Zuska's Disease: A Nipple Infection

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Zuska's disease (ZD), also known as Zuska-Atkins disease, is a rare and often painful disorder characterized by breast duct fistulas and recurrent abscesses located in and about the nipple. Zuska was the first to describe this condition in 1951. In subsequent years, different authors proposed other names including mammillary fistula, mammary duct ectasia, lactiferous fistula, periductal mastitis or periareolar abscess. 

ZD usually affects young women during their childbearing years. ZD is most common in women who smoke cigarettes. It is not associated with pregnancy or breastfeeding. It occurs in 1-10% of women, and may also occur in men.

ZD is caused from plugging and blockage of the breast ducts from abnormal changes in the ducts, called squamous metaplasia of lactiferous ducts. A significant factor for this ductal change is cigarette smoking

Zuska’s disease can present in stages. 

  • In the first stage, there is enlargement and plugging of the ducts near the nipple. This stage  can be asymptomatic (the patient has no symptoms) in 30% of cases. 
  • The next stage would include the ducts filling with pus and debris. This ductal change causes sporadic, recurrent, non-menstrual cycle related breast pain near and around the nipple. Nipple area abscesses may then develop which can be further accompanied by redness, fever, swelling and purulent discharge from the edge of the nipple. 
  • The final stage is the development of a draining tract, or sinus. This is called a periareolar fistula, which is a connection between a subareolar duct and the skin. A characteristic finding is milky or pasty drainage from a nipple sinus tract. This is almost always associated with an inverted nipple. 

It is important to differentiate ZD from breast cancer.  Patients should be carefully checked to make sure that they do not have breast cancer, which can include having a physical exam, mammogram and ultrasound. In Zuska’s disease, breast imaging may show a cystic mass or sometimes multiple cystic masses. A biopsy, or tissue sample may need to be obtained to rule out cancer.

ZD treatment may require antibiotics.  Abscesses should be drained when they reach “a head”. Bacteria that live with and without oxygen (aerobic and anaerobic bacteria) may be isolated in cultures from patients with ZD, with the most common organisms being anaerobic Peptostreptococci together with aerobic Staphylococci

Surgery is the only curative treatment for a lactiferous fistula, a disease tract between the abscess and the breast skin. Core excision of the fistula and all of the associated infected breast tissue is the definitive treatment. Smoking cessation is also  essential to help prevent recurrences of ZD.

For more information on Zuska’s disease:
www.onlinelibrary.wiley.com/doi/10.1002/bjs.1800790121/full