CK7(-) Top Differential Diagnoses • Squamous cell carcinoma in situ • Melanoma in situ • Clear cell papulosis • Benign mucinous metaplasia of penis • Pagetoid dyskeratosis • Pagetoid reticulosis • Melanoma in situ Clinical Appearance of EMPD This case presented [basicmedicalkey.com]

Patients commonly present with hyper- or hypopigmented pruritic patches or plaques with a non-specific clinical appearance, thus leading to a significant delay between initial presentation and diagnosis. [jcasonline.com]

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]

• Pain

  To our knowledge, there are no prior reports of a metastatic extramammary Paget disease presenting as a painful pathologic vertebral body compression fracture. [ncbi.nlm.nih.gov]

  These therapies involve exercises and techniques that can help reduce pain, improve movement and make everyday tasks easier. [nhs.uk]

• Lymphadenopathy

  Based on this clinical observation, multiple lymphadenopathy may represent an initial sign of EMPD. [ncbi.nlm.nih.gov]

  No patient had inguinal lymphadenopathy. Two patients were alive with disease. Immunohistochemically, GATA3 and GCDFP15 were expressed in 6/6 cases, CK7 in 8/8 cases, and androgen receptor in 13/13 cases. HER2 was positive in 5/12 cases. [jhu.pure.elsevier.com]

  The remaining 2 cases were metastatic Paget at diagnosis, one with extensive pelvic and retroperitoneal lymphadenopathy and osseous involvement, the other with right inguinal lymphadenopathy at diagnosis. [pagepress.org]

  While admitted to the hospital, the patient underwent excision of primary site with a sentinel node biopsy (SLNB) of the left inguinal lymphadenopathy. [e-ijd.org]

  One patient had metastatic lymphadenopathy at the groin and simultaneous lymph node dissection was performed. Primary closure of the cutaneous defect was achieved only in 8.3% of the operations. [hindawi.com]

• Surgical Procedure

  Paget’s Disease of the Vulva is best treated using surgical procedures. In most cases, a wide surgical excision and removal of the entire tumor is the preferred treatment option. [dovemed.com]

  There were no significant differences among the three surgical procedures with respect to the cosmetic results. [kiss.kstudy.com]

  The edges of the growth can be really hard to see, so a special surgical procedure, Mohs microscopic surgery, may be used to help find the edges of the tumour. [allthingsvagina.com]

  Many patients undergo multiple surgical procedures, including wide local excision, simple vulvectomy or radical vulvectomy. [doi.org]

• Severe Pain

  Mammography and ultrasonography could not be performed because of severe pain and erosive wetness. [file.scirp.org]

• Short Stature

  […] disease Partial lipodystrophy with Rieger anomaly and short stature - See SHORT syndrome Partial monosomy 10p - See Chromosome 10p deletion Partial monosomy 10q - See Chromosome 10q deletion Partial monosomy 11p - See Chromosome 11p deletion Partial [elbiruniblogspotcom.blogspot.com]

• Dyspnea

  After two cycles of carboplatin/gemcitabine, he developed significant dyspnea on exertion and his chemotherapy was changed to cisplatin/doxorubicin. [doi.org]

• Nausea

  Objectives A previously well 6-year old girl was referred with 6 weeks of abdominal pain, nausea, weight loss, night sweats, & lethargy. Examination was unremarkable apart from a 2cm lump in the right popliteal fossa. [ard.bmj.com]

  The patient reported marked local irritation, erosions, and tenderness, but did not report flulike symptoms, nausea or diarrhea. [doi.org]

  […] estrogen and progesterone pills Used if the woman does not desire to become pregnant They are more effective than using estrogen / progesterone alone They decrease blood loss by 50% and prevent dysmenorrhoea Side effects include breakthrough bleeding, nausea [t.me]

  […] with the added clinical benefit of delivering the intracellular antimicrotubular agent DM1 into the HER2-overexpressing tumor cells by receptor-mediated endocytosis.31 TDM1 is well tolerated, with the most common reported adverse events being fatigue, nausea [ascopubs.org]

