Calciphylaxis and chronic mastitis: a case report

A 69-years old female patient with end-stage renal disease was treated with hemodialysis for 7 years. Blood tests showed elevated levels of parathyroid hormone and phosphorus and normal calcium levels. For 3 weeks she had a 5-8 cm palpable mass in the right breast with inflammation, skin thickness, and redness.

A bilateral diagnostic mammogram was performed with Tomosynthesis-technique. Images showed skin thickening on the right side. The breast consisted of dense breast tissue, BI-RADS D classification. Widespread vascular calcifications were identified. Additional mixed types of calcifications with linear and heterogeneous aspects were seen throughout both breast parenchyma. The dilated veins in the right breast were remarkable. No mass or stellate distortion was noted in the right breast mammographically. 

Right breast ultrasound demonstrated a heteroreflective aspect of the breast tissue with anechoic ‘pseudocystic’ lesions and hyperechoic changes in the surrounding fat. The abnormalities were in the subcutaneous tissue.

Dilated veins mainly peripherally were also noted on either side. A mass, abscess-collection, or axillary lymphadenopathy could not be identified in the right breast.

Among possible diagnoses, chronic mastitis was assumed at first, based on calcifications on mammography. But a combination of the patient’s history, typical acute clinical and biochemical findings associated with end-stage renal disease, and abnormal imaging findings in the skin and subcutaneous fat tissue, especially additional extensive arterial and soft-tissue calcifications, led to the diagnosis of calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy, is commonly seen in patients with end-stage renal disease [1]. The exact cause of calciphylaxis is unknown, but people with calciphylaxis have elevated levels of calcium, phosphorus, and aluminium [2]. In addition to dialysis, hyperparathyroidism is also a risk factor [2]. Parathyroid hormone regulates the level of calcium and phosphorus in the body. It leads to calcium deposits in the arterioles, which causes fat necrosis due to blood clots. 

Calciphylaxis in the breast is a rare condition. Patients present with skin manifestation because of the occlusion of small vessels within the dermis and subcutaneous tissues leading to skin and soft tissue necrosis, nodules, and necrotic ulcerations [1].

On mammography, it is characterized by extensive arterial calcifications, scattered (coarse) calcifications, architectural distortions, and even spiculations can be seen [3]. Inhomogeneous patterns of fat necrosis and shadowing caused by calcifications are seen on ultrasound.

Radiology can serve an essential role in the diagnosis of calciphylaxis [4]. When it occurs in the breast, it can mimic inflammatory breast cancer with peau d’orange skin changes [1]. Parenchymal distortion and spiculation can be seen in calciphylaxis with fat necrosis and breast cancer.

The damaged microcirculation impairs proper wound healing and is a major threat to any invasive procedures such as breast biopsy [5]. Non-healing infections and sepsis can be side effects of a biopsy. Histology can be inconclusive due to patchy calcifications patterns. Sonography and mammography are sufficient for diagnosis in some patients. It is important to assess whether a biopsy is necessary. The final diagnosis should be made by an interdisciplinary team.

In this case, the diagnosis was based on the diagnostic mammogram and breast ultrasound in combination with the clinical manifestation, patient history, and biochemical findings. No mass was identified in the right breast either mammographically or on ultrasound. So, a biopsy was not needed. The patient was admitted to the nephrology service for medical optimization including a stop of iron- and vitamin D supplementation and initiation of vitamin K supplementation. Regular blood tests were necessary to monitor the levels of calcium, phosphorus, and parathyroid hormone.

The learning point for this case includes recognizing calciphylaxis with chronic mastitis as a cause of a lesion of the breast, especially in a patient with end-stage renal disease. The typical imaging features with extensive microcalcifications can help make the diagnosis.

Written informed patient consent for publication has been obtained.