Symptoms are not specific; most patients report itching, burning, and soreness. A small subset of patients may be asymptomatic. Presence of vulvar pain, bleeding, and tumor formation are reported to be more common in patients affected by invasive disease.Signs and symptoms are skin lesions, often mistaken as eczema, that may be itchy or painful.

Vulvar Paget disease presents as a variety of clinical lesions that may occur over a protracted course. Initially it is velvety, soft, and red or bright pink with scattered white islands of hyperkeratosis. (a strawberry and cream appearance) The lesions become erythematous, plaque like, and desquamating especially when located in dry areas. Rarely the appearance is ulcerated. The borders appear irregular, slightly elevated, and sharply demarcated. The visible borders of vulvar Paget disease are often misleading as Paget cells may spread along the basal layers of normal appearing skin with multicentric foci. Involvement may be extensive including the perianal region, genitocrural, and inguinal folds. Clinical examination should determine the presence of periurethral and perianal lesions. In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur.

A benign papillomatous tumor is derived from epithelium, with cauliflower-like projections that arise from the mucosal surface.

It may appear white or normal colored. It may be pedunculated or sessile. The average size is between 1–5 cm.

Neither sex is significantly more likely to develop them. The most common site is the palate-uvula area followed by tongue and lips. Durations range from weeks to 10 years.

Genital leiomyomas (also known as "Dartoic leiomyomas") are leiomyomas that originate in the dartos muscles of the genitalia, areola, and nipple.

An inverted nipple (occasionally invaginated nipple) is a condition where the nipple, instead of pointing outward, is retracted into the breast. In some cases, the nipple will be temporarily protruded if stimulated. Women and men can have inverted nipples.

A papilloma (plural papillomas or papillomata) ("" + "") is a benign epithelial tumor growing exophytically (outwardly projecting) in nipple-like and often finger-like fronds. In this context refers to the projection created by the tumor, not a tumor on an already existing papilla (such as the nipple).

When used without context, it frequently refers to infections (squamous cell papilloma) caused by human papillomavirus (HPV), such as warts. Human papillomavirus infection is a major cause of cervical cancer, although most HPV infections do not cause cancer. There are, however, a number of other conditions that cause papilloma, as well as many cases in which there is no known cause.

Signs of duct ectasia can include nipple retraction, inversion, pain, and sometimes bloody discharge.

The most common causes of nipple inversion include:

- Born with condition

- Trauma which can be caused by conditions such as fat necrosis, scars or it may be a result of surgery

- Breast sagging, drooping or ptosis

- Breast cancer

- breast carcinoma

- Paget's disease

- Inflammatory Breast Cancer (IBC)

- Breast infections or inflammations

- mammary duct ectasia

- breast abscess

- mastitis

- Genetic variant of nipple shape such as

- Weaver syndrome

- congenital disorder of glycosylation type 1A & 1 L

- Kennerknecht-Sorgo-Oberhoffer syndrome

- Gynecomastia

- Holoprosencephaly, recurrent infections and monocytosis

- Tuberculosis

Around 10–20% of all women are born with this condition. Most common nipple variations that women are born with are caused by short ducts or a wide areola muscle sphincter.

Inverted nipples can also occur after sudden and major weight loss.

Intraductal papillomas of the breast are benign lesions with an incidence of approximately 2-3% in humans.

Two types of intraductal papillomas are generally distinguished. The central type develops near the nipple. They are usually solitary and often arise in the period nearing menopause. On the other hand, the peripheral type are often multiple papillomas arising at the peripheral breasts, and are usually found in younger women. The peripheral type are associated with a higher risk of malignancy.

They are the most common cause of bloody nipple discharge in women age 20-40 and generally do not show up on mammography due to their small size. They may be detectable on ultrasound. A galactogram is the most definitive test but is somewhat invasive.

The masses are often too small to be palpated or felt. A galactogram is therefore necessary to rule out the lesion.

Excision is sometimes performed. Microdochectomy/microdochotomy (removal of a breast duct) is the treatment of choice.

Breast eczema (also known as "Nipple eczema") may affect the nipples, areolae, or surrounding skin, with eczema of the nipples being of the moist type with oozing and crusting, in which painful fissuring is frequently seen, especially in nursing mothers. It will often occur in pregnancy even without breast feeding.

Persisting eczema of the nipple in the middle-aged and elderly needs to be discussed with a doctor, as a rare type of breast cancer called Paget's disease can cause these symptoms.

The key affected features of this condition are described in its name.

Scalp: There are raised nodules over the posterior aspect of the scalp, covered by scarred non-hair bearing skin.

Ears: The shape of the pinnae is abnormal, with the superior edge of the pinna being turned over more than usual. The size of the tragus, antitragus and lobule may be small.

Nipples: The nipples are absent or rudimentary. The breasts may be small or virtually absent.

