Generic selectors
Exact matches only
Search in title
Search in content
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 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
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
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
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
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
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 Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Observation Letter
2018:84:3;341-344
doi: 10.4103/ijdvl.IJDVL_525_17
PMID: 29536968

Extramammary Paget disease with histologic presence of epidermolytic hyperkeratosis: An unusual presentation

Anil Kumar Bhatta, Yeqiang Liu, Uma Keyal
 Department of Dermatopathology, Skin Disease Hospital, Shanghai, Tongji University School of Medicine, Shanghai, China

Correspondence Address:
Yeqiang Liu
Department of Dermatopathology, Shanghai Skin Disease Hospital, No. 1278, Baode Road, Jingan District, Shanghai - 200 443
China
Published: 09-Mar-2018
How to cite this article:
Bhatta AK, Liu Y, Keyal U. Extramammary Paget disease with histologic presence of epidermolytic hyperkeratosis: An unusual presentation. Indian J Dermatol Venereol Leprol 2018;84:341-344
Copyright: (C)2018 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Extramammary Paget disease is an intraepithelial neoplasm that presents typically in apocrine-rich skin as an irregular, pruritic plaque. Epidermolytic hyperkeratosis is a histological reaction pattern seen in a variety of conditions, both acquired and congenital including benign, premalignant as well as malignant disease processes. Sometimes, epidermolytic hyperkeratosis can be seen as an incidental finding.

Extramammary Paget disease, first described by Crocker in 1889, is an intraepithelial neoplasm that typically presents as an irregular pruritic plaque at an apocrine gland-bearing skin site such as vulva, axillae, groin, scrotum and perianal region.[1] The term epidermolytic hyperkeratosis refers to a distinctive set of histopathologic features of hyperkeratosis, perinuclear vacuolization of keratinocytes in the upper layers of epidermis and coarse keratohyaline granules.[2] The presence of these findings is also noted in various other entities such as epidermolytic ichthyosis, Vorner's epidermolytic palmoplantar keratoderma, epidermal nevus (especially systematized variant) and solitary epidermolytic acanthomas.[3] However, we were unable to find any previous paper reporting its presence in extramammary Paget disease. Here, we report a case of extramammary Paget disease, which on histopathology examination showed the findings of both extramammary Paget disease and epidermolytic hyperkeratosis.

A 75-year-old otherwise healthy man presented at our hospital with an itchy lesion on the left groin for 1 year. The lesion had gradually increased in size to approximately 4 × 2 cm in diameter. On physical examination, a well-demarcated erythematous plaque with lichenification and peripheral hypopigmentation was noted [Figure - 1]. Systemic examination revealed no abnormalities and no regional lymphadenopathy was detected. A biopsy was performed with a clinical suspicion of Bowen's disease or extramammary Paget disease. Histopathological examination revealed hyperkeratosis, parakeratosis, acanthosis and vacuolated Paget cells in suprabasilar epithelium. Immunohistochemistry showed Paget cells positive for cytokeratin7, carcinoembryonic antigen (CEA), epithelial membrane antigen, gross cystic disease fluid protein-15 and periodic acid-Schiff (PAS), but negative for cytokeratin20. Ultrasonography of the abdomen was done to look for the primary site but no abnormalities were detected. Prostate-specific antigen was within normal limit. Based on these findings, a diagnosis of primary extramammary Paget disease was made. In addition, hyperkeratosis, papillomatosis, clumped keratohyalin granules and superficial epidermal vacuolization was also seen, which are specific for epidermolytic hyperkeratosis [Figure - 2].

Figure 1: A well-demarcated erythematous plaque with lichenification and hypopigmented surrounding skin
Figure 2

Histopathologically, epidermolytic hyperkeratosis has been noted in a variety of acquired and congenital conditions. Various benign diseases (acanthoma, epidermoid cyst, infundibular cyst, epidermal nevus, hidradenoma, nevus comedonicus, seborrheic keratosis, trichilemmal cyst), premalignant lesions (actinic keratosis, leukoplakia, atypical/dysplastic nevus), as well as malignant lesions (basal cell carcinoma, melanoma, squamous cell carcinoma) have shown concurrent findings of epidermolytic hyperkeratosis on histopathology examination. The exact clinical significance of epidermolytic hyperkeratosis findings in these conditions is still unknown.[4]

Although the presence of specific histologic features of epidermolytic hyperkeratosis in other disease conditions is well reported in literature, its clinical significance is still unknown.[1],[2] In our patient, a pruritic lesion leading to chronic itch and scratch might have given rise to these findings. Whether this is just an incidental finding with no diagnostic value or it holds a clinical significance is yet to be established.

Treatment of choice in extramammary Paget's disease remains surgical excision. Wide local excision and Moh's micrographic surgical excision have been tried with variable recurrence rates.[5] For patients who decline or are unfit for surgery, alternative treatment options include topical chemotherapy and immunomodulators such as 5-fluorouracil, bleomycin and imiquimod as adjunctive or primary modalities of treatment.[6] In our patient, complete excision under local anesthesia was done. The healing and recovery post procedure was uneventful. At 1-year follow-up no recurrence was noted.

Acknowledgment

This study was funded by grants from National Natural Science Foundation of China (NSFC 81360236), Shanghai Hospital Development Center project (SHDC12014217) and Shanghai Committee of Science and Technology, China (16411961500).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Funaro D, Krasny M, Lam C, Desy D, Sauthier P, Bouffard D, et al. Extramammary Paget disease: Epidemiology and association to cancer in a Quebec-based population. J Low Genit Tract Dis 2013;17:167-74.
[Google Scholar]
2.
Kumar P, Kumar R, Mandal RK, Hassan S. Systematized linear epidermolytic hyperkeratosis. Dermatol Online J 2014;20:21248.
[Google Scholar]
3.
Fletcher JW, Ramamurthi A, Parekh P. Presentation of epidermolytic acanthomas as multiple tan papules on the vulva. Proc (Bayl Univ Med Cent) 2016;29:198-9.
[Google Scholar]
4.
Vincent J, Taube JM. Pigmented extramammary Paget disease of the abdomen: A potential mimicker of melanoma. Dermatol Online J 2011;17:13.
[Google Scholar]
5.
Moretto P, Nair VJ, Hallani SE, Malone S, Belanger E, Morash C, et al. Management of penoscrotal extramammary Paget disease: Case series and review of the literature. Curr Oncol 2013;20:e311-20.
[Google Scholar]
6.
MartIn-Fuentes A, Sánchez-Herreros C, Cuevas-Santos J, De Eusebio-Murillo E. Extramammary Paget disease secondary to a transitional cell carcinoma of the bladder. Indian J Dermatol Venereol Leprol 2015;81:318-20.
[Google Scholar]

Fulltext Views
455

PDF downloads
491
Show Sections