Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Quiz
89 (
1
); 73-75
doi:
10.25259/IJDVL_608_19
pmid:
34114413

Well-demarcated erythematous plaque on the arm

Department of Dermatology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Corresponding author: Prof. Young Min Park, Department of Dermatology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seochogu, Seoul, 137-701, Korea. 96015367@cmcnu.or.kr

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Song YM, Han JH, Bang CH, Lee JH, Lee JY, Park YM. Well-demarcated erythematous plaque on the arm. Indian J Dermatol Venereol Leprol 2023;89:73-5.

A 67-year-old Caucasian man presented with an asymptomatic, well-defined, irregular shaped, erythematous scaly plaque on the left upper arm for five years [Figure 1]. There was no cervical or axillary lymphadenopathy. A skin punch biopsy was performed from the plaque.

An irregular shaped erythematous plaque with scaling, on the left upper arm
Figure 1:
An irregular shaped erythematous plaque with scaling, on the left upper arm

Question

What is your diagnosis?

Answer

Ectopic extramammary Paget disease

Histopathological examination showed hyperkeratosis, acanthosis and atypical large pale cells with abundant vacuolated cytoplasm in the epidermis.

There was no dermal invasion [Figures 2a and b]. Tumor cells were positive for cytokeratin 7, but negative for cytokeratin 20, P63 and Melan A by immunohistochemistry [Figures 3a-d].

Histological examination showed hyperkeratosis, acanthosis and atypical large pale cells with abundant vacuolated cytoplasm in the epidermis (H and E, ×200)
Figure 2a:
Histological examination showed hyperkeratosis, acanthosis and atypical large pale cells with abundant vacuolated cytoplasm in the epidermis (H and E, ×200)
Intraepithelial large cells with a vesicular nucleus and prominent nucleolus (H and E, ×400)
Figure 2b:
Intraepithelial large cells with a vesicular nucleus and prominent nucleolus (H and E, ×400)
Tumor cells were positive for cytokeratin 7 (immunohistochemistry, ×200)
Figure 3a:
Tumor cells were positive for cytokeratin 7 (immunohistochemistry, ×200)
Tumor cells were negative for cytokeratin 20 (immunohistochemistry, ×200)
Figure 3b:
Tumor cells were negative for cytokeratin 20 (immunohistochemistry, ×200)
Tumor cells were negative for P63 whereas normal epidermal cells were positive (immunohistochemistry, ×200)
Figure 3c:
Tumor cells were negative for P63 whereas normal epidermal cells were positive (immunohistochemistry, ×200)
Tumor cells were negative for Melan A (immunohistochemistry, ×200)
Figure 3d:
Tumor cells were negative for Melan A (immunohistochemistry, ×200)

The lesion was totally excised with 2 mm negative margins and was lost to follow up.

Discussion

Extramammary Paget disease is a rare intraepidermal adenocarcinoma that primarily affects apocrine gland-bearing skin most commonly over the anogenital area. When the disease affects nonapocrine bearing skin, it is called ectopic extramammary Paget disease.1 The pathophysiology of ectopic extramammary Paget disease is unknown. Sawada et al. reported the possibility that ectopic extramammary Paget disease originates potentially not only from the apocrine gland but also from the eccrine gland.2 Clinically and histologically, there is no difference between ectopic extramammary Paget disease and conventional one.2 Extramammary Paget disease is well known for its association with malignancy. Chanda reported the incidence of underlying adnexal carcinoma and concurrent internal malignancy associated with extramammary Paget disease as 24% and 12%, respectively.3 The location of the underlying internal malignancy appears to correlate with the site of extramammary Paget disease. For example, the perianal and perineal location is associated with adenocarcinoma of the digestive system and genitourinary malignancy, respectively.4

Till now, there have been 46 cases of ectopic extramammary Paget disease in literature, of which only two cases have been reported in association with internal malignancy. The first case is ectopic extramammary Paget disease of the lateral thigh arising in association with sweat gland carcinoma of the same area,5 the second being ectopic extramammary Paget disease in the midline of the abdomen with underlying colon adenocarcinoma.6

Whole-body evaluation for malignancy should be performed. For evaluation of internal neoplasm, positron emission tomography-computed tomography, upper and lower endoscopy and laboratory tests were recommended. However, the patient refused further testing.7

Extramammary Paget disease is clinically similar to Bowen disease and superficial basal cell carcinoma.8 Thus, clinicians should keep in mind the possibility of ectopic extramammary Paget disease when there is treatment resistance skin lesion with eczematous morphology on the apocrine gland poor regions.

The diagnosis of extramammary Paget disease, in this case, was supported by immunohistochemical findings. Pagetoid cells in pagetoid Bowen disease usually demonstrate immunohistochemical features of squamous cells, which are negative for CK7 and positive for P63.9 In this case, positivity for CK7 but negativity for p63 and Melan A supported the diagnosis of extramammary Paget disease, ruling out Bowen disease and melanoma in situ. A negative result of CK20 immunohistochemical stain supported the diagnosis of primary extramammary Paget disease rather than secondary extramammary Paget disease from an internal malignancy.9 This case has been reported as a rare case of ectopic extramammary Paget disease appearing on an unusual site.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

  1. , . Ectopic extramammary paget’s disease on the head: Case report and literature review. Int J Dermatol. 2015;54:e483-6.
    [CrossRef] [PubMed] [Google Scholar]
  2. , , , , , , et al. Ectopic extramammary paget’s disease: Case report and literature review. Acta Derm Venereol. 2010;90:502-5.
    [CrossRef] [PubMed] [Google Scholar]
  3. . Extramammary paget’s disease: Prognosis and relationship to internal malignancy. J Am Acad Dermato. 1985;13:1009-14.
    [CrossRef] [Google Scholar]
  4. , , . Definition, Association with malignancy, biologic behavior, and treatment of ectopic extramammary paget’s disease: A review of the literature. J Clin Aesthet Dermatol. 2019;12:40-4.
    [Google Scholar]
  5. , , . Extramammary Paget’s disease. Arising in knee region in association with sweat gland carcinoma. Arch Pathol Lab Med. 1984;108:713-6.
    [Google Scholar]
  6. , . Pigmented extramammary paget disease of the abdomen: A potential mimicker of melanoma. Dermatol Online J. 2011;17:13.
    [Google Scholar]
  7. , , , , , , et al. Paget’s disease In: Fitzpatrick’s Dermatology (9th ed). New York: McGraw-Hill Education; . p. :1940.
    [Google Scholar]
  8. , , , , , . Pagetoid bowen disease initially misdiagnosed as ectopic extramammary paget’s disease. Ann Dermatol. 2018;30:218-21.
    [CrossRef] [PubMed] [Google Scholar]
  9. , . The use of cytokeratins 7 and 20 in the diagnosis of primary and secondary extramammary paget’s disease. Br J Dermatol. 2000;142:243-7.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
8,915

PDF downloads
5,382
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections