Zuska's breast disease: Lactiferous fistula

Zuska's disease is rare but painful breast disorder which can often cause concern for possible breast cancer. However, Zuska's breast disease is not related to cancer at all, but it is still a serious condition. Also known as lactiferous fistula,or Zuska-Atkins disease, it is a rare and recurrent disorder characterized by draining abscesses around the nipple. Zuska's breast disease can also lead to nipple retraction. Though it is a rare breast disease, many workers in the field believe that it occurs much more frequently than reported.

Zuska's breast disease is basically a form of breast metaplasia

Zuska's disease is actually a form of epithelial squamous breast metaplasia , which is essentially an overproduction of epithelial squamous cells resulting in the obstruction and plugging of the breast ducts. It that sense, it does have some similarities to a potential breast cancer growth, although none of the new cells show any atypical features. Zuska's disease is not associated with pregnancy and lactation, which can raise suspicious of something more worrisome such as breast cancer. The main concern with Zuska's disease is to have it correctly diagnosed as a benign condition, and not breast cancer.

breast ducts are obstructed by keratin debris

Except for the lactational period, the development of subareolar abscesses in the female (or male) breast are generally caused by keratinizing epithelium which has extended deep into the lacticerous ducts. The breast ducts then become obstructed by the keratinacous debris, and may tear. The contents of the breast ducts then 'spill' into the surrounding breast stromal tissue, resulting in this chronic and active granulomatous inflammatory reactive state. If this 'keratinizing epithelium' is not removed, it is highly likely that the condition will recur, and in some cases a 'fistulous tract' (a channel to a discharging sore) can open at the edge of the areola. There is some speculation that this 'lactiferous fistula' fistula development (or Zuska's disease) may be the result of prolonged tobacco use, but this is purely speculative at this point.

Symptoms of Zuska's breast disease

As a result of the blockage of breast ducts , a range of secondary symptoms are caused, including bacterial infection. Other symptoms of Zuska's breast disease may include swelling or a subareolar mass, a draining fistula from behind the nipple,(sometimes called a draining sinus), and possibly a thick, pasty nipple discharge which can also be painful. Zuska's disease can occur in one or both breasts, and can cause recurrent episodes of erysipela (streptococcus bacterial infection) and the presence of painful nodules under the areola.

Mammographic and cytological aspects of Zuska's breast disease

In order to ascertain the cause of the discharges, a full breast cancer screening process can be expected. Mammograms of the disorder will tend to suggest a cystic mass or multiple cystic masses, with increased density. Ultrasonography of the breast will also tend to show the cystic nature of the masses, more clearly than mammography.

keratinized squamous epithelium

On histologic examination, in all instances of Zuska's breast disease, keratinizing squamous epithelium had replaced the lining of one or more lactiferous ducts right into the subareolar tissue. It is possible that the recurrent breast abscesses could be caused by primary or secondary immune deficiencies, for example IgA deficiency or common variable immunodeficiency (CVID), as they are the most common immunodeficiency diseases in adults. But an analysis of blood samples and a comparision of imaging studies should quickly rule those types of diseases out.

Treatment and management of Zuska's disease of the breast

The first treatment issue, of course, is to assertain than the cause of the recurrent absesses in not breast cancer, but secondary infection resulting from the accumulation of keratinized epithelium blocking breast duct channels. But even though it is a benign disease as far as cancerous cell growth and other of 'neoplastic' of hyperplastic breast diseases, it still is a very serious and painful health issue, and the treatment requires dramatic measures, unfortunately. In an acute of chronic phase, Zuska's breast disease must be treated with antibiotics. Sometimes incisional-drainage procedures have been utlized to relieve the symptoms, but this fails to address the fundamental and recurrent problem. Multiple drainage excisions can also lead to many undesired results such as scarring, nipple and breast distortion, and yet not cure the problem. Mismanagement of this nature can even lead to mastectomy. Only about half of patients can have the condition successfully managed using antibiotics and drainage excisions.

breast Zuska's disease is treated with surgery

So, the definitive therapy for Zuska's breast disease is the surgical excision of the fistula, all of the retroareolar fibroglandular tissue, and any ductal tissue within the nipple. Sometimes this procedure is technically referred to as a "radial elliptical incision with primary closure." The cure rate with this surgical procedure is around 91%, and cosmetically, about 95% of women express satisfaction with the outcome of the nipple and breast.

 

References

  1. Zuska, JJ., Crile, G., Ayres, WW. (March 1951). "Fistulas of lactifierous ducts". Am. J. Surg. 81 (3): 3127.
  2. Passar, M. et al. (1993). "Lactiferous Fistula." Journal of the American College of Surgery178(1), 29-32.
  3. Lester, Susan. Subareolar Abscess (Zuska's Disease): A Specific Disease Entity With Specific Treatment and Prevention Strategies. Pathology Case Reviews. 4(5):189-193.
  4. Guadagni M, Nazzari G Zuska's disease. G Ital Dermatol Venereol 2008 Apr; 143(2):157-60.
  5. Guray M, Sahin A. Benign breast diseases: classification, diagnosis, and management. Oncologist 2006; 11:435–449.
  6. Donegan WL. Common benign conditions of the breast. In: Donegan WL, Spratt JS, editors. Cancer of the Breast, Fifth Edition. St. Louis; Saunders: 2002. p.67–110.
  7. Li S, Grant CS, Degnim A, Donohue J. Surgical management of recurrent subareolar breast abscesses:Am J Surg. 2006 Oct;192(4):528-9.
  8. Lannin DR. Twenty-two year experience with recurring subareolar abscess andlactiferous duct fistula treated by a single breast surgeon. Am J Surg. 2004 Oct;188(4):407-10.
  9. Passaro ME - J Am Coll Surg - 01-JAN-1994; 178(1): 29-32.
  10. Rosen PP, ed. Chapter 4. Specific infections. In: Rosen’s Breast Pathology, Second Edition. Philadelphia: Lippincott Williams & Wilkins, 2001: 65–75.
  11. Furlong AJ, al-Nakib L, Knox WF et al. Periductal inflammation and cigarette smoke. J Am Coll Surg 1994;179:417–420.
  12. Singletary, S. Eva; Robb, Geoffrey L. (2004). Advanced Therapy of Breast Disease. Hamilton, Ont: B.C. Decker. pp. 4.

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Copyright Steven B. Halls, MD Last edited 21-August-2011

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