Other features of the condition include:

Dental abnormalities: missing or widely spaced teeth

Syndactyly: toes or fingers may be partially joined proximally

Renal abnormalities: renal hypoplasia, pyeloureteral duplication

Eye abnormalities: Cataract, coloboma of the iris and asymmetric pupils.

Many conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square meters, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm that forms the dermis and subcutaneous tissues.

The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. Nourishment is provided to these layers by diffusion from the dermis, since the epidermis is without direct blood supply. The epidermis contains four cell types: keratinocytes, melanocytes, Langerhans cells, and Merkel cells. Of these, keratinocytes are the major component, constituting roughly 95 percent of the epidermis. This stratified squamous epithelium is maintained by cell division within the stratum basale, in which differentiating cells slowly displace outwards through the stratum spinosum to the stratum corneum, where cells are continually shed from the surface. In normal skin, the rate of production equals the rate of loss; about two weeks are needed for a cell to migrate from the basal cell layer to the top of the granular cell layer, and an additional two weeks to cross the stratum corneum.

The dermis is the layer of skin between the epidermis and subcutaneous tissue, and comprises two sections, the papillary dermis and the reticular dermis. The superficial papillary dermis with the overlying rete ridges of the epidermis, between which the two layers interact through the basement membrane zone. Structural components of the dermis are collagen, elastic fibers, and ground substance. Within these components are the pilosebaceous units, arrector pili muscles, and the eccrine and apocrine glands. The dermis contains two vascular networks that run parallel to the skin surface—one superficial and one deep plexus—which are connected by vertical communicating vessels. The function of blood vessels within the dermis is fourfold: to supply nutrition, to regulate temperature, to modulate inflammation, and to participate in wound healing.

The subcutaneous tissue is a layer of fat between the dermis and underlying fascia. This tissue may be further divided into two components, the actual fatty layer, or panniculus adiposus, and a deeper vestigial layer of muscle, the panniculus carnosus. The main cellular component of this tissue is the adipocyte, or fat cell. The structure of this tissue is composed of septal (i.e. linear strands) and lobular compartments, which differ in microscopic appearance. Functionally, the subcutaneous fat insulates the body, absorbs trauma, and serves as a reserve energy source.

Conditions of the human integumentary system constitute a broad spectrum of diseases, also known as dermatoses, as well as many nonpathologic states (like, in certain circumstances, melanonychia and racquet nails). While only a small number of skin diseases account for most visits to the physician, thousands of skin conditions have been described. Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. Therefore, most current textbooks present a classification based on location (for example, conditions of the mucous membrane), morphology (chronic blistering conditions), etiology (skin conditions resulting from physical factors), and so on. Clinically, the diagnosis of any particular skin condition is made by gathering pertinent information regarding the presenting skin lesion(s), including the location (such as arms, head, legs), symptoms (pruritus, pain), duration (acute or chronic), arrangement (solitary, generalized, annular, linear), morphology (macules, papules, vesicles), and color (red, blue, brown, black, white, yellow). Diagnosis of many conditions often also requires a skin biopsy which yields histologic information that can be correlated with the clinical presentation and any laboratory data.

Nipple adenomas may be felt as a lump under the nipple or areola. They may come to attention because of nipple pain, ulceration, swelling or discharge.

A supernumerary nipple (also known as a third nipple, triple nipple, accessory nipple, polythelia or the related condition: polymastia) is an additional nipple occurring in mammals, including humans. Often mistaken for moles, supernumerary nipples are diagnosed in humans at a rate of approximately 1 in 18 people.

The nipples appear along the two vertical "milk lines," which start in the armpit on each side, run down through the typical nipples and end at the groin. They are classified into eight levels of completeness from a simple patch of hair to a milk-bearing breast in miniature.

"Polythelia" refers to the presence of an additional nipple alone while "polymastia" denotes the much rarer presence of additional mammary glands.

Although usually presenting on the milk line, pseudomamma can appear as far away as the foot.

A possible relationship with mitral valve prolapse has been proposed.

Noninvasive methods to determine duct diameter in live patients are available only recently and it is not clear how the results should be compared with older results from biopsies.

Histologically, dilation of the large duct is prominent. Duct widening with associated periductal fibrosis is frequently included in the wastebasket definition of fibrocystic disease.

In plasma cell rich lesions diagnosed on core biopsies, steroid-responsive IgG4-related mastitis can be identified by IgG/IgG4 immunostaining.

The signs and symptoms are similar to other cervical cancers and may include post-coital bleeding and/or pain during intercourse (dyspareunia). Early lesions may be completely asymptomatic.

Accessory breasts, also known as polymastia, supernumerary breasts, or mammae erraticae, is the condition of having an additional breast. Extra breasts may appear with or without nipples or areolae. It is a condition and a form of atavism which is most prevalent in male humans, and often goes untreated as it is mostly harmless. In recent years, many affected women have had a plastic surgery operation to remove the additional breasts, for purely aesthetic reasons.