  […] subarachnoid haemorrhage]. 31368432 Lungeherniering igennem thoraxvæggen. 31366426 [ADHD in preschoolers and early parent training interventions]. 31366425 [Danish influence on global health]. 31366424 [Prophylaxis and treatment of radiation-induced nausea [nlm.life]

• Vomiting

  […] kan være et tegn på malignitet. 31267942 [Folate deficiency as a differential diagnosis to severe pre-eclampsia]. 31267941 [Tick-borne encephalitis-associated meningoradiculoneuritis acquired in the south-western part of Denmark]. 31267940 [Nausea and vomiting [nlm.life]

  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]

  Side effects are dose related and resolve with the decreased frequency of application or with rest periods from treatment.80 Also reported are post‐inflammatory hypo‐pigmentation or hyperpigmentation, nausea and vomiting and, rarely, a pseudo‐influenza [onlinelibrary.wiley.com]

• Hiccup

  See Chronic hiccups Persistent hyperinsulinemic hypoglycemia of infancy - See Congenital hyperinsulinism Persistent Mullerian duct syndrome Persistent mullerian duct syndrome, types 1 and 2 - See Persistent Mullerian duct syndrome Persistent oviduct [elbiruniblogspotcom.blogspot.com]

• Dermatitis

  Allergic / irritant contact dermatitis Seborrheic dermatitis Psoriasis Lichen simplex chronicus Bowen disease Basal cell carcinoma Candidiasis Tinea cruris Intertrigo Hailey-Hailey disease Glucagonoma syndrome Lichen sclerosus Lichen planus Erythroplasia [visualdx.com]

  It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and [ncbi.nlm.nih.gov]

  Interface dermatitis; Spongiotic dermatitis; Psoriasiform dermatitis; Vesiculobullous and pustular dermatoses; Intraepidermal (see Fig. 0.13E); Subepidermal (see Fig. 0.13F); Vasculitis/pseudovasculitis; Nodular and diffuse dermatitis; Folliculitis/ [worldcat.org]

• Skin Lesion

  Metastatic EMPD showed significantly shorter in the delay in diagnosis and the greater length of skin lesion in contrast to others. [ncbi.nlm.nih.gov]

  Associations GI malignancy GU malignancy solitary or multiple dry, reddened, elevated skin lesions with indolent growth locations include penoscrotal, anorectal and vulval lesions may ulcerate pruritus (may occur before any visible skin abnormality) initially [radiopaedia.org]

  We present a case of a 65-year-old woman with inflammatory skin lesions of both axillae clinically unresponsive to long-term conventional topical therapy. [jcasonline.com]

  During operation the skin lesion was observed to have extended to the anal canal and the rectum. Histopatholagical exarninatian showed the same kind of carcinoma cells, i.e.,Paget's cells in the anal canal tumor as well as the skin lesion. [pifukezazhi.com]

  If you have a specific question or concern about a skin lesion or disease, please consult a dermatologist. [aocd.org]

• Pruritus

  […] carcinoma), face, scalp, chest, abdomen, and extremities Presentation • Circinate or annular moist erythematous scaly patches or plaques • Other presentations: Gray-white eczematous patches, hypopigmented or pigmented macules, ulcerated plaques/tumors • Pruritus [basicmedicalkey.com]

  Here, we report a 76-year-old man presented with pruritus in the perianal area. Investigation revealed a fist-sized perianal erythema, diffuse liver tumors, and right inguinal lymph node swelling. [ncbi.nlm.nih.gov]

  Associations GI malignancy GU malignancy solitary or multiple dry, reddened, elevated skin lesions with indolent growth locations include penoscrotal, anorectal and vulval lesions may ulcerate pruritus (may occur before any visible skin abnormality) initially [radiopaedia.org]

  Published: 03/09/2012 Authors: van der Schoot JHF and Roumen RM A 72-year-old man presented with a solitary eczematous perianal lesion causing a painful pruritus. A biopsy showed an in situ adenocarcinoma with typical Paget’s cells. [bjs.co.uk]

