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
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 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
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 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
2012:78:4;517-518
doi: 10.4103/0378-6323.98094

Multiple skin nodules over the scalp and trunk

Mohanan Sarita1 , Rashmi Kumari1 , Nachiappa Ganesh Rajesh2 , Devinder Mohan Thappa1
1 Department of Dermatology and STD, JIPMER, Pondicherry, India
2 Department of Pathology, JIPMER, Pondicherry, India

Correspondence Address:
Rashmi Kumari
Assistant Professor, Department of Dermatology and STD, JIPMER, Pondicherry-605006
India
How to cite this article:
Sarita M, Kumari R, Rajesh NG, Thappa DM. Multiple skin nodules over the scalp and trunk. Indian J Dermatol Venereol Leprol 2012;78:517-518
Copyright: (C)2012 Indian Journal of Dermatology, Venereology, and Leprology

A 50-year-old man came with multiple asymptomatic nodules on the face, scalp and upper trunk for the past 10 years. These nodules were slowly increasing in size. There was no family history of similar lesions. On cutaneous examination, there were multiple large, shiny, skin colored, lobulated, firm, non-tender nodules of varying sizes, largest measuring 5 × 4 cm in size over the scalp [Figure - 1]. He also had multiple soft, non-tender papules and nodules of size 1 × 1.5 cm over the central face, forehead and trunk. Button-hole sign was negative. Patient also had ill-defined depigmented macules, suggestive of vitiligo on trunk, lips and scalp. An excisional biopsy was done from the large nodule on scalp and the soft papules on the trunk. Histopathology revealed closely set mosaic like masses and columns of cells surrounded by a thick basal membrane [Figure - 2] and [Figure - 3]. The hyaline thick basal membrane was periodic acid schiff stain positive [Figure - 4] and diastase resistant.

Figure 1: Multiple skin colored nodules and papules seen over the scalp and face
Figure 2: Section shows nests of basaloid tumor cells arranged in a peculiar pattern. Tumor cells secrete thick basement membrane material (H and E, × 100)
Figure 3: Section shows nests of basaloid tumor cells exhibiting peripheral palisading with center comprised nests of pale cells (H and E, × 400)
Figure 4: Section shows PAS positivity of basement membrane material secreted by the tumor cells, (PAS stain, × 400)

What is your diagnosis?

Answer: Cylindroma

Histopathology section showed nests of basaloid tumor cells arranged in jigsaw puzzle pattern. The nests of basaloid tumor cells exhibited peripheral palisading with center of the nests comprised of pale cells. Tumor cells secreted thick basement membrane material, which was PAS positive and diastase resistant. The tumor cells showed nuclear positivity for p63 in the myoepithelial cells of the tumor (IHC DAB stain, × 400) [Figure - 5]. These features were suggestive of cylindroma. Surgical excision of the tumors was done for 2 large nodules after which the patient is on follow-up.

Figure 5: Section shows nuclear positivity for p63 in the myoepithelial cells of the tumor (IHC DAB stain, × 400)

Discussion

Cylindroma is a rare, benign appendageal tumor arising from eccrine glands. They are also purported to arise from stem cells of hair follicle. [1] They are twice as common in females as compared to males and rarely undergo malignant transformation. Multiple cylindromas can occur as part of familial cylindromatosis (OMIM 313270), Brooke-Spiegler syndrome (OMIM 605041) and multiple familial trichoepitheliomas (OMIM 601606). [1] Brooke-Spiegler syndrome is an autosomal dominant condition caused by mutation in the cylindromatosis CYLD gene, located on 16 q12-q13. [2] The gene product represses the tumor necrosis factor-α (TNF-α) pathway, which increases the expression of nuclear factor-κβ (NF-κβ), which regulates a number of anti-apoptotic genes involved in proliferation of skin appendages. [1],[2] Multiple cylindromas can cover the entire scalp resulting in hair loss, forming "turban tumors". The condition is associated with multiple trichoepitheliomas and spiradenomas in addition to trichoblastomas, basal-cell carcinomas, follicular cysts, organoid nevi etc. [3] Differential diagnoses to be considered eccrine spiradenoma, follicular infundibulum tumor and pilar cyst. Malignant transformation is said to occur more commonly with multiple cylindromas than in solitary cylindroma. 4 Hence, it is important to do a biopsy in patients with multiple cylindromas. Histopathology reveals well-circumscribed dermal lesions with areas of basaloid cell proliferation arranged in jigsaw-puzzle pattern. Rarely, neoplasms with hybrid features are seen including occurrence of all 3 adnexal tumors in a single lesion. [3] Histopathologically, it has to be differentiated from spiradenoma and adenoid cystic carcinoma. Staining with p63 indicates cells of myoepithelial origin, suggesting differentiation towards eccrine secretory cells. In addition to neoplasms of the skin appendages, patients with Brooke-Spiegler syndrome also are at risk for developing tumors of the salivary glands, such as basal-cell adenomas and adenocarcinomas of the parotid glands and minor salivary glands. [1] Reported treatments of cylindromas include excision, dermabrasion, electrodessication, CO 2 laser, cryotherapy and radiotherapy. Wide local excision is also preferred to avoid recurrence. [2] To avoid occurrence of new tumors, topical aspirin derivatives are currently being tried, which reduce the expression of NF-κβ. [1],[2]

References
1.
Rajan N, Langtry JAA, Ashworth A, Roberts C, Chapman P, Burn J, et al. Tumor mapping in two large multigenerational families with CYLD mutations: Implications for patient management and tumor induction. Arch Dermatol 2009;145:1277-84.
[Google Scholar]
2.
Doherty SD, Barrett TL, Joseph AK. Brooke-Spiegler syndrome: Report of a case of multiple cylindromas and trichoepitheliomas. Dermatol Online J 2008;14:8.
[Google Scholar]
3.
Kim C, Kovich OI, Dosik J. Brooke-Spiegler syndrome. Dermatol Online J 2007;13:10.
[Google Scholar]

Fulltext Views
3,103

PDF downloads
1,472
Show Sections