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Paget's disease
Medical expert of the article
Last reviewed: 04.07.2025
[ Causes and pathogenesis of Paget's disease
Paget's disease is a precancerous condition. Extramammary forms are considered to be associated with sweat gland carcinoma. Lesions in the mammary glands are considered to be metastases by per continutatem of breast cancer. Paget's disease can be precipitated by trauma, cicatricial changes, and other endo- and exogenous factors.
Histopathology
Acanthosis, papillomatosis, polymorphism of spinous cells, presence of isjet cells (large cells with light cytoplasm and pale-stained or hyperchromic nucleus) are observed. The cells lack intercellular connections. Inflammatory reaction is observed in the dermis, consisting mainly of lymphocytes, plasma cells and a few mast cells.
Pathomorphology
In fresh elements in the epidermis there is acanthosis with elongation and expansion of epidermal outgrowths, in old elements the epidermis is thinned. The presence of Paget cells among the epithelial cells is characteristic - large, bridgeless cells with 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, displacing and deforming the cells of the epidermis. Glycogen, neutral glycosaminoglycans and a substance like sialomucin are detected in their cytoplasm. Some cells may contain melanin penetrating into them from neighboring melanocytes, while Paget cells themselves are DOPA-negative.
Inflammatory infiltrates of varying intensity are observed in the papillary layer of the dermis. In the epidermis, near the milk ducts, cellular cords of atypical cells are sometimes determined. Electron microscopy revealed that Paget's cells are poorly differentiated, have desmosomes and microvilli. This picture resembles the structure of epithelial cells of the ducts of apocrine or eccrine glands. Histochemically, signs of apocrine differentiation were found in these cells.
Pathohistological changes in extramammary forms of Paget's disease are similar to those in cases of localization in the mammary gland.
Histogenetically, Paget's disease of mammary localization is associated with cells of milk duct cancer, as well as carcinoma from modified apocrine glands. In extramammary localization, a tumor may develop from cells of the infundibular region of hair follicles and apocrine glands due to the ectopic location of appendage cells in the epidermis.
Differential diagnosis should be carried out with microbial eczema, scabies, primary syphilis, chronic pyoderma, superficial candidiasis, basalioma, fungal mycosis, neurodermatitis, herpes infection, melanoma. Paget's disease should be differentiated from Bowen's disease, in which vacuolated epithelial cells can also be detected. However, unlike the latter, Paget's cells do not have intercellular bridges and contain a PAS-positive substance and sialomucin, which gives metachromasia when stained with toluidine blue and thionine. In addition, in Bowen's disease, unlike Paget's disease, lump formation of nuclei inside giant multinucleated epithelial cells, as well as dyskeratosis phenomena are observed. Paget's disease differs from Pagetoid malignant melanoma in that tumor cells penetrate the dermis; Some malignant melanoma cells contain small amounts of melanin and react positively with the monoclonal antibody HMB-45.
Symptoms of Paget's disease
Paget's disease usually develops in people over 40 years of age, mainly in women.
The disease begins as a limited spotted lesion, flaking and resembling eczema. The outline of the lesion is irregular, polycyclic. Over several months or years, the size of the lesion increases, maceration increases, the compaction becomes more distinct, especially at the edges, an erosion is formed, covered with serous-bloody crusts, after removal of which a moist, granular, slightly bleeding surface is visible. Scarring may be observed in the center, as a result of which in some cases the nipple is retracted. Patients with extramammary Paget's disease may have malignant tumors in other locations. The clinical picture of extramammary Paget's disease may resemble psoriasis, limited eczema, or Bowen's disease.
The typical location of the lesion is the areola of the nipple, much less often - other areas of the skin (genitals, perineum, abdomen, armpits). Unilateral lesions of the mammary glands are characteristic. Paget's disease begins with redness, barely noticeable peeling of the nipple of the mammary gland. Then exudative phenomena are noted up to slight oozing, itching. The outline of the lesion is often irregular, polycyclic, its zone very slowly expands and goes beyond the areola. The lesion resembles eczema. Over time (months, years), the edges of the lesion become dense, the surface is covered with serous-bloody crusts. After removing the crusts, a moist granular (due to vegetation), slightly bleeding surface is revealed.
As a result, the nipple is drawn inward until it disappears completely. Palpation reveals tumor-like tissue compaction and enlargement of regional lymph nodes. Itching, burning, and pain are noted to varying degrees.
In extramammary localization, Paget's disease is located in the anogenital area and is clinically manifested by high polymorphism. The lesions can gradually spread to nearby areas of the skin.
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Treatment of Paget's disease
Paget's disease is treated with surgery and/or radiation therapy.