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MAMMARY PAGET
DISEASE (MPD)
MAMMARY PAGET DISEASE (MPD)/
PD OF THE NIPPLE/ PD OF THE BREAST
MPD is an uncommon skin malignancy
characterized by a chro...
MAMMARY PAGET DISEASE (MPD)/
PD OF THE NIPPLE/ PD OF THE BREAST
A progressive, well marginated (well
circumscribed), chron...
A 55-year-old woman presented with one year history of unilateral
pruritic eczema-like rash confined to the areolar area o...
MPD
A similar condition that involves the skin of the anogenital
regions of female and male where there are an abundance
o...
INCAIDENCE OF MPD
INCIDENCE OF MPD
Uncommon, 1-4% of female breast carcinoma cases are
associated with PD of the nipple, the areola, and the...
ETIOLOGY OF MPD
ETIOLOGY OF MPD
Intraepidermal extension of malignant ductal
epithelial cells (Paget cells) through the
lactiferous ducts ...
ETIOLOGY OF MPD
PAGET
CELLS
Glandular
stem
cells
Epi-
dermal
Toker
cells
ETIOLOGY OF MPD
PAGET CELLS MAY BE DERIVED FROM:
1. GLANDULAR STEM CELLS:
Paget cell share similar immunohistochemical cha...
ETIOLOGY OF MPD
PAGET CELLS MAY BE DERIVED FROM:
2. EPIDERMAL TOKER CELLS (clear cells of the nipple epithelium):
Due to t...
C/P OF MPD
Paget's disease. Images macroscopic woman than 50 years that had
significant areolar eczema in the left region, which had ...
A 15yr old girl presented to the clinic with complaints of itching in the
nipple area of left breast for the past 2 years.
Showing an ulcerated erythematous plaque covering whole of the left
breast, the sub mammary area and adjacent part of abdo...
Erythema, erosions and bloody discharge of 20 years old female
C/P OF MPD
SYMPTOMS 1. Nipple rash
2. Redness
3. Itching – Burning - pain
4. Oozing or Bloody Nipple discharge
5. Nipple r...
C/P OF MPD
PHYSICAL EXAMINATION AT EARLY STAGE:
The lesion usually begins at the nipple and
gradually spread to the areola...
C/P OF MPD
PHYSICAL EXAMINATION AT A LATER STAGE:
Skin of the breast is erythematous and moist or crusted  sharply
margin...
C/P OF MPD
The regional LN should be examined;
they are rarely enlarged when a mass
cannot be felt, but are enlarged in mo...
C/P OF MPD
The changes may occasionally involve not only the skin of
the breast but also spread on to the chest wall.
Poor...
C/P OF MPD
Pigmented mammary PD and pigmented
extramammary PD are rare clinical entities in
both males and females.
These ...
DDx OF MPD
DIFFERENCE BETWEEN MPD & ECZEMA
OF THE NIPPLE
DDx OF MPD
1) Eczema of the nipple
2) Bowen’s disease (very uncommon on the nipple)
3) Superficial BCC (very uncommon on th...
INVESTIGATIONS OF MPD
INVESTIGATIONS OF MPD
• Mammography
• MRI
• Ultrasound
I. Imaging: “3”
MPD: an area with microcalcifications in the lower inner left quadrant with
extension towards the nipple which shows retra...
Paget's disease. Cranio-caudal mammograms of left breast of a patient
diagnosed with Paget. Area of 10 cm that affects the...
MPD in right breast, unifocal, isolated in nipple.
BREAST PAGET DISEASE. 75 years old woman presenting a lesion in the
left nipple since 2 weeks ago. ULTRASOUND left retroar...
INVESTIGATIONS OF MPD
1. MAMMOGRAPHY:
Radiographic changes seen in MPD include the following: “4”
1. Subareolar microcalci...
INVESTIGATIONS OF MPD
2. MRI of the involved breast can detect otherwise occult PD in
the setting of negative mammography ...
INVESTIGATIONS OF MPD
3. ULTRASOUND to establish whether or not there is deeper
pathology in the underlying breast, as thi...
