Polyarteritis nodosa (PAN) was first described as an inflammatory arterial disorder by Kussmaul and Maier in 1866 [1]. PAN was characterized as a necrotizing vasculitis of small- to-medium-sized arteries. It was subclassified into systemic, cutaneous/subcutaneous, and microscopic types. Cutaneous/subcutaneous PAN is generally recognized to have a good prognosis [1]. PAN-associated vasculitis localized to the breast is very rare, with few reported cases [2–9]. Most of the cases were diagnosed as giant cell arteritis (GCA) [2–5], as PAN was considered uncommon [6–8]. We herein report a case of PAN of the breast associated with polyenthesitis. A 71-year-old Japanese woman presented with bilateral breast pain, myalgia, enthesitis of both patellar ligaments, fever, weight loss, and general fatigue. She was admitted to our hospital. Physical examination was significant for five tender breast nodules, approximately 1–2 cm in diameter, with associated erythema (Figure 1A). There were no other skin lesions. Laboratory tests demonstrated elevated levels of C-reactive protein (CRP) of 19.8 mg/dl, erythrocyte sedimentation rate (ESR) of 124 mm/1 h, and white blood cell count of 18.4 × 109/L. Anti-proteinase 3, myeloperoxidase neutrophil cytoplasmic antibodies, and anti-nuclear antibodies were all negative. Hepatic and renal functions were normal. Human leukocyte antigen (HLA) typing demonstrated positive A2, A31, B46, and B61 antigens. Physical examination, mammography, and ultrasonography did not show any architectural distortions and calcifications which are considered as the sign of breast cancer. A biopsy of a nodule in the left breast revealed leukocytoclastic medium-sized arteritis without giant cell invasion (Figure 1B and C), indicating PAN. Breast nodule did not reveal typical septal and lobular panniculitis. Flurodeoxyglucose (FDG)-positron emission tomography/computed tomography showed high FDG uptake in multiple entheses, including spinous processes (Figure 1D), both ischial tuberosities, and patellar ligaments (Figure 1E), indicating polyenthesitis. Ultrasonography of the left patellar ligament demonstrated a positive power Doppler signal in the tibial insertion, indicating tibial tuberosity enthesitis (Figure 1F). The patient was diagnosed with PAN limited to the breasts associated with polyenthesitis. Treatment with daily oral prednisolone (40 mg) was initiated, resulting in immediate improvement in the patient's symptoms and normalization of the serum CRP and ESR values. She was discharged and had experienced no subsequent complications or flare-ups for 3 years.
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Apr 19, 2019
Kelly L Brown + 13
Open Access