Extramammary Paget's disease - AbsoluteAstronomy.com

Extramammary Paget’s disease (EMPD), also Extramammary Paget disease, is a rare, slow-growing, usually non-invasive intraepithelial (in the skin

Skin

-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...

) adenocarcinoma

Adenocarcinoma

Adenocarcinoma is a cancer of an epithelium that originates in glandular tissue. Epithelial tissue includes, but is not limited to, the surface layer of skin, glands and a variety of other tissue that lines the cavities and organs of the body. Epithelium can be derived embryologically from...

outside of the mammary gland

Mammary gland

A mammary gland is an organ in mammals that produces milk to feed young offspring. Mammals get their name from the word "mammary". In ruminants such as cows, goats, and deer, the mammary glands are contained in their udders...

and includes Paget's disease of the vulva
Vulva
The vulva consists of the external genital organs of the female mammal. This article deals with the vulva of the human being, although the structures are similar for other mammals....

and the extremely rare Paget's disease of the penis
Penis
The penis is a biological feature of male animals including both vertebrates and invertebrates...

. Extramammary Paget’s disease involves primarily the epidermis but occasionally extends into the underlying dermis. It has a predilection for apocrine gland bearing areas, mostly the perineum

Perineum

In human anatomy, the perineum is a region of the body including the perineal body and surrounding structures...

, vulva, axilla, scrotum

Scrotum

In some male mammals the scrotum is a dual-chambered protuberance of skin and muscle containing the testicles and divided by a septum. It is an extension of the perineum, and is located between the penis and anus. In humans and some other mammals, the base of the scrotum becomes covered with curly...

, and penis.

Prognosis and comorbity

Extramammary Paget's disease is usually seen in isolation and is not usually associated with an underlying invasive malignancy. This is unlike Paget's disease of the breast

Paget's disease of the breast

Paget's disease of the breast is a malignant condition that outwardly may have the appearance of eczema, with skin changes involving the nipple of the breast....

, which is almost always associated with an underlying invasive malignancy, i.e. breast cancer

Breast cancer

Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...

(e.g. mammary ductal carcinoma).

Signs and symptoms

Signs and symptoms are a skin lesion often mistaken as eczema

Eczema

Eczema is a form of dermatitis, or inflammation of the epidermis . In England, an estimated 5.7 million or about one in every nine people have been diagnosed with the disease by a clinician at some point in their lives.The term eczema is broadly applied to a range of persistent skin conditions...

that may be itch

Itch

Itch is a sensation that causes the desire or reflex to scratch. Itch has resisted many attempts to classify it as any one type of sensory experience. Modern science has shown that itch has many similarities to pain, and while both are unpleasant sensory experiences, their behavioral response...

y or painful. A biopsy

Biopsy

A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...

will establish the diagnosis

Diagnosis

Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...

. The histology

Histology

Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...

of the lesion is the same as for Paget's disease of the breast.

Types

Paget's disease of the vulva, a rare disease, may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra

Urethra

In anatomy, the urethra is a tube that connects the urinary bladder to the genitals for the removal of fluids out of the body. In males, the urethra travels through the penis, and carries semen as well as urine...

, or the rectum

Rectum

The rectum is the final straight portion of the large intestine in some mammals, and the gut in others, terminating in the anus. The human rectum is about 12 cm long...

and thus be secondary. Patients tend to be postmenopausal.

Paget's disease of the penis may also be primary or secondary and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease

Bowen's disease

Bowen's disease is a neoplastic skin disease, it can be considered as an early stage or intraepidermal form of squamous cell carcinoma. It was named after Mark Bowen...

. Isolated Paget's disease of the penis is extremely rare.

Primary disease

It is important to exclude that the lesion is associated with another cancer. Primary disease is usually treated by surgical

Surgery

Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...

excision

Excision

Excision is the alias of Jeff Abel, a dubstep DJ and music boss from British Columbia, Canada. He frequently works with fellow Canadian dubstep producers Datsik and Downlink. As one of the first dubstep producers and DJs in North America, he has played a significant role in the genre's growth in...

History

James Paget

Sir James Paget, 1st Baronet was a British surgeon and pathologist who is best remembered for Paget's disease and who is considered, together with Rudolf Virchow, as one of the founders of scientific medical pathology. His famous works included Lectures on Tumours and Lectures on Surgical Pathology...

described Paget's disease of the nipple in 1874. Radcliffe Crocker reported the first case of EMPD in 1889 when he described a patient with a skin lesion affecting the penis and scrotum