Zuska's Disease
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Also called Zuska's disease (only nonpuerperal case), subareolar abscess is a subcutaneous
abscess An abscess is a collection of pus that has built up within the tissue of the body, usually caused by bacterial infection. Signs and symptoms of abscesses include redness, pain, warmth, and swelling. The swelling may feel fluid-filled when pre ...
of the breast tissue beneath the
areola The human areola (''areola mammae'', or ) is the pigmented area on the breast around the nipple. More generally, an areola is a small circular area on the Human body, body with a different histology from the surrounding Tissue (biology), tissue ...
of the nipple. It is a frequently aseptic inflammation and has been associated with squamous metaplasia of
lactiferous duct Lactiferous ducts are ducts that converge and form a Morphogenesis#Branching morphogenesis, branched system connecting the nipple to the lobules of the mammary gland. When lactogenesis occurs, under the influence of hormones, the breast milk, mil ...
s. The term is usually understood to include breast abscesses located in the retroareolar region or the periareolar region but not those located in the periphery of the breast. Subareolar abscess can develop both during lactation or extrapuerperal, the abscess is often flaring up and down with repeated
fistula In anatomy, a fistula (: fistulas or fistulae ; from Latin ''fistula'', "tube, pipe") is an abnormal connection (i.e. tube) joining two hollow spaces (technically, two epithelialized surfaces), such as blood vessels, intestines, or other h ...
tion.


Pathophysiology

90% of cases are smokers, however only a very small fraction of smokers appear to develop this lesion. It has been speculated that either the direct toxic effect or hormonal changes related to smoking could cause squamous metaplasia of lactiferous ducts. It is not well established whether the lesion regresses after
smoking cessation Smoking cessation, usually called quitting smoking or stopping smoking, is the process of discontinuing tobacco smoking. Tobacco smoke contains nicotine, which is Addiction, addictive and can cause Substance dependence, dependence. As a resu ...
. Extrapuerperal cases are often associated with
hyperprolactinemia Hyperprolactinaemia (also spelled hyperprolactinemia) is a condition characterized by abnormally high levels of prolactin in the blood. In women, normal prolactin levels average to about 13 ng/mL, while in men, they average 5 ng/mL. ...
or with
thyroid The thyroid, or thyroid gland, is an endocrine gland in vertebrates. In humans, it is a butterfly-shaped gland located in the neck below the Adam's apple. It consists of two connected lobes. The lower two thirds of the lobes are connected by ...
problems. Also
diabetes mellitus Diabetes mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained hyperglycemia, high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or th ...
may be a contributing factor in nonpuerperal
breast abscess Mastitis is inflammation of the breast or udder, usually associated with breastfeeding. Symptoms typically include local pain and redness. There is often an associated fever and general soreness. Onset is typically fairly rapid and usually occ ...
.


Treatment

Treatment is problematic unless an underlying endocrine disorder can be successfully diagnosed and treated. A study by Goepel and Panhke provided indications that the inflammation should be controlled by bromocriptine even in absence of hyperprolactinemia. Antibiotic treatment is given in case of acute inflammation. However, this alone is rarely effective, and the treatment of a subareaolar abscess is primarily surgical. In case of an acute abscess, incision and drainage are performed, followed by antibiotics treatment. However, in contrast to peripheral breast abscess which often resolves after antibiotics and incision and drainage, subareaolar breast abscess has a tendency to recur, often accompanied by the formation of
fistula In anatomy, a fistula (: fistulas or fistulae ; from Latin ''fistula'', "tube, pipe") is an abnormal connection (i.e. tube) joining two hollow spaces (technically, two epithelialized surfaces), such as blood vessels, intestines, or other h ...
s leading from inflammation area to the skin surface. In many cases, in particular in patients with recurrent subareolar abscess, the excision of the affected lactiferous ducts is indicated, together with the excision of any chronic abscess or fistula. This can be performed using radial or circumareolar incision. There is no universal agreement on what should be the standard way of treating the condition. In a recent review article, antibiotics treatment, ultrasound evaluation and, if fluid is present,
ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...
-guided
fine needle aspiration Fine-needle aspiration (FNA) is a diagnostic procedure used to investigate lumps or masses. In this technique, a thin (23–25 gauge (0.52 to 0.64 mm outer diameter)), hollow needle is inserted into the mass for sampling of cells that, ...
of the abscess with an 18 gauge needle, under saline lavage until clear, has been suggested as initial line of treatment for breast abscess in puerperal and non-puerperal cases including central (subareolar) abscess (see
breast abscess Mastitis is inflammation of the breast or udder, usually associated with breastfeeding. Symptoms typically include local pain and redness. There is often an associated fever and general soreness. Onset is typically fairly rapid and usually occ ...
for details). Elsewhere, it has been stated that treatment of subareolar abscess is unlikely to work if it does not address the ducts as such. Duct resection has been traditionally used to treat the condition; the original Hadfield procedure has been improved many times but long-term success rate remains poor even for radical surgery. Petersen even suggests that damage caused by previous surgery is a frequent cause of subareolar abscesses. Goepel and Pahnke and other authors recommend performing surgeries only with concomitant bromocriptine treatment.