  Vulvar localization remains by far the most common localization. 6 patients, all females, had pruritus (vulvar); 2 (22%) felt pain from the lesions. [alliedacademies.org]

• Hyperpigmentation

  A week after the completion of radiation therapy, the patient’s skin exhibited minimal erythema with surrounding hyperpigmentation to the affected inguinal skin, suggesting clearance of the disease. [cureus.com]

  While these lesions appear as red patches and plaques in fair-skinned persons, in darker-skinned individuals, hyperpigmentation may be prominent. Cohen JM et al. Risk stratification in extramammary Paget disease. [accessmedicine.mhmedical.com]

  For this reason further studies are necessary to clarify the mechanism of hyperpigmentation and the relationship between reactive dendritic melanocytes, naevus cells, melanophages, and Paget's cells in PEMPD. References 1. Lloyd J, Flanagan AM. [jpatholtm.org]

  Lesions occasionally show hypopigmentation or hyperpigmentation. Long-standing lesions may be modified by repeated trauma/excoriation or superimposed infection. [ncbi.nlm.nih.gov]

• Genital Lesions

  The genital lesion exhibited a moderate acanthosis of the epidermis filled with Paget’s cell nests (Fig. 2 a). In both the axillary lesions and the umbilical one, Paget’s cells were located in the thin epidermis (Figs 2 b–d). [medicaljournals.se]

  ROSEN, T.: "Update on genital lesions". JAMA, 209: 1001. 2003. [ Links ] **6. LAI, Y.L.; YANG, W.G.; TSAY, P.K. y cols.: "Penoscrotal extramammary Paget's disease: A review of 33 cases in a 20-year experience". Plast. Reconstr. [scielo.isciii.es]

  Case report and review of the literature === 25 & 26 Titels: 1.Associations of non-melanoma skin cancer and me lanoma, extra-cutaneous cancers and smoking in adults: a US population-based study 2.Non-invasive assessment of benign pigmented genital lesions [oncodermix.nl]

• Confusion

  Histological diagnostic confusion can arise between Paget's disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous disease. [ncbi.nlm.nih.gov]

  Aggregates of atypical lymphocytes (Pautrier microabscesses) may be seen, which can be confused with glandular clusters. [jcp.bmj.com]

  EMPD can occur in both benign or malignant form and is often confused with chronic dermatitis. [aboutcancer.com]

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]

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]

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

• Colitis

  We describe a single case of Paget disease in the peristomal skin of a 61-year-old woman with ulcerative colitis who underwent a proctocolectomy 33 years prior to now with chronic intestinal obstruction and an area of "ectopic mucosa" adjacent to her [ncbi.nlm.nih.gov]

  Misdiagnosed amoebic colitis leading to severe dysentery and necrotizing colitis-Report of a case and review of the literature. Scandinavian Journal of Infectious Diseases 46(3), 235-239. 2014. [regionshospitalet-randers.dk]

  […] carcinoma Pediatric MS - See Pediatric multiple sclerosis Pediatric multiple sclerosis Pediatric onset Crohn's disease - See Pediatric Crohn's disease Pediatric T-cell leukemia Pediatric thyroid cancer - See Thyroid cancer, childhood Pediatric ulcerative colitis [elbiruniblogspotcom.blogspot.com]

  Adenocarcinoma arising from the rectal stump eleven years after excision of an ileal J-pouch in a patient with ulcerative colitis: report of a case. I: Diseases of the Colon and Rectum : vol. 51, nr. 7, s. 1146-1148. 98. Pedersen Søren, Bateman ED. [docplayer.net]

• Complete surgical excision is treatment of choice for primary EMPD Patients require long-term follow-up because of multifocal nature of condition and high recurrence rate • Treatment in secondary lesions will depend on associated carcinoma Only gold [basicmedicalkey.com]

Surgical excision is basically performed as a treatment for EMPD. However, therapeutic options for EMPD in an advanced stage are limited. [karger.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]

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

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]

Patients generally have a very good prognosis depending on the extent of the malignancy. [aocd.org]

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]

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]

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]

[…] 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]

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]

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]

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]

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