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Pagets disease of the nipple

  • Paget’s disease of the breast is a rare form of breast cancer that almost exclusively occurs in women
  • May occur at any age from 24 to 84, although it is most common around the menopause.
  • Most nipple rashes are simply a minor skin infection or a reaction to some irritant. 
  • A nipple rash that does not get better with topical steroid treatments requires further evaluation with advanced imaging and biopsy to rule out serious conditions like Malignant melanoma or mammary Paget disease, etc.  

Symptoms 

pagetseczema
Erythematous, scaly, crusty, and thickened plaque-like lesionerythematous patch is sharply demarcated and deeply infiltrated, unlike eczematous dermatitis

Unilateral, persistentItching or burning sensation.

Discharge and/or bleeding from the nipple.
Ulceration.
Destruction of the nipple.
Inversion of the nipple.
Sometimes palpable breast lump
Bilateral
Irritation.
Acute inflammation, weeping vesicles or blisters.
Chronic problems with dry, thickened areas.
Often history of atopic eczema with personal or family history of hay fever or asthma.
Allergic response to contact allergen.
May present during lactation.Nipple preserved.
No breast lump.
ManagementManagement
Punch, wedge or excisional biopsy.
Urgent referral to breast unit.
Local excision or mastectomy, chemotherapy and/or radiotherapy
Avoid aggravating cause.
Moisturisers.
Topical corticosteroid cream or ointment.
Oral antihistamine if itching is troublesome.
Patch testing if allergen suspected.Mammogram, cytology or biopsy if necessary

Prognosis

  • If PDB presents initially with a palpable mass, it is usually associated with more advanced disease than cases without a palpable mass. 
  • When breast mass is not palpable
    • 92% of patients survive five years after excision
    • 82% survive ten years. 
  • When breast mass is palpable
    • 38% survive five years
    • 22% survive ten years
    • Prognosis is worse when there is lymphadenopathy.

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