Vulvar Paget's Disease

Summary about Disease


Vulvar Paget's disease is a rare type of intraepithelial (within the skin) adenocarcinoma, meaning it's a slow-growing cancer arising from glandular cells in the skin of the vulva. It often presents as a chronic, itchy, and sometimes painful skin condition. While it can be localized to the vulva, it sometimes indicates an underlying invasive vulvar cancer or, rarely, another internal malignancy.

Symptoms


Common symptoms include:

Persistent itching (pruritus)

Red, scaly, eczematous-like patches on the vulva

Burning sensation

Pain or soreness

Bleeding

Lump or thickening of the skin

Less commonly, ulceration or crusting

Causes


The exact cause is not fully understood. However, there are two primary theories:

Primary Vulvar Paget's Disease: Arises independently within the skin of the vulva.

Secondary Vulvar Paget's Disease: Originates from an underlying adenocarcinoma of the vulva, or, less commonly, from another internal cancer (e.g., colon, rectum, bladder) and spreads to the skin.

Medicine Used


Treatment often involves surgical excision of the affected skin. If surgical removal is not possible, or for extensive disease, other treatments may be considered, including:

Topical imiquimod: An immune response modifier cream.

Laser ablation: To destroy abnormal cells.

Radiation therapy: To target cancerous cells.

Chemotherapy: In cases of invasive disease or associated underlying cancers.

Is Communicable


No. Vulvar Paget's disease is not contagious or communicable. It is not spread through contact.

Precautions


There are no specific precautions to prevent vulvar Paget's disease, as the cause is not fully understood. However, if you experience persistent itching, redness, or other unusual changes on your vulva, it is important to see a doctor promptly for evaluation. Regular self-exams may aid in early detection.

How long does an outbreak last?


Vulvar Paget's disease is a chronic condition that, without treatment, can persist indefinitely. Symptoms will likely continue unless medical intervention is initiated.

How is it diagnosed?


Diagnosis typically involves:

Physical Examination: Visual inspection of the vulva.

Biopsy: A small sample of the affected skin is removed and examined under a microscope to confirm the diagnosis and rule out other conditions. Immunohistochemical staining is performed on the biopsy specimen to help differentiate Paget's cells from other cells and to determine the origin of the disease.

Colposcopy: Examination of the vulva with a magnifying instrument.

Imaging Studies: CT scans, MRI, or PET scans may be used to look for underlying invasive cancer or other internal malignancies, particularly if secondary Paget's disease is suspected.

Timeline of Symptoms


The onset of symptoms is usually gradual.

Initial Stage: Mild itching and redness may be the first signs.

Progression: The affected area becomes more scaly, irritated, and painful. Lesions can spread over time.

Advanced Stage: Ulceration, bleeding, and lump formation may occur in advanced cases, potentially indicating invasive cancer.

Important Considerations


Underlying Cancer: Ruling out an underlying invasive vulvar cancer or other internal malignancy is crucial in the management of vulvar Paget's disease.

Recurrence: Recurrence after treatment is possible, so regular follow-up examinations are important.

Psychological Impact: Chronic vulvar conditions can have a significant impact on a woman's quality of life, including sexual function and self-esteem. Psychological support and counseling may be beneficial.