It is exceedingly rare for EMPD to present on the face, chest, or abdomen, and even more unusual for it to present as a pigmented lesion. [ncbi.nlm.nih.gov]

• Perianal Rash

  Fig. 1 Colonoscopy examination in the perianal Paget’s disease (PPD) patient revealed skin condition improvement (a) Presence of intense perianal rash with xeroderma, peeling skin, warty lesions and a severe stenosis of the anal orifice. b-d After 4, [bmccancer.biomedcentral.com]

• Dysphasia

  In September 2011, the patient was experiencing vomiting, dysphasia to solids, and retrosternal pressure. Restaging ct imaging revealed a node compressing the esophagus. [current-oncology.com]

• Penile Lesion

  Biopsy of the penile lesion demonstrated empd. [current-oncology.com]

The diagnosis is based on clinical and histopathological findings and extensive workup to rule out an underlying primary carcinoma is crucial. Surgical excision is the gold standard. [omicsonline.org]

31036146 [Voriconazole-induced periostitis]. 31036145 [Mindfulness in the treatment of sexual difficulties]. 31036144 [Glomuvenous malformations]. 31036143 [Tetanus in Denmark - a forgotten but dangerous disease]. 31036142 [Workup of dysphagia in Denmark [nlm.life]

Therefore, a DM workup should be undertaken for GGA-affected patients who are over 40 years of age. [kiss.kstudy.com]

More recently radiotherapy has been suggested as a suitable treatment when surgical excision or other modalities are not appropriate. We report a case of anogenital extramammary Paget disease and the clinical response to treatment with radiotherapy. [ncbi.nlm.nih.gov]

The prognosis for affected patients is generally favourable; however, the risk of secondary malignancies is unknown. [ncbi.nlm.nih.gov]

If caught early and treated, prognosis is generally good. [en.wikipedia.org]

Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. [escholarship.org]

Three patients had a focal dermal invasion, 1 of whom reportedly died of another etiology 25 months post diagnosis and 2 were disease-free at 24 and 68 months. No patient had inguinal lymphadenopathy. Two patients were alive with disease. [jhu.pure.elsevier.com]

Early recognition is imperative because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease.[1][2][3][4][5] Etiology The etiology of primary EMPD has been debated but appears to be of apocrine origin. [ncbi.nlm.nih.gov]

TERMINOLOGY Abbreviations • Extramammary Paget disease (EMPD) Definitions • Adenocarcinoma involving epidermis and commonly extending to epithelium of eccrine glands &/or hair follicles (adenocarcinoma in situ) • May involve dermis in minority of cases ETIOLOGY [basicmedicalkey.com]

OBJECTIVE: This study aimed to further characterize the epidemiology, clinical manifestations, pathology, immunopathology, outcome from therapy, and associated underlying malignancy in extramammary Paget disease (EMPD). [ncbi.nlm.nih.gov]

This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease. [escholarship.org]

Historical and epidemiological aspects of vulvar Paget disease are summarized. [journals.lww.com]

[…] of cases – Genital EMPD is often associated with genitourinary carcinomas and perianal EMPD is more frequently associated with gastrointestinal carcinomas – Most frequent in perianal EMPD (underlying rectal carcinoma in 1/3 of cases) CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

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]

Clinical examination should determine the presence of periurethral and perianal lesions.[14] In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur.[14] Pathophysiology[edit] H&E stained micrograph of Extramammary Paget's [en.wikipedia.org]

Pathophysiology The precise pathogenesis of EMPD is still the subject of controversy. The literature seems to indicate that extramammary Paget disease does not have a uniform histogenesis. [ncbi.nlm.nih.gov]

Its pathophysiology is still poorly understood. It is associated with synchronous or metachronous malignancies in 50% of cases [ 1 ]. [omicsonline.org]

Please see the following for more information: Pathophysiology and Etiology Extramammary Paget disease (EMPD) arises as a primary cutaneous adenocarcinoma in most cases. [emedicine.medscape.com]

Awareness of these structures in vulvectomy specimens for EMPD is important to prevent misinterpretation of the syringoma-like lesions as an invasive component of the EMPD. [ncbi.nlm.nih.gov]

Click here to view the NCCN Guidelines Panel Members individual disclosures NCCN Guidelines for Treatment of Cancer by Site NCCN Guidelines for Detection, Prevention, & Risk Reduction NCCN Guidelines for Supportive Care NCCN Guidelines for Age Related [nccn.org]

Currently, no specific preventative methods are known for Paget’s Disease of the Vulva. [dovemed.com]