Abstract

Extramammary Paget’s disease (EMPD) is a rare intraepithelial neoplasm, which most commonly involves labia majora, scrotum, and perineum. Due to the insidious onset of disease and its rarity, it can be easily misdiagnosed both clinically and on biopsy. Therefore, it’s important for dermatologists and dermatopathologists to have a high clinical suspicion for EMPD, when evaluating eczematous lesions of genital area in both men and women. Herein, we report a case of EMPD of scrotum in an 81 year old male. This patient initially presented with the complaint of itching in the genital area. On examination, he had a large erythematous rash in the scrotal area. The shave biopsy of the lesion revealed an invasive carcinoma with in-situ component with features favoring squamous cell carcinoma. Subsequently, the patient underwent wide excision. Histological examination revealed intra-epithelial proliferation of large oval cells with ample pale cytoplasm and intracytoplasmic mucin. Additionally, areas of dermal invasion and lymphovascular invasion were identified. Immunohistochemical studies showed that the tumor cells expressed cytokeratin 7, CEA, GATA3, and GCDFP and were negative for markers of squamous cell carcinoma, prostate carcinoma, and carcinoma of germ cell origin or gastrointestinal origin. Therefore, the patient was finally diagnosed with EMPD with dermal invasion. This case reinforces the clinical and histological findings of EMPD. It also highlights the importance of having EMPD in the differentials, to avoid misdiagnosis, when evaluating the eczematous lesions of genital area.

Financial Disclosure:
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