INVESTIGATIONS OF MPD
• Tzanck smear
• Biopsy of the tumor
• Sentinel lymph node biopsy
II. Tissue Analysis: “3”
INVESTIGATIONS OF MPD
TISSUE ANALYSIS: “3”
1. TZANCK SMEAR: The presence of large cells with a high nuclear-to-
cytoplasmi...
Histopathological section showing large atypical round to oval cells
(arrow) infiltrating the lower part of epidermis havi...
(A) The epidermis of the nipple infiltrated by large Paget’s cells with pale
abundant cytoplasm
(B) Single groups of Paget...
HISTOPATHOLOGY OF MPD
THE EPIDERMIS:
Hyperkeratosis or parakeratosis.
Acanthosis, with papillomatosis.
Enlargement of the ...
HISTOPATHOLOGY OF MPD
Their ultrastructural features of Paget’s cells are those of
glandular epithelial cells it’s cytopla...
HISTOPATHOLOGY OF MPD
PAGET CELLS CLASSICALLY HAVE THE
FOLLOWING HISTOLOGICAL FEATURES:
1. Large rounded or ovoid atypical...
HISTOPATHOLOGY OF MPD
In the ulcerated lesions of MPD, the epidermis is totally
replaced by Paget cells.
A large biopsy or...
HISTOPATHOLOGY OF MPD
THE DERMIS  chronic inflammatory reaction in the upper
dermis contains a dense infiltrate of lymphoc...
HISTOPATHOLOGY OF MPD
Several histologic variants of PD are as
follows: “5”
1. Adenocarcinomalike cell type
2. Spindle cel...
The clear appearance of cytoplasm in Paget’s disease is due to their
abundant content of neutral and acidic mucopolysaccha...
The malignant cells are usually immunoreactive for Carcinoembryonic
antigen (CEA)
Immunohistochemistry showing positivity with epithelial membrane
antigen (EMA)
SPECIAL STAINS OF PAGET’S CELLS
1. PAS STAIN:
Paget’s cells shows PAS positive diastase-resistant granules,
indicating the...
IMMUNOHISTOCHEMISTRY
POSITIVE MARKERS OF PAGET'S CELLS ARE “5”:
1. CEA (Carcinoembryonic Antigen)
2. EMA (Epithelial Membr...
IMMUNOHISTOCHEMISTRY
NEGATIVE MARKERS OF PAGET'S CELLS, which serve as
differentiating features from malignant melanoma “5...
STAGING OF MPD
STAGING OF MPD
Mammary Paget disease has been classified into 4 clinical stages
Stage 0
Lesion confined to the epidermis, ...
Rx OF MPD
Rx OF MPD
I. Mastectomy (radical or modified)
and LN clearance
II. Photodynamic therapy (PDT)
III. Conservative management
Rx OF MPD
I. Mastectomy (radical or modified) and LN
clearance in cases with palpable mass and
underlying invasive breast ...
Rx OF MPD
II. Photodynamic therapy (PDT) using aminolevulinic acid (5-
ALA) for low-risk malignant cells
Rx OF MPD
III. Conservative management: In patients with no evidence of
an underlying breast carcinoma. Combination of “3 ...
EXTRAMAMMARY
PAGET DISEASE
EXTRAMAMMARY PAGET DISEASE
EMPD is an uncommon tumor characterized
by a chronic eczema-like lesion of the skin
around the ...
Grossly inflamed erythema on the vulva extending to the perineum.
There were whitish cheesy lesions on the wall of the vag...
This man presented with a pruritic, erythematous, scaly, eroded
dermatitis-like rash involved the perianal area of two mon...
Extramammary Paget disease, gross. The patient’s lesion on the glans
penis.
Clinical presentation of the well-demarked pink erythematous patch in
the left axilla
EMPD HAS BEEN CLASSIFIED INTO
SEVERAL SUBTYPES:
primary cutaneous extramammary Paget disease arises from apocrine
glands w...
Rx OF EMPD
Wide local excision, vulvectomy, or Mohs micrographic
surgery is the standard treatment.
Recurrence is common (...
Rx OF EMPD
Non-surgical treatments for recurrent disease may include:
1. Radiotherapy
2. Laser ablation
3. Photodynamic th...
REFERENCES
Rook 8th edition.
