Discussion
IGLM is a rare, chronic benign inflammatory breast disease of unknown etiology and more commonly reported in individuals of Mediterranean descent.13 It is believed that hyperprolactinemia causing extravasation of fat and protein during milk letdown leads to lymphocyte and macrophage migration, resulting in a localized autoimmune response in the breast ducts.10,14
There are 2 types of granulomatous mastitis: idiopathic and specific. Infectious, autoimmune, and malignant causes of granulomatous mastitis (ie, tuberculosis, sarcoidosis, Corynebacterium spp, granulomatosis with polyangiitis, systemic lupus erythematosus, Behçet disease, ductal ectasia, or granulomatous reaction in a carcinoma) must be excluded prior to establishing an IGLM diagnosis, as these can be fatal if left untreated.15 The most frequent findings on ultrasound and mammography are hypoechoic masses and focal asymmetric densities, respectively.3,5 MRI has been proposed more for surveillance in patients with chronic IGLM.4,5 Histopathology—featuring lobular noncaseating granulomas with epithelioid histiocytes; and multinucleated giant cells in a background of neutrophils, lymphocytes, plasma cells, and eosinophils—is the gold standard for diagnosing IGLM.1-12
There are currently no universal treatment guidelines and management usually consists of observation, systemic and topical steroids, or surgery.3,13 Topical and injectable steroids have been effective in treating both initial and recurrent IGLM in patients who are unable to be treated with systemic steroids.16-18 Due to reported high recurrence rates with steroid tapers, adjunctive therapy with methotrexate, azathioprine, colchicine, and hydroxychloroquine have been proposed.1,3-6,10-12
Additionally, antibiotics are recommended only in the management of IGLM when microbial co-infection is concerning, such as with Corynebacterium spp.9,11,19-22 Histologically, this bacterium is distinct from IGLM and demonstrates granulomatous, neutrophilic inflammation within cystic spaces.19-21 Wide surgical excision with negative margins is the only definitive treatment to reduce recurrence and expedite recovery time.2,3,7-10 Notably, surgical excision has been associated with poor wound healing and occasional recurrence compared with medication alone.5,11
Although IGLM is normally a benign process, chronic disease has been related (without causality) to infiltrating breast carcinoma.4 A proposed theory for the development of malignancy suggests that chronic inflammation leading to free radical formation can result in cellular dysplasia and cancer.23
Conclusions
Fifty years after its first description, IGLM is still a poorly understood disease. There remains no consensus behind its etiology or management. In our case, we demonstrated a stepwise treatment progression, beginning with medical therapy before proceeding to surgical cure. Given concerns for poor wound healing and postsurgical infections, monitoring the response and recurrence to an initial trial of conservative medical treatment is not unreasonable. Because of possible risk for malignancy with chronic IGLM, patients should not delay surgical excision if their condition remains refractory to medical therapy alone.