Paget's skin disease (Paget's skin cancer) is a type of skin cancer that is very easily confused with other skin conditions. For this reason, Paget's skin cancer is often diagnosed too late, reducing the chances of recovery. What are the causes and symptoms of Paget's skin disease? What is the treatment?

Paget's disease of the skin , or EMPD Extrammamary Paget's disease, is a very rare type of skin cancer, namely, intra-epidermal cancer that originates in the apocrine glands of the skin.

Paget's disease of the skin - causes

There are two types of extra-mammary Paget's disease. In the first type, the tumor begins to develop in the epidermis and then spreads to the adjacent epithelium of the hair follicles and sweat gland ducts. The second hypothesis is that the neoplastic process begins in the epidermis and spreads to the layer of the dermis, from where it metastasizes.

In the second type, the cancer begins in the genitourinary or digestive system, from where it spreads into the adjacent epidermis.

Paget's Disease - Symptoms

In the early stages of the disease, erythema spots appear on the skin, causing itching and a burning sensation. There may be discharge from them. Then, erosions (often bleeding) may appear.

Changes usually occur in the area of ​​the external genitalia: vulva, anus, penis and scrotum - these are areas rich in apocrine and eccrine glands. Less commonly, the cancer affects the top of the thighs, buttocks, armpits and the navel area. There are single cases of localization of neoplastic lesions in areas of the skin devoid of apocrine glands, e.g. in the external auditory canals, on the eyelids, in the esophagus, in the urethra.

Paget's disease of the skin - diagnosis

A fragment of the lesion (biopsy) is taken for histopathological examination, during which the presence of characteristic Paget cells is detected.

The doctor should exclude other skin diseases that may be the cause of the above-mentioned. symptoms - psoriasis, Bowen's disease, atopic dermatitis, seborrheic dermatitis, contact dermatitis, mycosis, melanoma, histiocytosis, mycosis fungoides.

Paget's Disease - Treatment

Treatment consists in cutting the lesion together with a safety margin. For example, forPaget's vulvatreatment includes vulvectomy (removal of part or all of the female external genitalia), a wide excision with a he althy tissue margin, and a skin graft covering the defect. The organ does not lose its functions. Unfortunately, the percentage of relapses is very high, reaching even 43%.

If cancer has infiltrated the perianal area, an abdominoperineal resection may be required, or surgery and chemoradiotherapy may be required.

Paget's disease - prognosis

If the tumor is confined to the epidermis, the prognosis is good. When the cancer also affects the skin or metastasizes through the lymphatic vessels, the prognosis is less favorable.

Bibliography: Głowacka A., Zbarańska K., Chodorowska G.,Extra mammary form of Paget's disease (EMPD) , "Nowa Medycyna" 2005, No. 2.

Category:

Oncology