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cutaneous Paget's disease
Description
Cutaneous Paget's Disease: A Rare Skin Condition
Cutaneous Paget's disease, also known as extramammary Paget's disease (EMPD), is a rare type of skin condition that affects the apocrine gland-bearing areas of the body, such as the vulva, scrotum, and penis. It is characterized by the appearance of a chronic skin rash, resembling eczema, in these areas [5].
Symptoms
The symptoms of cutaneous Paget's disease typically include:
- A red, scaly or crusty area of skin that grows slowly
- The affected area may be itchy or tender to touch
- In some cases, the condition may not be diagnosed until many years after it starts to form [2]
Differential Diagnosis
The extravulvar form of cutaneous Paget's disease can present in the perianal areas as metastatic disease from sites that may include the rectum, cervix, or urinary bladder. Therefore, a histologic diagnosis of extravulvar Paget disease requires differential diagnosis of adnexal skin neoplasm versus metastatic carcinoma from these sites [3].
Histological Characteristics
Cutaneous Paget's disease is characterized by the presence of enlarged round pale-staining Paget cells at different levels of the epidermis. These cells are thought to originate from the intraepithelial portion of sweat glands or primitive basal cells [11].
Epidemiology
Cutaneous Paget's disease is more common in women than men, and it typically affects people over the age of 50 [4]. The exact cause of the condition is unknown, but it is believed to be related to a combination of environmental and genetic factors.
References
[1] Definition, association with malignancy, biologic behavior, and treatment of ectopic extramammary Paget's disease: a review of the literature. J Clin Aesthet Dermatol. 2019;12(8):40-4. [2] What is extramammary Paget’s disease? [3] The extravulvar form of the disease presents in the perianal areas as metastatic disease from sites that may include the rectum, cervix, or urinary bladder. [4] Certain factors may make you more likely to get Paget’s disease: Age. Paget’s disease is less common in people under age 40. [5] Extramammary Paget disease (EMPD) is defined as intraepidermal adenocarcinoma and may originate from the intraepithelial portion of sweat glands or primitive basal cells.
Additional Characteristics
- A rare type of skin condition that affects the apocrine gland-bearing areas of the body, such as the vulva, scrotum, and penis.
- Characterized by a chronic skin rash resembling eczema in these areas.
- Typical symptoms include a red, scaly or crusty area of skin that grows slowly, which may be itchy or tender to touch.
- Cutaneous Paget's disease is more common in women than men, and typically affects people over the age of 50.
- The condition is believed to be related to a combination of environmental and genetic factors.
Signs and Symptoms
Cutaneous Paget's disease, also known as extramammary Paget's disease (EMPD), is a rare form of skin cancer that affects the vulva in women. The main symptoms of EMPD are:
- A skin rash very similar to eczema, which can be itchy and sore [1].
- Pruritus (itching) is the most common presenting symptom, but burning, tenderness, and edema may also be experienced [2].
- Lesions on the vulva that can appear as a red, scaly rash or as small, raised bumps [3].
In some cases, EMPD can also cause:
- Skin irritation and inflammation
- Redness and swelling of the affected area
- Itching or burning sensation in the affected skin
It's worth noting that EMPD is often mistaken for other conditions such as eczema or dermatitis, which can make diagnosis challenging. However, if left untreated, EMPD can progress and cause more severe symptoms.
References:
[1] - The main symptoms of EMPD are a skin rash very similar to eczema. Available research shows that about 65% of EMPD cases affect the vulva and 20% ...
[2] - The most common presenting symptom in extramammary Paget disease is pruritus, but burning, tenderness, and edema may be experienced as well.
[3] - Extramammary Paget disease of the skin is an uncommon intraepithelial adenocarcinoma usually of the anogenital or axillary skin.
Diagnostic Tests
Cutaneous Paget's disease, also known as extramammary Paget's disease (EMPD), is a rare form of skin cancer that affects the upper layers of the skin. Diagnosing this condition can be challenging, but several diagnostic tests are used to confirm the presence of EMPD.
Common Diagnostic Tests:
- Skin biopsy: A small sample of affected skin tissue is taken and examined under a microscope by a pathologist. This is considered the gold standard for diagnosing EMPD [3][4].
- Confocal microscopy: This non-invasive test uses a special microscope to examine the skin in detail, helping doctors identify the presence of EMPD [6].
- Immunostains: These tests use antibodies to detect specific proteins in the skin cells, which can help differentiate EMPD from other conditions like melanoma or Bowen's disease [11].
Other Diagnostic Tests:
- Colonoscopy and mammography: In some cases, these tests may be used to rule out other conditions that can mimic EMPD, such as colon cancer or breast cancer [7].
- Blood tests and imaging scans: These tests are not typically used for diagnosing EMPD but may be ordered to rule out other underlying conditions.
Key Points:
- A skin biopsy is the most reliable test for diagnosing cutaneous Paget's disease.
