Clinical and dermoscopic characteristics of extramammary Paget disease: a study of 35 cases

Mun, Je‐Ho; Park, S.-M.; Kim, G.-W.; Song, Margaret; Kim, H.-S.; Ko, Hyun‐Chang; Kim, B.-S.; Kim, M.-B.

doi:10.1111/bjd.14300

Cited by 44 publications

(46 citation statements)

References 15 publications

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“…Statistically significant dermoscopic findings of EMPD included a greater number of milky‐red areas than in ED, FI, and BD, more common dermoscopic vascular structures than in ED or FI, and more ulcers than in FI. Invasive EMPD was statistically correlated with polymorphous vessels . Vascular patterns that distinguished EMPD from benign diseases included dotted vessels, observed in 51% (18/35) of cases, and glomerular vessels, noted in 20% (7/35) of cases.…”

Section: Resultsmentioning

confidence: 90%

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A review of non‐invasive imaging in extramammary Paget's disease

Bayan

Khanna

Rotemberg

et al. 2018

Acad Dermatol Venereol

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Extramammary Paget's Disease (EMPD) is a rare intraepithelial adenocarcinoma that classically manifests with pruritic, erythematous and scaling plaques. The clinical picture frequently mimics inflammatory or infectious conditions and is thus commonly misdiagnosed. The assessment of tumour margins is equally challenging as tumours have a propensity to spread beyond clinically visible boundaries. Appropriate non-invasive diagnostic tools can assist in the early detection, diagnosis and management of EMPD. This paper will review the literature on non-invasive imaging modalities used in EMPD. Articles from the PubMed database were selected based on relevance to the topic of this review. Articles that were not specific to EMPD and non-invasive imaging were excluded. Search strategy is further described in the methods section below. Eighteen articles were selected for this review: six PET/CT, five reflectance confocal microscopy (RCM), two photodynamic diagnosis (PDD), two dermoscopy, two MRI and one optical coherence tomography (OCT) paper(s). Dermoscopy, PDD, RCM and OCT can help to distinguish malignant conditions, including EMPD, from benign conditions. RCM and OCT can identify atypical cells in real-time, and have the potential to improve the accuracy of surgical margins intraoperatively and overall management. Distinctive confocal characteristics of EMPD have been described using RCM. The sensitivity and specificity of these findings require additional validation. Radiographic techniques also play a central role in the diagnosis of EMPD and assessment of disease spread. PET/CT and MRI can detect primary disease, nodal and distant metastases, with superior delineation of disease spread on MRI. Limitations of PET/CT are mainly related to primary tumour thickness, and size and FDG-avidity of nodal and distant metastases. Limitations of MRI include the fact that few studies have examined its use in EMPD; additional research is warranted. Randomized controlled trials and large prospective studies evaluating the use of non-invasive imaging in EMPD are needed.

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Section: Resultsmentioning

confidence: 90%

“…A few dark‐brown dots and large structureless brown and blue‐grey areas were also found. Similarly to Mun, they were unable to distinguish EMPD from other malignancies. A biopsy was obtained, and the patient was diagnosed with pigmented EMPD.…”

Section: Resultsmentioning

confidence: 92%

A review of non‐invasive imaging in extramammary Paget's disease

Bayan

Khanna

Rotemberg

et al. 2018

Acad Dermatol Venereol

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“…The most common sites affected are the vulva, penis, scrotum, perineum, perianal region, and axillae [ 1 ] . The most common sites affected are the vulva, penis, scrotum, perineum, perianal region, and axillae [ 1 ] .…”

Section: Discussionmentioning

confidence: 99%

“…Extramammary Paget's disease usually presents as well-demarcated, eczematous or eroded plaques in the anogenital region [ 3 ] . Misdiagnosis is common [ 1 ] , and symptoms may be present for a long time before the correct diagnosis is made [ 4 ] . Misdiagnosis is common [ 1 ] , and symptoms may be present for a long time before the correct diagnosis is made [ 4 ] .…”

Section: Discussionmentioning

confidence: 99%

Erythematous plaques of the vulvo‐perineal region: diagnostic role of dermatoscopy

Barisani

Dika

Fanti

et al. 2017

J Deutsche Derma Gesell

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A 78-year-old Caucasian woman presented with a fi ve-year history of pruritic vulvar and perianal lesions. Previous treatment with topical corticosteroids and antifungal agents had been ineffective. Clinical examination showed well-demarcated, erythematous, eczema-like plaques in the vulvar and perianal region (Figure 1 ). The patient was otherwise healthy, and blood tests were within normal limits.

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“…Although several mechanisms have been proposed to underlie the characteristic hypopigmentation, none of these hypothesized mechanisms has been fully accepted [1] [2] [3]. Dermoscopic observation has shown that-in comparison to other skin malignancies-a milky-red area is observed significantly more frequently in EMPDs [4]. In the present study, we evaluated the microscopic and ultrascopic features of hypopigmented lesions in detail.…”

Section: Introductionmentioning

confidence: 98%

The Disturbance of Melanogenesis and Melanosome Transfer in the Leukoderma Lesions of Extramammary Paget’s Disease

Tanemura¹,

Tanaka²,

Yang³

et al. 2018

JCDSA

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We frequently encounter characteristic color variation including hypopigmentation, hyperpigmentation, and erythema in extramammary Paget's disease (EMPD) lesions. Owing to unclear hypopigmentation, the lesional border of EMPD can be poorly defined and it is likely insufficient to perform its complete resection. Although the existence of Toker's cells and lack of lesional bFGF production have been reported to cause hypopigmentation inside of EMPD lesions, exact mechanisms of hypopigmentation in EMPD are not fully explored. We experienced three EMPD patients with obviously hypopigmented EMPD macules and histopathologically confirmed a reduced number of melanocytes on the hypopigmented macules and their loss on the erythematous plaques or nodules. An ultrastructural analysis on the hypopigmented lesions revealed disturbance of melanosome maturation and melanosome transfer to the adherent Pagets' cell on the basal layer. No Paget's cells even adhered to remaining melanocytes with dendrites contained matured melanosome and a few number of matured melanosome complexes were observed in basal keratinocytes. In the present study, we hypothesize that severe disturbance of not only melanogenesis but also melanosome transfer to surrounding Paget's cells and basal keratinocytes may cause characteristic hypopigmentation in EMPD. Future bioanalysis would reveal molecular mechanisms for hypopigmentation in EMPD.

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Clinical and dermoscopic characteristics of extramammary Paget disease: a study of 35 cases

Cited by 44 publications

References 15 publications

A review of non‐invasive imaging in extramammary Paget's disease

A review of non‐invasive imaging in extramammary Paget's disease

Erythematous plaques of the vulvo‐perineal region: diagnostic role of dermatoscopy

The Disturbance of Melanogenesis and Melanosome Transfer in the Leukoderma Lesions of Extramammary Paget’s Disease

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