A case of extramammary paget’s disease: NEJM
An 89-year-old man presented with a 1-year history of a nonhealing groin rash. The rash was not painful or pruritic. He had previously been treated empirically with oral and topical antibiotics, topical antifungal agents, and topical glucocorticoids without improvement. The physical examination revealed an erythematous velvety plaque with superficial white scale affecting the skin of the groin and scrotum (Panel A).

Results of an incisional skin biopsy showed epidermal infiltration by large atypical cells that had eosinophilic cytoplasm and prominent nuclei, findings that are consistent with Paget cells (Panel B, arrows). Immunohistochemical staining was strongly positive for cytokeratin stains CK7 and CAM 5.2.

Extramammary Paget’s disease (not associated with Paget’s disease of the bone) is an intraepithelial adenocarcinoma that is sometimes misdiagnosed as dermatitis. It most commonly involves the vulva but can also occur in the perianal skin, scrotum, penis, and axilla.

In some patients, it is associated with underlying adnexal or visceral cancer. Treatment options include radiotherapy, photodynamic therapy, topical immunomodulators, and surgery. After discussion of the available treatments, the patient opted for regular surveillance because the lesion was asymptomatic and slow-growing. He died of unrelated causes 12 months after diagnosis.

Source: https://www.nejm.org/doi/full/10.1056/NEJMicm1610755
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