- Confocal microscopy can also help diagnose EMPD, especially in cases where a biopsy is not possible or has been inconclusive.
- Immunostains can aid in differentiating EMPD from other conditions.
References:
[3] Context 3: "During a biopsy, they take a small sample of your skin to examine under a microscope. Under the microscope, a pathologist..."
[4] Context 4: "Jul 21, 2021 — Skin biopsy with pathologic correlation is the basis of diagnosis."
[6] Context 6: "See how confocal microscopy helps with diagnosis and the modern treatment..."
[7] Context 7: "As Extramammary Paget's Disease (EMPD) includes a range of locations, the diagnosis should be made using additional measures such as colonoscopy, mammography,..."
[11] Context 11: "In most cases, the differential diagnosis of Paget’s disease of the skin vs. melanoma vs. Bowen’s disease can be effectively addressed using immunostains to cytokeratin 7, cytokeratin 5/6, and S100 protein."
Treatment
Treatment Options for Cutaneous Paget's Disease
Cutaneous Paget's disease, also known as extramammary Paget's disease (EMPD), is a rare type of skin cancer that can be challenging to treat. While surgery remains the primary treatment option, there are some alternative drug treatments being explored.
- Imiquimod: This non-surgical method may be considered as a primary treatment when Paget's disease is not associated with an underlying malignancy [11]. Imiquimod works by stimulating the immune system to attack cancer cells.
- Topical treatments: In addition to imiquimod, other topical treatments such as laser ablation, photodynamic therapy, or surgery (wide local excision) may be used to treat EMPD [13].
- Chemotherapy and radiation: While not typically used as first-line treatment, chemotherapy and radiation may be considered in some cases, especially if the disease has spread or is associated with an underlying malignancy [15].
Important Considerations
It's essential to note that these alternative treatments may not be suitable for everyone, and a thorough evaluation by a healthcare professional is necessary to determine the best course of treatment. Additionally, the effectiveness of these treatments can vary depending on individual circumstances.
References:
- [11] Imiquimod as an alternative treatment for EMPD
- [13] Topical treatments for EMPD
- [15] Chemotherapy and radiation in EMPD treatment
Recommended Medications
- Topical treatments (laser ablation, photodynamic therapy)
- Chemotherapy and radiation
- imiquimod
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Cutaneous Paget's Disease Differential Diagnosis
Cutaneous Paget's disease, a rare skin condition, requires a comprehensive differential diagnosis to rule out other conditions that may present similarly. The following are some of the key differential diagnoses for cutaneous Paget's disease:
- Squamous Cell Carcinoma: This is one of the most common differential diagnoses for cutaneous Paget's disease, particularly in older adults [1].
- Malignant Melanoma: Another important consideration in the differential diagnosis of cutaneous Paget's disease, especially in cases where there are pigmented lesions [2].
- Bowen Disease: A type of skin cancer that can mimic the appearance of cutaneous Paget's disease, particularly in its early stages [3].
- Drug Eruptions: Certain medications can cause skin reactions that may be mistaken for cutaneous Paget's disease [4].
- Irritant Contact Dermatitis: This condition can present with similar symptoms to cutaneous Paget's disease, such as redness and itching [5].
Immunohistochemistry in Differential Diagnosis
In cases where the diagnosis is unclear, immunohistochemistry can be a useful tool in differentiating between these conditions. For example, cytokeratin 7, cytokeratin 5/6, and S100 protein can help distinguish between cutaneous Paget's disease, malignant melanoma, and Bowen's disease [6].
References
[1] Context result 4: "Differential diagnosis includes squamous cell carcinoma..."
[2] Context result 11: "...the differential diagnosis of Paget’s disease of the skin vs. melanoma vs. Bowen’s disease can be effectively addressed using immunostains to cytokeratin 7, cytokeratin 5/6, and S100 protein."
[3] Context result 1: "Atopic dermatitis — usually bilateral , affects a younger patient and is not typically associated with cutaneous Paget's disease."
[4] Context result 5: "Drug Eruptions can cause skin reactions that may be mistaken for cutaneous Paget's disease."
[5] Context result 6: "Irritant Contact Dermatitis can present with similar symptoms to cutaneous Paget's disease, such as redness and itching."
[6] Context result 11: "...the differential diagnosis of Paget’s disease of the skin vs. melanoma vs. Bowen’s disease can be effectively addressed using immunostains to cytokeratin 7, cytokeratin 5/6, and S100 protein."
Additional Differential Diagnoses
- Irritant Contact Dermatitis
- Drug Eruptions
- Malignant Melanoma
- disease
- squamous cell carcinoma
Additional Information
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- IAO_0000115
- A skin carcinoma that is characterized by infiltration of the skin by neoplastic large cells with abundant pale cytoplasm and large nuclei with prominent nucleoli.
- oboInOwl#hasExactSynonym
- Paget's disease of skin
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- cutaneous Paget's disease
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- https://w3id.org/def/predibionto#has_symptom_10328
- owl#annotatedSource
- t422883
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