Presentation
When present, the prognosis is the poorest. About 31 (62%) of 50 patients with mammary Paget disease present with a detectable breast mass. [web.archive.org]
Pigmented mammary Paget disease (PMPD) is a rare disease that may mimic cutaneous melanoma both in clinical presentation and on histology. [ncbi.nlm.nih.gov]
Since, the initial presentation is limited to skin; it is the dermatologist who plays a key role in making a diagnosis, thus, facilitating proper management. [ijdvl.com]
Presentation It is usually unilateral but can occur bilaterally. Some cases may occur following mastectomy despite no breast tissue being present. [ 4 ] It usually involves the nipple but can extend to involve other areolar skin. [patient.info]
Gastrointestinal
- Overeating
Paget's disease of the breast occurs most often in women over age 50. Most women with Paget's disease of the breast have underlying infiltrating ductal breast cancer. Only in rare cases is the cancer confined to the nipple itself. [web.archive.org]
The procedure consists in the removal of the breast, the lining over the chest muscles and a part of the lymph nodes from under the arm. [en.wikipedia.org]
Any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy. [ijdvl.com]
- Diarrhea
This is attributed to immune-mediated destruction of precursors in marrow as a consequence of bovine virus diarrhea vaccination of dams, with calves receiving offending allo-antibodies from colostrum. [eclinpath.com]
steatorrhea and bleeding with bile acid diarrhea and vitamin B12 malabsorption commonly found due to ileal involvement. [en.wikipedia.org]
Skin
- Hyperpigmentation
Local production of melanocytic chemotactic factor by neoplastic cells of the mammary carcinoma when they reach the dermoepidermal junction has been postulated as the cause of the melanocytic proliferation and clinical hyperpigmentation of these epidermotropic [ncbi.nlm.nih.gov]
Nipple invagination, deformed nipple-areola complex, marked erythema, and alternating hyperpigmentation and hypopigmentation noted in the adjacent skin of the breast in a 65-year-old woman with biopsy-proven Paget disease. [web.archive.org]
Nipple invagination, deformed nipple-areola complex, marked erythema, and alternating hyperpigmentation and hypopigmentation noted in adjacent skin of breast in 65-year-old woman with biopsy-proven Paget disease. [emedicine.medscape.com]
Lesions occasionally show hyperpigmentation or hypopigmentation. 26 In the anogenital region atypical appearances occur; ulceration or areas of leukoplakia have been reported. [doi.org]
- Excoriation
It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. [ncbi.nlm.nih.gov]
Erythema Scaling Itching Burning sensation Ulceration Oozing with serosanguineous discharge Bleeding Some combination of the above symptoms Early symptoms and signs of mammary Paget disease Excoriation from itching Resolution and recurrence of small vesicles [web.archive.org]
[…] several symptoms, including the following [16] : Erythema Scaling Itching Burning sensation Ulceration Oozing with serosanguineous discharge Bleeding Some combination of the above symptoms Early symptoms and signs of mammary PD include the following: Excoriation [emedicine.medscape.com]
[…] adjuvant chemotherapy + Disease free 5 months 5 Nakamura S [ 8 ] 83 Bloody discharge, pigmented − Simple mastectomy N/A Disease free 9 months 6 Bernardi M [ 19 ] 52 Discolored, pruritus − Failed to return for follow-up N/A N/A 7 Ucar AE [ 10 ] 74 Bilateral excoriation [surgicalcasereports.springeropen.com]
Extramammary Paget's disease is a slow growing tumour and the appearances of long standing lesions may be modified by repeated traumatisation/excoriation or superimposed infection. An associated tumour may be palpable. [doi.org]
Breast
- Nipple Discharge
Other symptoms include: Redness Swelling Oozing nipple discharge Scaling Bloody nipple discharge Ulceration Inversion of the nipple Scaly, crusty and thickened plaques often form on the nipple, which then spreads to the surrounding area. [dermnetnz.org]
During a biopsy, your doctor obtains a small sample of tissue from the skin of your nipple for examination under a microscope. If you have nipple discharge, a sample of the discharge might also be collected for microscopic examination. [web.archive.org]
- Bloody Nipple Discharge
discharge A flattened or inverted nipple Affected areas beyond the nipple and areola A distinct lump underneath the nipple and areola Skin and nipple changes usually in one breast only Fluctuating skin changes early on, making it appear as if your skin [web.archive.org]
Other symptoms include: Redness Swelling Oozing nipple discharge Scaling Bloody nipple discharge Ulceration Inversion of the nipple Scaly, crusty and thickened plaques often form on the nipple, which then spreads to the surrounding area. [dermnetnz.org]
nipple discharge A flattened or turned-in (inverted) nipple A lump in the breast Thickening skin on the breast Signs and symptoms usually occur in one breast only. [mayoclinic.org]
- Bleeding from the Nipple
[…] an ulcer the nipple might flatten out or turn inwards (invert) there may be a discharge or bleeding from the nipple the area might feel itchy or sore there may be a lump in the same breast. [breast-cancer.canceraustralia.gov.au]
Workup
