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Erythematous Velvety Plaque of the Scrotum and Penis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

A 70-year-old Chinese male with a medical history of hypertension, hyperlipidemia, and ischemic heart disease was referred to the urology clinic for 1 week of dysuria. He was previously given a course of antibiotics by a primary care physician for the presumptive treatment of urinary tract infection. On examination, there was a nonpruritic, erythematous, velvety plaque with superficial white scaling over the left base of his penis extending to the proximal aspect of the penile shaft (Figure 1). There was a palpable right inguinal lymph node. He was otherwise asymptomatic and claimed that his primary care physician had been treating it as chronic eczema for a few years. He denied exposure to sex workers or application of any creams to the affected area.

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B. Extramammary Paget disease

The patient was diagnosed with extramammary Paget disease (EMPD) of the penis and scrotum. This is a rare genitourinary malignancy primarily affecting men 60 to 70 years old.1 Commonly, EMPD involves the penoscrotal regions, and may sometimes manifest in the perineal and perianal regions. A skin biopsy specimen should be obtained. The differential diagnoses for this lesion include squamous cell carcinoma, Bowen disease, mycosis fungoides, tinea cruris, contact dermatitis, inverse psoriasis, and atopic dermatitis. Clinically, EMPD has an insidious onset, and patients may report itching, burning, or pain in the affected areas prior to appearance of visual lesions. However, an erythematous, velvety, ill-defined plaque is classical of EMPD1 and a skin biopsy is instrumental in obtaining histological proof for diagnosis.

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Article Information

Correction: This article was corrected on October 11, 2018, to correct the display and tagging of author surnames in the byline.

Correspondencing Author: Terence Law, BSc (Hons), MBBS, MRCS, Department of Urology, Singapore General Hospital, Level 5, The Academia, 20 College Rd, Singapore 169856, Singapore (terencelaw83@gmail.com).

Published Online: December 21, 2017. doi:10.1001/jamaoncol.2017.4064

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Bagby  CM, MacLennan  GT.  Extramammary Paget’s disease of the penis and scrotum.  J Urol. 2009;182(6):2908-2909.PubMedGoogle ScholarCrossref
2.
Chanda  JJ.  Extramammary Paget’s disease: prognosis and relationship to internal malignancy.  J Am Acad Dermatol. 1985;13(6):1009-1014.PubMedGoogle ScholarCrossref
3.
Herrel  LA, Weiss  AD, Goodman  M,  et al.  Extramammary Paget’s disease in males: survival outcomes in 495 patients.  Ann Surg Oncol. 2015;22(5):1625-1630.PubMedGoogle ScholarCrossref
4.
Moretto  P, Nair  VJ, Hallani  SE,  et al.  Management of penoscrotal extramammary Paget disease: case series and review of the literature.  Curr Oncol. 2013;20(4):e311-e320.PubMedGoogle ScholarCrossref
5.
Kato  T, Fujimoto  N, Fujii  N, Tanaka  T.  Mapping biopsy with punch biopsies to determine surgical margin in extramammary Paget’s disease.  J Dermatol. 2013;40(12):968-972.PubMedGoogle ScholarCrossref
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