Bolongia 3rd edition.
Google images.
http://www.dermnetnz.org
www.facebook.com/groups/dermato...
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)
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Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)

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MPD is an uncommon skin malignancy characterized by a chronic eczema-like lesion of the nipple and adjacent areolar skin. Paget cells originate in either invasive intraduct carcinoma or ductal carcinoma in-situ (DCIS) of in the deeper breast tissue.
Extramammary Paget disease (EMPD). A similar condition that involves the skin of the anogenital regions of female and male where there are an abundance of apocrine glands.

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Mammary Paget Disease (MPD) & Extramammary Paget disease (EMPD)

  1. 1. MAMMARY PAGET DISEASE (MPD)
  2. 2. MAMMARY PAGET DISEASE (MPD)/ PD OF THE NIPPLE/ PD OF THE BREAST MPD is an uncommon skin malignancy characterized by a chronic eczema-like lesion of the nipple and adjacent areolar skin.
  3. 3. MAMMARY PAGET DISEASE (MPD)/ PD OF THE NIPPLE/ PD OF THE BREAST A progressive, well marginated (well circumscribed), chronic eczematous change due to invasion of the epidermis by malignant Paget cells. Paget cells originate in either invasive intraduct carcinoma or ductal carcinoma in-situ (DCIS) of in the deeper breast tissue.
  4. 4. A 55-year-old woman presented with one year history of unilateral pruritic eczema-like rash confined to the areolar area of one breast.
  5. 5. MPD A similar condition that involves the skin of the anogenital regions of female and male where there are an abundance of apocrine glands is known as extramammary Paget disease (EMPD).
  6. 6. INCAIDENCE OF MPD
  7. 7. INCIDENCE OF MPD Uncommon, 1-4% of female breast carcinoma cases are associated with PD of the nipple, the areola, and the surrounding skin. Almost exclusively in ♀ involvement of the male breast is rarely reported. Is most frequent in the 5th and 6th decade mean age at diagnosis of 55 years. Nearly 100% of mammary PD cases are associated with an underlying carcinoma, either invasive intraduct carcinoma (90%) or ductal carcinoma in-situ (10%).
  8. 8. ETIOLOGY OF MPD
  9. 9. ETIOLOGY OF MPD Intraepidermal extension of malignant ductal epithelial cells (Paget cells) through the lactiferous ducts and ductules into the epidermis (EPIDERMOTROPISM)  infiltrate and proliferate in the epidermis.
  10. 10. ETIOLOGY OF MPD PAGET CELLS Glandular stem cells Epi- dermal Toker cells
  11. 11. ETIOLOGY OF MPD PAGET CELLS MAY BE DERIVED FROM: 1. GLANDULAR STEM CELLS: Paget cell share similar immunohistochemical characteristics with eccrine and apocrine sweat gland epithelium. Paget cells are periodic acid-Schiff (PAS) positive and diastase resistant; and they are Alcian blue positive or…..
  12. 12. ETIOLOGY OF MPD PAGET CELLS MAY BE DERIVED FROM: 2. EPIDERMAL TOKER CELLS (clear cells of the nipple epithelium): Due to the similarity of the immunophenotypes. Toker cells have been found in about 10% of normal nipples and rarely in supernumerary nipples and apocrine bearing areas. Like Paget cells of both mammary and extramammary sites, Toker cells contain prominent clear (vacuolated) cytoplasm, and they are considered benign counterparts of Paget cells & sometimes proliferate, resulting in a condition known as clear cell papulosis.
  13. 13. C/P OF MPD
  14. 14. Paget's disease. Images macroscopic woman than 50 years that had significant areolar eczema in the left region, which had been increasing in the last six months, accompanied by itching. See as there accompanying nipple retraction.
  15. 15. A 15yr old girl presented to the clinic with complaints of itching in the nipple area of left breast for the past 2 years.
