Paget's disease

Paget's disease (syn: Extramammary Paget's disease, adenocarcinoma acrospinocellulaie epidermotropicum) - cancer in situ, is localized, usually around the nipples or on the nipples of the mammary gland, less often in the genital area, axillary cavities.

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The causes and pathogenesis of Paget's disease

Paget's disease refers to precancerous conditions. It is believed that extramammary forms are associated with carcinoma of the sweat glands. Foci in the area of mammary glands are considered as metastases by per continual breast cancer. As the provoking factors of Paget's disease may be trauma, scar changes and other endo- and exogenous factors.

Histopathology

There are acanthosis, papillomatosis, polymorphism of spine-like cells, the presence of ijet cells (large cells with a light cytoplasm and a pale-stained or hyperchromic nucleus). Cells are devoid of intercellular bonds. In the dermis, an inflammatory reaction is observed, consisting predominantly of lymphocytes, plasma and a few mast cells.

Pathomorphology

In fresh elements in the epidermis - acanthosis with elongation and expansion of epidermal outgrowths, in the old elements the epidermis is thinned. Characteristically, among the epithelial cells of Paget cells - large, devoid of bridges cells with a light cytoplasm and a large nucleus. They are usually especially numerous in the basal layer of the epidermis, they cause its disorganization. As a rule, tumor cells do not penetrate into the dermis. When there are a lot of Paget cells, they form cells, pushing and deforming the cells of the epidermis. In their cytoplasm, glycogen, neutral glycosaminoglycans and a substance of the sialomucin type are detected. Some cells may contain melanin, which penetrates them from neighboring melanocytes, while the Peget cells themselves are DOPA-negative.

In the papillary layer of the dermis inflammatory infiltrates are noted of varying intensities. In the epidermis, near the milk passages, the cellular strands from atypical cells are sometimes determined. Electron microscopy revealed that Paget cells are slightly differentiated, have desmosomes and microvilli. A similar pattern resembles the structure of the epithelial cells of the ducts of the apocrine or eccrine glands. Histochemically, these cells showed signs of apocrine differentiation.

Pathohistological changes with extramammary forms of Paget's disease are similar to those in the localization in the mammary gland.

Histogenetically, Paget's disease of mammary localization is associated with breast cancer cells, as well as carcinomas from modified apocrine glands. With extramammary localization, a tumor can develop from the cells of the infundibular region of the hair follicles and the apocrine glands in connection with the ectopic location of the epididymis of the epididymis.

Differential diagnosis should be conducted with microbial eczema, scabies, primary) syphilis, chronic pyoderma, superficial candidiasis, basioma, fungal mycosis, neurodermatitis, herpetic infection, melanoma. The differentiation of Paget's disease follows from Bowen's disease, in which vacuolated epithelial cells can also be detected. However, unlike the latter, Paget cells do not have intercellular bridges and contain SHIC-positive substances and sialomucine, which gives metachromasia when staining with toluidine blue and thionine. In addition, in Bowen's disease, in contrast to Paget's disease, clotting of nuclei inside giant multinucleated epithelial cells and also the phenomenon of dyskeratosis are observed. From pagetoid malignant melanoma, Paget's disease is characterized by the fact that tumor cells penetrate the dermis; in some malignant melanoma cells there is a small amount of melanin, and they give a positive reaction with monoclonal antibodies HMB-45.

Symptoms of Paget's Disease

Paget's disease develops, as a rule, in people older than 40, mainly in women.

The disease begins as a limited spotted lesion, peeling and resembling eczema. The outlines of the focus are incorrect, polycyclic. Within a few months or years, the size of the focus increases, the maceration increases, the compaction becomes more distinct, especially along the edges, erosion is formed, covered with serous bloody crusts, after which a moist, granular, slightly bleeding surface is visible. In the center, scarring can occur, as a result of which, in some cases, the nipple is drawn. In patients with extramammary form of Paget's disease, malignant tumors of other sites may be observed. The clinical picture of the extramammary localization of Paget's disease may resemble psoriasis, limited eczema or Bowen's disease.

A typical location of the lesion is a halo of the nipple, much less often other parts of the skin (genitalia, perineum, abdomen, axillary cavities). One-sided lesion of mammary glands is characteristic. Paget's disease begins with redness, barely noticeable peeling of the nipple of the breast. Then exudative phenomena are noted up to an easy moknutija, an itch. The contour of the lesion is more often irregular, polycyclic, its zone very slowly widens and extends beyond the nipple circle. The lesion resembles eczema. Over time (months, years), the edges of the focus become dense, the surface is covered with sero-bloody crusts. After removing the crusts, a moist, granular (due to vegetation), a slightly bleeding surface is found.

As a result, the nipple retracts deeper until it disappears completely. Palpation reveals a tumor-like densification of the tissues and an increase in regional lymph nodes. In varying degrees of marked itching, burning and soreness.

With extramammary localization, Paget's disease is located in the anogenital region and is clinically manifested by large polymorphism. The foci can gradually spread to nearby skin areas.

Treatment of Paget's disease

Paget's disease is treated with surgical and / or radiation therapy.