Paget´s
disease is an intraepithelial adenocarcinoma of the mammary glands. It’s
extra-mammary location was described afterwards. Extra-mammary Paget’s disease
(EMPD) is a rare and slow progressive skin lesion arising from the apocrine
glands located in the perineum, perianal region, groin, scrotum and vulva. Its
incidence and prevalence are not well known. The main histologic feature are
the Paget cells; large, basophil, vacuolated, PAS+ cells. As noted in previous
publications Perianal Paget’s disease can be associated with synchronous ormetachronous visceral malignancies.
This makes necessary to complete the study
of the patient with a colonoscopy and imagery, such as CT scan. And also
imposes a long-term follow-up. Symptoms are nonspecific sometimes leading to a
delay diagnosis. Clinical presentation includes exfoliated, exudative, and
warty or hypopigmented patches associated with perianal pruritus or pain.
Dermatologists generally diagnose it after the lack of response to both local
and systemic treatments. Read more......................
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