  16. 16. Showing an ulcerated erythematous plaque covering whole of the left breast, the sub mammary area and adjacent part of abdominal wall, covered with purulent to hemorrhagic crusts
  17. 17. Erythema, erosions and bloody discharge of 20 years old female
  18. 18. C/P OF MPD SYMPTOMS 1. Nipple rash 2. Redness 3. Itching – Burning - pain 4. Oozing or Bloody Nipple discharge 5. Nipple retraction 6. Scaling- Crusting of skin 7. Swelling 8. Ulceration
  19. 19. C/P OF MPD PHYSICAL EXAMINATION AT EARLY STAGE: The lesion usually begins at the nipple and gradually spread to the areola. The early changes may be minimal, with a unilateral small, crusted and intermittently moist area on the nipple giving a brownish stain on clothing, or producing itching, pricking or burning sensations. Less often, there is a serous or blood-stained discharge from the nipple, or a lump may be noticed in the breast. The surface changes persist and gradually spread to produce an eczematous appearance.
  20. 20. C/P OF MPD PHYSICAL EXAMINATION AT A LATER STAGE: Skin of the breast is erythematous and moist or crusted  sharply marginated, indurated & thickened plaques and may spare a segment of the areola. The edge is slightly raised and irregular in outline. If the crusts are removed, a red, glazed, moist or vegetating surface is revealed. Itching may be a prominent symptom and excoriations may be found in the established lesion. The nipple itself may be retracted, and a subjacent palpable mass or a lump deeper in the breast may be felt. Nipple invagination is sometimes seen.
  21. 21. C/P OF MPD The regional LN should be examined; they are rarely enlarged when a mass cannot be felt, but are enlarged in more than half the cases with a detectable tumor.
  22. 22. C/P OF MPD The changes may occasionally involve not only the skin of the breast but also spread on to the chest wall. Poor prognosis is associated with invasive disease and the presence of a palpable mass.
  23. 23. C/P OF MPD Pigmented mammary PD and pigmented extramammary PD are rare clinical entities in both males and females. These diseases may mimic malignant melanoma both clinically and histopathologically. They may also mimic melanoma on dermoscopic examination. In pigmented lesions of PD,  numbers of benign melanocytes are present, which may interfere with the correct diagnosis of PD.
  24. 24. DDx OF MPD
  25. 25. DIFFERENCE BETWEEN MPD & ECZEMA OF THE NIPPLE
  26. 26. DDx OF MPD 1) Eczema of the nipple 2) Bowen’s disease (very uncommon on the nipple) 3) Superficial BCC (very uncommon on the nipple) 4) Psoriasis 5) Amyloidosis 6) Erosive adenomatosis of the nipple. 7) Nipple duct adenoma 8) Drug Eruptions 9) Malignant melanoma
  27. 27. INVESTIGATIONS OF MPD
  28. 28. INVESTIGATIONS OF MPD • Mammography • MRI • Ultrasound I. Imaging: “3”
  29. 29. MPD: an area with microcalcifications in the lower inner left quadrant with extension towards the nipple which shows retraction and some calcifications within, there is also minimal extension towards the lower outer left quadrant.
  30. 30. Paget's disease. Cranio-caudal mammograms of left breast of a patient diagnosed with Paget. Area of 10 cm that affects the external cuadrants which identifies multiple pleomorphic microcalcifications, very suspicious of malignancy, which at higher magnification (right) continue the road of the nipple lactiferous duct (arrow)
  31. 31. MPD in right breast, unifocal, isolated in nipple.
  32. 32. BREAST PAGET DISEASE. 75 years old woman presenting a lesion in the left nipple since 2 weeks ago. ULTRASOUND left retroareolar area: where microcalcifications can be identied.
  33. 33. INVESTIGATIONS OF MPD 1. MAMMOGRAPHY: Radiographic changes seen in MPD include the following: “4” 1. Subareolar microcalcifications (helpful in evaluating and locating clinically occult, nonpalpable underlying breast carcinoma) 2. Architectural distortion 3. Thickening of the nipple and the areola (reflecting edema) 4. Nipple changes (in a minority of patients) Negative preoperative mammography findings did not reliably exclude an underlying carcinoma.
  34. 34. INVESTIGATIONS OF MPD 2. MRI of the involved breast can detect otherwise occult PD in the setting of negative mammography findings.
  35. 35. INVESTIGATIONS OF MPD 3. ULTRASOUND to establish whether or not there is deeper pathology in the underlying breast, as this will help determine the extent of surgery required.
