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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 1  |  Issue : 2  |  Page : 73-75

Chronic macerated plaques over the genitalia in an elderly female: A diagnostic challenge


1 Department of Dermatology, Father Muller Medical College, Mangalore, Karnataka, India
2 Department of Pathology, Father Muller Medical College, Mangalore, Karnataka, India

Date of Web Publication28-Jul-2017

Correspondence Address:
S Suvarna
Department of Dermatology, Father Muller Medical College, Mangalore - 575 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_7_17

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  Abstract 


Extramammary Paget's disease (EMPD) is a rare dermatosis occurring in sites rich in apocrine glands, such as the vulva, anogenital region, and axilla. It can be subdivided into primary and secondary disease. An 84-year-old female presented with itchy, whitish, oozy raised lesions over the genitalia and groin since 2 years, associated with burning sensation. Examination revealed erythema, maceration and white oozy plaques over bilateral groin folds, perineum, and labia majora. She was treated as candidal intertrigo with secondary infection, with antibiotics and antifungals with minimal improvement. Routine blood investigations were within normal limits. Skin biopsy done from the vulva showed Paget cells in the lower two-thirds of the epidermis. She was diagnosed to have EMPD and referred to the oncosurgeon for further management. We present this case due to the rarity of its occurrence and to emphasize the high index of clinical suspicion required for chronic, macerated, erythematous plaques over the perineum, which do not respond to conventional treatment, in an elderly patient.

Keywords: Extramammary, Paget's, vulva


How to cite this article:
Suvarna S, Martis J, Nandakishore B, Kamath GH, Kini RG. Chronic macerated plaques over the genitalia in an elderly female: A diagnostic challenge. Clin Dermatol Rev 2017;1:73-5

How to cite this URL:
Suvarna S, Martis J, Nandakishore B, Kamath GH, Kini RG. Chronic macerated plaques over the genitalia in an elderly female: A diagnostic challenge. Clin Dermatol Rev [serial online] 2017 [cited 2023 Jan 29];1:73-5. Available from: https://www.cdriadvlkn.org/text.asp?2017/1/2/73/211785




  Introduction Top


Extramammary Paget's disease (EMPD) is a rare dermatosis occurring in sites rich in apocrine glands, such as the vulva, anogenital region, and axilla. It commonly affects women in the postmenopausal age group. It can present as itchy, chronic, oozy, erythematous lesions over the perineum, which are usually nonresponsive to conventional therapy. They are usually misdiagnosed as candidiasis or exudative dermatitis.


  Case Report Top


An 84-year-old female presented with itchy, whitish, oozy raised lesions over the genitalia since 2 years, which were associated with burning sensation. The lesions progressed to involve to groin and medial aspect of both the thighs. She also gave a history of burning micturition. There was no history of any other systemic comorbidities. She had been previously treated for the above complaints as candidal intertrigo with secondary infection, with systemic antibiotics, along with oral and topical antifungals, with minimal improvement.

Local examination revealed erythema, maceration and white oozy plaques over bilateral groin folds, perineum and labia majora [Figure 1]. Per speculum examination showed an unremarkable vagina and cervix. There was no regional lymphadenopathy. General examination and systemic examination revealed no abnormalities. Complete hemogram, hepatic and renal function tests, blood sugar levels, and urine analysis were within normal limits.
Figure 1: Erythematous, macerated, oozy plaques over the perineum

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Skin biopsy done from the vulva showed atypical, large round cells with pale eosinophilic cytoplasm containing mucin and large pleomorphic nucleus exhibiting prominent nucleoli (Paget cells), present in the lower two-thirds of the epidermis, along with hyperkeratosis, acanthosis, and focal hypergranulosis [Figure 2] and [Figure 3]. The presence of mucin was detected with special stains, namely, periodic acid–Schiff (PAS), PAS-D, and mucicarmine stain [Figure 4] and [Figure 5]. Ultrasound and computed tomography scans of the abdomen and pelvis did not show evidence of any underlying malignancy. Based on the histopathological findings and lack of underlying malignancy, she was diagnosed to have primary EMPD and referred to oncosurgery for further management.
Figure 2: Atypical cells in the lower two-thirds of the epidermis (H and E, ×10)

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Figure 3: Large round cells with pale eosinophilic cytoplasm and large pleomorphic nucleus with prominent nucleoli (H and E, ×40)

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Figure 4: The presence of mucin in the atypical cells (Mucicarmine, ×40)

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Figure 5: The presence of mucin (×40, PAS-D-positive)

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  Discussion Top


EMPD is a rare dermatosis occurring in sites rich in apocrine glands, such as the vulva, anogenital region, and axilla. In 1937, it was hypothesized to arise from the apocrine sweat glands.[1] It is distinct from Paget's disease of the nipple, which is always associated with underlying breast neoplasia. The most common site is the vulva. It commonly affects women in the postmenopausal age group.[2]

It can be subdivided into primary and secondary disease. Primary/cutaneous EMPD is an intraepithelial adenocarcinoma arising in the epidermis or the epithelia of the local skin appendages. Secondary EMPD refers to epidermal involvement from a noncutaneous internal neoplasm, either by direct extension or metastasis. The most common tumors associated are anorectal adenocarcinoma and urothelial carcinoma of the bladder or urethra.[3],[4] It can present as itchy, chronic, oozy, erythematous lesions over the perineum, which are usually nonresponsive to conventional therapy. They are usually misdiagnosed as candidiasis or exudative dermatitis.[5]

Histopathology from the lesions shows the presence of Paget cells, which contain eosinophilic cytoplasm and prominent nucleoli. The cytoplasm is PAS positive and diastase resistant, due to the presence of neutral polysaccharides.[6]

The most common modality of treatment employed for EMPD is surgical resection followed by reconstructive surgery. However, the recurrence rate is high, ranging from 31% to 61%.[6]

We report this case due to the rarity of its occurrence and to emphasize the high index of suspicion required for a chronic, macerated, erythematous plaques over the perineum, which do not respond to conventional treatment, in an elderly patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Png JC, Tung KH, Wong YE, Mokal N, Rajagopalan R, Chong PY. Extramammary Paget's disease: A report of three cases and review of the literature. Ann Acad Med Singapore 1995;24:636-9.  Back to cited text no. 1
    
2.
Piura B, Rabinovich A, Dgani R. Extramammary Paget's disease of the vulva: Report of five cases and review of the literature. Eur J Gynaecol Oncol 1999;20:98-101.  Back to cited text no. 2
    
3.
Powell JL. Extramammary Paget's disease. J Am Coll Surg 2003;196:824.  Back to cited text no. 3
    
4.
Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol 2007;21:581-90.  Back to cited text no. 4
    
5.
Khoo JJ, Choon SE. Extramammary Paget's disease: A report of 2 cases and a review of the literature. Malays J Pathol 2003;25:73-8.  Back to cited text no. 5
    
6.
Tanaka VD, Sanches JA, Torezan L, Niwa AB, Festa Neto C. Mammary and extramammary Paget's disease: A study of 14 cases and the associated therapeutic difficulties. Clinics (Sao Paulo) 2009;64:599-606.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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