease [141,151,156,163]. However, considering their potential side-effects, these surgical or immunosuppressive treatments should be proposed only after a period of observation: indeed, David and coll.…”