  36. 36. INVESTIGATIONS OF MPD • Tzanck smear • Biopsy of the tumor • Sentinel lymph node biopsy II. Tissue Analysis: “3”
  37. 37. INVESTIGATIONS OF MPD TISSUE ANALYSIS: “3” 1. TZANCK SMEAR: The presence of large cells with a high nuclear-to- cytoplasmic ratio, occasional acinar formation, and intracytoplasmic vacuoles is diagnostic for malignant Paget cells. 2. BIOPSY OF THE TUMOR: Punch, wedge, or excisional biopsy. 3. SENTINEL LYMPH NODE BIOPSY: is performed in cases with an invasive component.
  38. 38. Histopathological section showing large atypical round to oval cells (arrow) infiltrating the lower part of epidermis having a pale cytoplasm with prominent hyperchromatic nuclei
  39. 39. (A) The epidermis of the nipple infiltrated by large Paget’s cells with pale abundant cytoplasm (B) Single groups of Paget’s cells with vesicular nuclei and prominent nucleoli
  40. 40. HISTOPATHOLOGY OF MPD THE EPIDERMIS: Hyperkeratosis or parakeratosis. Acanthosis, with papillomatosis. Enlargement of the rete ridges. Characteristic Paget’s cells singly or in clusters (nests) are dispersed between the prickle cells. They vary in number, and when profuse the Malpighian layer may be disrupted and the surface covered by a crust. In the later stages, the epidermis may be atrophic or eroded.
  41. 41. HISTOPATHOLOGY OF MPD Their ultrastructural features of Paget’s cells are those of glandular epithelial cells it’s cytoplasm is PAS-positive & packed with numerous rounded, membrane-bound mucin granules. Infiltration occurs by variable numbers of signet-ring forms tumor cells that are present in all layers of the epidermis. Mitotic figures are occasionally identified.
  42. 42. HISTOPATHOLOGY OF MPD PAGET CELLS CLASSICALLY HAVE THE FOLLOWING HISTOLOGICAL FEATURES: 1. Large rounded or ovoid atypical cells 2. Abundant pale-staining cytoplasm 3. Mucin-positive 4. Enlarged, scattered mitochondria 5. Large rounded or ovoid vesicular-to- hyperchromatic nuclei with prominent nucleoli. 6. Scanty nuclear chromatin 7. They are devoid of intercellular bridges
  43. 43. HISTOPATHOLOGY OF MPD In the ulcerated lesions of MPD, the epidermis is totally replaced by Paget cells. A large biopsy or excision may demonstrate the presence of epidermal Paget cells and an underlying infiltrating or intraductal carcinoma of the breast. The Paget’s cells may also be seen in appendage ducts.
  44. 44. HISTOPATHOLOGY OF MPD THE DERMIS  chronic inflammatory reaction in the upper dermis contains a dense infiltrate of lymphocytes, histiocytes, plasma cells, and occasionally eosinophils. AN UNDERLYING BREAST CARCINOMA  may be seen on large biopsy. The cells may accumulate within and distend the ducts and spread in both directions. A number of ducts are usually involved. At a later stage, the carcinoma becomes invasive and behaves like classic breast carcinoma.
  45. 45. HISTOPATHOLOGY OF MPD Several histologic variants of PD are as follows: “5” 1. Adenocarcinomalike cell type 2. Spindle cell type 3. Anaplastic cell type 4. Acantholytic cell type 5. Pigmented cell type
  46. 46. The clear appearance of cytoplasm in Paget’s disease is due to their abundant content of neutral and acidic mucopolysaccharides which can be demonstrated by PAS stain
  47. 47. The malignant cells are usually immunoreactive for Carcinoembryonic antigen (CEA)
  48. 48. Immunohistochemistry showing positivity with epithelial membrane antigen (EMA)
  49. 49. SPECIAL STAINS OF PAGET’S CELLS 1. PAS STAIN: Paget’s cells shows PAS positive diastase-resistant granules, indicating the presence of neutral mucopolysaccharides and supports the glandular origin of the cells. 2. Alcian blue: Positive.