A related condition, in which extra nipples form, is called "supernumerary nipple" or "polythelia".

Ulnar–mammary syndrome or Schinzel syndrome is a cutaneous condition characterized by nipple and breast hypoplasia or aplasia.

It has been associated with TBX3.

In some cases, the accessory breast may not be visible at the surface. In these cases, it may be possible to distinguish their appearance from normal breast tissue with MRI. In other cases, accessory breasts have been known to lactate, as illustrated in a woodcut showing a child nursing at ectopic breast tissue on the lateral thigh.

There is some evidence that the condition may be more common in Native American populations.

Paget's disease of the breast can affect the nipple and areola. Symptoms typically only affect one breast. Symptoms may include:

- "Skin." The first symptom is usually an eczema-like rash. The skin of the nipple and areola may be red, itchy and inflamed. After a period of time, the skin may become flaky or scaly.

- "Discharge." A discharge, which may be straw-colored or bloody, may ooze from the area.

- "Sensation." Some women have a burning sensation. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to consult. Lumps or masses in the breast occur in 50% of the patients. In more advanced stages, the disease may cause tingling, increased sensitivity and pain.

- Nipple changes. The nipple may turn inwards be inverted.

- Breast changes. There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.

The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to wax and wane.

Most women do not visit the doctor because they assume Paget's disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed.

Villoglandular adenocarcinoma of the cervix, also villoglandular papillary adenocarcinoma, papillary villoglandular adenocarcinoma and well-differentiated villoglandular adenocarcinoma, abbreviated VGA, is a rare type of cervical cancer that, in relation to other cervical cancers, is typically found in younger women and has a better prognosis.

A similar lesion, "villoglandular adenocarcinoma of the endometrium", may arise from the inner lining of the uterus, the endometrium.

Nipple adenomas most commonly occur in 30- to 40-year-old women, but can also occur in men. They can also occur at any age, including in the elderly, in adolescence, and in infants.

Nipple discharge refers to any fluid that seeps out of the nipple of the breast. Discharge from the nipple does not occur in lactating women. And discharge in non-pregnant women or women who are not breasfeeding may not cause concern. Men that have discharge from their nipples are not typical. Discharge from the nipples of men or boys may indicate a problem. Discharge from the nipples can appear without squeezing or may only be noticeable if the nipples are squeezed. One nipple can have discharge while the other does not. The discharge can be clear, green, bloody, brown or straw-colored. The consistenct can be thick, thin, sticky or watery.

Some cases of nipple discharge will clear on their own without treatment. Nipple discharge is most often not cancer (benign), but rarely, it can be a sign of breast cancer. It is important to find out what is causing it and to get treatment. Here are some reasons for nipple discharge:

- Pregnancy

- Recent breastfeeding

- Rubbing on the area from a bra or t-shirt

- Trauma

- Infection

- Inflammation and clogging of the breast ducts

- Noncancerous pituitary tumors

- Small growth in the breast that is usually not cancer

- Severe underactive thyroid gland (hypothyroidism)

- Fibrocystic breast (normal lumpiness in the breast)

- Use of certain medicines

- Use of certain herbs, such as anise and fennel

- Widening of the milk ducts

- Intraductal pipilloma

- Subareolar abscess

- Mammary duct ectasia

- Pituitary tumor

Sometimes, babies can have nipple discharge. This is caused by hormones from the mother before birth. It usually goes away in 2 weeks. Cancers such as Paget disease (a rare type of cancer involving the skin of the nipple) can also cause nipple discharge.

Nipple discharge that is NOT normal is bloody, comes from only one nipple, or comes out on its own without squeezing or touching the nipple. Nipple discharge is more likely to be normal if it comes out of both nipples or happens when the nipple is squeezed. Squeezing the nipple to check for discharge can make it worse. Leaving the nipple alone may make the discharge stop.

The periareolar glands of Montgomery in the breast are also called Montgomery tubercles or Morgagni tubercles. These periareolar glands are small, papular tissue projections at the edge of the areola (nipple).

Obstruction of the Montgomery tubercles may result in an acute inflammation, a clear or light brownish fluid may drain out of the areola (nipple discharge), and an subareolar mass may develop, the cyst of Montgomery.

Amazia refers to a condition where one or both of the mammary glands is absent (the nipple and areola remain present). This may occur either congenitally or iatrogenically (typically the result of surgical removal and/or radiation therapy). Amazia can be treated with breast implants.

Amazia differs from amastia (the complete absence of breast tissue, nipple, and areola), although the two conditions are often (erroneously) thought to be identical. The terms "amazia" and "amastia" are thus often used interchangeably, even though the two conditions are medically different.