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Pagets disease of the breast or vulva

Category: Illness or disabilities

Type

Involuntary

Introduction and description

 

Sir James Paget gave his name to a number of diseases, the most well known being Paget’s disease of the bone [also on the site]. 

But he also gave his name to two more diseases - Paget’s disease of the breast and Paget’s disease of the vulva.  We have grouped them together because both conditions exhibit symptoms that are identical to eczema

In effect, they are eczema, but localised.  They are also serious, but then so is eczema

  • Paget's disease of the breast or Paget disease of the breast is a malignant condition that outwardly has the appearance of eczema, with skin changes involving the nipple of the breast. The condition accounts for 1 to 4.3% of all breast cancers and was first described by Sir James Paget in 1874.
  • Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to eczema itching, pain and bleeding. Calling it ‘eczema’ often leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows ‘Paget cells’.

Paget cells are large cells with “clear cytoplasm (clear halo) and eccentric, hyperchromic nuclei found throughout the epidermis”.

Symptoms

 

Both conditions may initially appear innocuous, limited to a small surface area, and not at the beginning either that inflamed or bleeding.  Then the area may enlarge and become more red and inflamed and there may be severe itching, pain, bleeding and pus filled spots appear, in other words eczema.

Eczema is itself a symptom and it is a symptom of viral infection, bacterial infection, fungal infection or toxic substance attack - such as heavy metals and chemicals.  All these are pathogens toxic to the body; depending how toxic to the body the substance is, the reaction may be mild or severe. 

The production of pus filled blisters is the mechanism by which the body rids itself of pathogens.  In other words, in all cases of eczema, a pathogen is to blame, either attacking the body from within, or attacking the skin from without.  Generally speaking one can find the pathogen by analysing the pus.

Eczema is thus a symptom of infection or attack by a pathogen.  And the pathogen may be extremely dangerous or if it is exterior it may be dangerous but capable of being removed relatively easily.

 

Causes

See Eczema

Treatment

The main method used by doctors is surgery, the removal of the affected cells.  But this may not work. 

If the pathogen is an external agent, for example, a toxic chemical or a cosmetic then the condition will return because the cause has not been eliminated.  If the pathogen is internal, it may be eliminated by surgery, if it is only to be found in that one area, but pathogens are generally not like that.  On the whole, there may be a manifestation in one spot, but the pathogen may have travelled over considerable areas of the body, particularly if some time has gone by.  For example

The treatment of choice [for Extramammary Paget disease] is wide excision with margins, which leads to sequelae, functional and aesthetic. Recurrence is common. This article reports the case of a 48-year-old female patient with history of vulvar itching for the past 2 years, who had been submitted to various treatments for benign pathologies. The patient was biopsied and was diagnosed with extensive EPD, being submitted to vulvectomy. This article aims to draw attention to the need for biopsy of pruritic vulvar lesions that do not respond to usual treatment.  PMID:  27832673

Ultimately any form of eczema should always be investigated for pathogens – external and internal.  For more help on this go to the section on Eczema.

The first line of treatment is thus to always FIND THE CAUSE - the pathogen responsible.

References and further reading

Rev Bras Ginecol Obstet. 2016 Nov 10. [Epub ahead of print]  Extramammary Paget Disease of the Vulva - Case Report.  Hillmann BR1, Pereira AA2, Sommacal LF3.

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