  50. 50. IMMUNOHISTOCHEMISTRY POSITIVE MARKERS OF PAGET'S CELLS ARE “5”: 1. CEA (Carcinoembryonic Antigen) 2. EMA (Epithelial Membrane Antigen) 3. CK7 Low molecular weight cytokeratins proposed as a specific and nearly 100% sensitive marker for MPD. 4. CAM-5.2 (Cellular adhesion molecule) Glandular epithelial cell markers. 5. erbB-2 (>90%)
  51. 51. IMMUNOHISTOCHEMISTRY NEGATIVE MARKERS OF PAGET'S CELLS, which serve as differentiating features from malignant melanoma “5”: 1. Anti-s-100 protein 2. Melan A (MART-1) 3. HMB-45 4. Tyrosinase 5. DOPA
  52. 52. STAGING OF MPD
  53. 53. STAGING OF MPD Mammary Paget disease has been classified into 4 clinical stages Stage 0 Lesion confined to the epidermis, without underlying in situ ductal carcinoma of the breast Stage 1 Associated with in situ ductal carcinoma just beneath the nipple Stage 2 Associated with extensive in situ ductal carcinoma Stage 3 Associated with invasive ductal carcinoma
  54. 54. Rx OF MPD
  55. 55. Rx OF MPD I. Mastectomy (radical or modified) and LN clearance II. Photodynamic therapy (PDT) III. Conservative management
  56. 56. Rx OF MPD I. Mastectomy (radical or modified) and LN clearance in cases with palpable mass and underlying invasive breast carcinoma.
  57. 57. Rx OF MPD II. Photodynamic therapy (PDT) using aminolevulinic acid (5- ALA) for low-risk malignant cells
  58. 58. Rx OF MPD III. Conservative management: In patients with no evidence of an underlying breast carcinoma. Combination of “3 measures”; 1. Local excision of the nipple, 2. Wedge resection of the underlying breast tissue, 3. Radiation therapy: according to the presence or absence of an invasive component.
  59. 59. EXTRAMAMMARY PAGET DISEASE
  60. 60. EXTRAMAMMARY PAGET DISEASE EMPD is an uncommon tumor characterized by a chronic eczema-like lesion of the skin around the anogenital regions of males and females. In women the most common area involved is the vulva. The clinical and the histopathological findings are very similar to the more common type of MPD.
  61. 61. Grossly inflamed erythema on the vulva extending to the perineum. There were whitish cheesy lesions on the wall of the vagina. Superficial erosions were noted on the left posterior area. Her regional nodes were not enlarged.
  62. 62. This man presented with a pruritic, erythematous, scaly, eroded dermatitis-like rash involved the perianal area of two months duration. Three months before rash appearance the patient had suffered constipation and many episodes of rectal bleeding. Sigmoidoscopy disclosed rectal carcinoma whereas perianal skin biopsy was consistent with extramammary Paget's disease.
  63. 63. Extramammary Paget disease, gross. The patient’s lesion on the glans penis.
  64. 64. Clinical presentation of the well-demarked pink erythematous patch in the left axilla
  65. 65. EMPD HAS BEEN CLASSIFIED INTO SEVERAL SUBTYPES: primary cutaneous extramammary Paget disease arises from apocrine glands within the epidermis (in situ) or underlying skin appendages primary cutaneous extramammary Paget disease (15-25%) is associated with invasive Paget disease or adenocarcinoma in situ. extramammary Paget disease originates from underlying anal or rectal adenocarcinoma extramammary Paget disease originates from bladder adenocarcinoma Type1a Type 1b Type 2 Type 3
  66. 66. Rx OF EMPD Wide local excision, vulvectomy, or Mohs micrographic surgery is the standard treatment. Recurrence is common (30-50%), so patients should be re- examined every 3 months after surgery for the next 2 years, after which annual follow-ups are recommended. Recurrence generally leads to further surgery.
  67. 67. Rx OF EMPD Non-surgical treatments for recurrent disease may include: 1. Radiotherapy 2. Laser ablation 3. Photodynamic therapy 4. 5-fluorouracil cream 5. Imiquimod cream
  68. 68. REFERENCES Rook 8th edition. Bolongia 3rd edition. Google images. http://www.dermnetnz.org www.facebook.com/groups/dermatologycourseonline/

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