Proper recognition of this disorder is required to initiate an appropriate workup (eg, skin biopsy) for differentiating it from other benign inflammatory dermatosis and for detecting an underlying breast carcinoma. [web.archive.org]
Treatment
We propose a treatment algorithm for patients presenting with Paget's disease of the breast. [ncbi.nlm.nih.gov]
The histogenesis of Paget's disease of the breast continues to be debated and is important when considering treatment options. [doi.org]
Surgery Surgery is the main treatment for Paget’s disease. [web.archive.org]
Prognosis
Patients generally have a very good prognosis depending on the extent of the malignancy. [aocd.org]
Treatment and Prognosis Mastectomy had been considered the first line of treatment for many years because patients were more commonly found to have more than one tumor within the same breast. [news-medical.net]
[…] term=extramammary+paget%27s+disease&recr Last updated: 3/8/2016 The prognosis for extramammary Paget disease (EMPD) depends on early diagnosis and surgical treatment. [rarediseases.info.nih.gov]
[…] with mammary Paget's disease and no underlying tumour, 30% may develop an invasive carcinoma at a later date and 20% of patients already have an associated in-situ carcinoma of the breast. [ 11 ] Factors of unfavorable prognosis include the presence [patient.info]
Etiology
The purpose of the work is to make a clinical laboratory evaluation of the role of urogenital opportunistic pathogenic flora in etiology and pathogenesis of pathological uterine cervix conditions and to identify possible preventive measures to uterine [giog.it]
Etiology The disease is caused by a mutation in the gene encoding 3-beta-hydroxy-delta-5-C27 steroid oxidoreductase (HSD3B7, 16p12-p11.2). Transmission is autosomal recessive. [orpha.net]
Occasionally two or three bone marrows performed over time are necessary to establish an etiology. Referral to a center that specializes in bone marrow failure disorders is indicated is such cases. [cancertherapyadvisor.com]
[…] genitalia and perineal skin may be associated with bladder, urethra, prostate cancer, or colorectal neoplasia. [3] Due to such associations, a complete investigation for an underlying carcinoma should accompany every confirmed diagnosis of EMPD. [4] Etiology [e-ijd.org]
Etiology MPD and EMPD are neoplastic processes. The causes and cells of origin are controversial. Pathophysiology MPD is largely believed to originate from underlying ductal carcinoma of the breast. [dermatologyadvisor.com]
Epidemiology
Oncology, Radiation Therapy (107) Genetics (94) Medical Chemistry and Biochemistry (88) Histology, Embryology (87) Neurology (75) Obstetrics, Gynaecology (72) Cardiology, Angiology (64) Dermatology (54) Otorhinolaryngology (54) Respiratory Medicine (53) Epidemiology [portal.mefanet.cz]
[…] invades the skin; the invasive carcinoma is usually large in size Paget disease with dermal invasion occurs when Paget cells cross the basement membrane of the epidermis; the invasive component is usually small in size ( Arch Pathol Lab Med 2013;137:72 ) Epidemiology [pathologyoutlines.com]
It is a rare type of cancer of the nipple-areola complex presenting as an eczematous lesion of the nipple and is often associated with an underlying in-situ or invasive carcinoma of the breast. [ 1 ] Epidemiology It is seen in 1-4% of cases of female [patient.info]
Summary Epidemiology Prevalence is unknown but may be around 1-9/1,000,000 for overall BAS defects, excluding cerebrotendinous xanthomatosis. [orpha.net]
According to NCI’s Surveillance, Epidemiology, and End Results program, the 5-year relative survival for all women in the United States who were diagnosed with Paget disease of the breast between 1988 and 2001 was 82.6 percent. [cancer.gov]
Pathophysiology
Pathology and Forensic Medicine (310) Internal Medicine (282) Paediatrics, Neonatology (224) Surgery, Traumatology and Orthopaedics (184) Physiology and Pathophysiology (150) Anatomy (149) Dentistry (136) Infectology (117) Microbiology (117) Gastroenterology [portal.mefanet.cz]
[…] of female breast cancer. [ 2 ] It is also seen in male breast cancer but extremely rarely. [ 3 ] Most cases of Paget's disease of the breast are associated with invasive breast cancer - although 40% are associated with ductal carcinoma in situ. [ 2 ] Pathophysiology [patient.info]
Pathophysiology The underlying reason for the condition is not yet established. It might be that tumor growth initiates in ducts before migrating to the external surface of the nipple. [news-medical.net]
In triple or quadruple extra-mammary Paget’s disease, the racial and gender differences in incidence, the pathophysiology underlying simultaneous distant occurrence of three or four lesions, and their malignant potentials are the matters to be elucidated [medicaljournals.se]
Prevention
KEY WORDS: Empowerment - Behavior - Cervical cancer prevention - Women of childbearing age. Last Issue Toc Last Viewed [giog.it]
Prevention Because the cause of Paget's disease of the breast is not known, prevention of this disease is not possible. [medical-dictionary.thefreedictionary.com]
Prevention Making changes in your daily life may help reduce your risk of breast cancer. Try to: Ask your doctor about breast cancer screening. [mayoclinic.org]
They usually undergo radiation therapy after the actual procedure to prevent recurrence. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer. [en.wikipedia.org]