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]

• Dyspepsia

  In April 2009, he re-presented with dyspepsia and some change in his bowel habits. Imaging by ct showed retroperitoneal, pelvic, and inguinal lymphadenopathy. Fine-needle aspiration of the left inguinal lymphadenopathy revealed empd. [current-oncology.com]

• Perianal Rash

  The colonoscopy showed the presence of intense perianal rash with xeroderma, peeling skin, warty lesions and a severe stenosis of the anal orifice (Fig. 1a). Also, no changes in the rectal mucosa and absence of polyps in the colon were observed. [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]

Of the 27 published cases that describe imiquimod treatment of EMPD, 6 report treatment failure (22%), but factors that may contribute to treatment failure are not well understood. [ncbi.nlm.nih.gov]

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

These results indicate that therapies that target HER2 may be useful in treating metastatic EMPD, for which the prognosis is poor. However, there is limited information on the expression and gene amplification of HER2 in metastatic EMPD. [ncbi.nlm.nih.gov]

The etiology of the disease is still unclear. We aimed to study the expression differences of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in EMPD tissues and corresponding adjacent normal tissues. [ncbi.nlm.nih.gov]

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]

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]

[…] 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]

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

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]

Unfortunately, the different treatment protocols chosen in these publications prevent from defining a clear protocol. Nevertheless, in our case, as in others [ 2 ], daily application was not well tolerated. [em-consulte.com]

Hence, exploring novel therapeutic strategies to prevent and treat metastatic EMPD is imperative. Table 1. Current systemic therapy for metastatic extramammary Paget’s disease in case studies and case reports. [doi.org]