Squamous metaplasia of lactiferous ducts

Squamous metaplasia of lactiferous ducts - abbreviated SMOLD is a change where the normal double layer cuboid epithelium of the lactiferous ducts is replaced by
squamous Epithelium or epithelial tissue is a thin, continuous, protective layer of cells with little extracellular matrix. An example is the epidermis, the outermost layer of the skin. Epithelial ( mesothelial) tissues line the outer surfaces of man ...
keratin Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. It is the key structural material making up Scale (anatomy), scales, hair, Nail (anatomy), nails, feathers, horn (anatomy), horns, claws, Hoof, hoove ...
izing cell layers. The resulting epithelium is very similar to normal skin, hence some authors speak of epidermalization. SMOLD is rare in premenopausal women (possibly 0.1-3%) but more frequent (possibly up to 25%) in
postmenopausal Menopause, also known as the climacteric, is the time when menstrual periods permanently stop, marking the end of the reproductive stage for the female human. It typically occurs between the ages of 45 and 55, although the exact timing can ...
women where it does not cause any problems at all. SMOLD appears to be a completely benign lesion and may exist without causing any symptoms. In principle it ought to be completely reversible as the classification as
metaplasia Metaplasia () is the transformation of a cell type to another cell type. The change from one type of cell to another may be part of a normal maturation process, or caused by some sort of abnormal stimulus. In simplistic terms, it is as if the ...
would suggest. Because of difficulties in observing the actual changes and rare incidence of the lesion this does not appear to be documented. The last section of the lactiferous ducts is always lined with squamous keratinizing epithelium which appears to have important physiological functions. For example, the keratin forms plugs sealing the duct entry and has bacteriostatic properties. In SMOLD the keratinizing lining which is supposed to form only the ends of the lactiferous ducts extends deep into the ducts. SMOLD is distinct from squamous metaplasia that may occur in papilomatous hyperplasia. It is believed to be unrelated to squamous cell carcinoma of the breast which probably arises from different cell types. The keratin plugs (debris) produced by SMOLD have been proposed as the cause for recurrent subareolar abscesses by causing secretory stasis. The epidermalized lining has also different permeability than the normal lining, hindering resorption of glandular secretions. The resorption is necessary to dispose of stalled secretions inside the duct - and at least equally important it affects osmotic balance which in turn is an important mechanism in the control of lactogenesis (this is relevant both in puerperal and nonpuerperal
mastitis Mastitis is inflammation of the breast or udder, usually associated with breastfeeding. Symptoms typically include local pain and redness. There is often an associated fever and general soreness. Onset is typically fairly rapid and usually occ ...
). While in lactating women this would appear to be a very plausible pathogenesis, there is some uncertainty about the pathogenesis in non-lactating women where breast secretions should be apriori minimal. It appears pathologic stimulation of lactogenesis must be present as well to cause subareolar abscess and treatment success with bromocriptin appears to confirm this as compared to poor success rate of the usual antibiotic and surgical treatments documented by Hanavadi et al. Further uncertainty in the relation of SMOLD and the subareolar abscess is that squamous metaplasia is very often caused by inflammatory processes. SMOLD could be the cause of the inflammation – or the result of a previous or longstanding inflammation. SMOLD usually affects multiple ducts and frequently (relative to extremely low absolute prevalence) both breasts hence it is very likely that systemic changes such as hormonal interactions are involved. At least the following factors have been considered in the aetiology of SMOLD: reactive change to chronic inflammation, systemic hormonal changes, smoking, dysregulation in beta-catenin expression, changes in retinoic acid and vitamin D metabolism or expression.
Vitamin A deficiency Vitamin A deficiency (VAD) or hypovitaminosis A is a lack of vitamin A in blood and tissues. It is common in poorer countries, especially among children and women of reproductive age, but is rarely seen in more developed countries. Vitamin A pla ...
may cause epidermilization of the ducts and squamous metaplasia and likely also contributes to infection. Vitamin A deficiency has been observed to cause squamous metaplasia in many types of epithelia. However supplementation with Vitamin A would be beneficial only in exceptional cases because normally the local catabolism of vitamin A will be the regulating factor. Squamous metaplasia of breast epithelia is known to be more prevalent in postmenopausal women (where it does not cause any problems at all). Staurosporine, a nonspecific protein kinase C inhibitor can induce squamous metaplasia in breast tissue while other known PKC inhibitors did not show this effect. cAMP stimulation can also induce squamous metaplasia.


Research

Multiple imaging modalities may be necessary to evaluate abnormalities of the nipple-areolar complex. In two studies performed in Japan, high-resolution
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
with a microscopy coil yielding 0.137-mm in-plane resolution has been used to confirm the presence of abscesses, isolated fistulas and inflammation and to reveal their position in order to guide surgery.


References


Further reading

* {{Diseases of the breast Breast diseases