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
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
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:

Case Letter
ARTICLE IN PRESS
doi:
10.25259/IJDVL_659_2023

Giant syringocystadenoma papilliferum along the Blaschko’s lines

Department of Dermatology and Venerology, The First Hospital of Jilin University, Changchun, Jilin Province, China
Plastic Surgery, The First Hospital of Jilin University, Changchun, Jilin Province, China

Corresponding author: Prof. Yaping Tian, Department of Dermatology and Venerology, The First Hospital of Jilin University, Changchun, Jilin Province, China. typ@jlu.edu.cn

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: Han B, Zhang W, Li S, Wang H, Tian Y. Giant syringocystadenoma papilliferum along the Blaschko’s lines. Indian J Dermatol Venereol Leprol. doi: 10.25259/IJDVL_659_2023

Dear Editor,

Syringocystadenoma papilliferum (SCAP) is a benign adnexal tumour originating from both apocrine and eccrine histogenesis. It was first reported as ‘naevus syringadenomatosus papilliferus’ by Stokes in 1917.1 SCAP typically presents as a papillary or solitary lesion in the head or neck region. On rare occasions, it presents as multiple small papules, occasionally along Blaschko lines. We report an unusual case of giant SCAP presenting along Blaschko lines on the thigh. We also reviewed the relevant literature on giant SCAP to further comprehend this disease.

A 24-year-old woman presented with red giant tumours over the right outer thigh in a linear configuration in 2023. The lesions began as several small papules shortly after birth, which then gradually increased in size and number without showing any other symptoms. However, the lesions progressed significantly with traumatic bleeding in 2022. The patient has no history of trauma or any notable medical issues. A physical examination revealed several red, dark-brown, moist, cauliflower-like papules measuring 1–5 mm in diameter and plaques of sizes 4 x 5 cm, 5 x 5 cm and 9 x 3 cm on the right thigh along the Blaschko lines [Figure 1]. All laboratory data were within the normal ranges. Squamous cell carcinoma, giant warts, tubular apocrine adenoma, and verrucous naevus were initially considered as differential diagnoses. Thereafter, a punch biopsy of the largest lesion revealed an exophytic configuration composed of epithelium-covered papillae [Figure 2a]. The inner layer of the papillae showed small cuboidal myoepithelial cells with hyperchromatic nuclei, while the outer layer was composed of tall columnar cells with eosinophilic cytoplasm, large vesicular nuclei and decapitation secretion [Figure 2b]. The stroma contained numerous plasma cells. Immunohistochemistry results indicated that the columnar cells were positive for AE1/AE3 [Figure 2c], CK-7 [Figure 2d] and carcinoembryonic antigen (CEA) [Figure 2e], while the cuboidal cells were positive for P63 [Figure 2f]. Plasma cells of the stroma were positive for CD38 [Figure 2g]. SCAP was diagnosed based on the clinical findings and characteristic histological features of the present case. All lesions were completely excised and closed with a tension-reducing sutures. The excised lesions were reconfirmed as SCAP by histopathological examination without any malignant transformation. The wound healed completely eight weeks after the surgery.

Multiple red, dark-brown, moist, cauliflower-like papules and plaques on the right thigh along Blaschko lines.
Figure 1:
Multiple red, dark-brown, moist, cauliflower-like papules and plaques on the right thigh along Blaschko lines.
Exophytic configuration composed of epithelium-covered papillae (Haematoxylin and Eosin, 40x).
Figure 2a:
Exophytic configuration composed of epithelium-covered papillae (Haematoxylin and Eosin, 40x).
The inner layer of the papillae included small cuboidal myoepithelial cells, while the outer layer was composed of tall columnar cells (Haematoxylin and Eosin, 100x).
Figure 2b:
The inner layer of the papillae included small cuboidal myoepithelial cells, while the outer layer was composed of tall columnar cells (Haematoxylin and Eosin, 100x).
Columnar cells were positive for CK cocktail AE1/AE3 (AE1/AE3 Immunohistochemistry, 100x).
Figure 2c:
Columnar cells were positive for CK cocktail AE1/AE3 (AE1/AE3 Immunohistochemistry, 100x).
Columnar cells were positive for CK-7 (CK-7 Immunohistochemistry, 100x).
Figure 2d:
Columnar cells were positive for CK-7 (CK-7 Immunohistochemistry, 100x).
Columnar cells were positive for CEA (CEA Immunohistochemistry, 100x).
Figure 2e:
Columnar cells were positive for CEA (CEA Immunohistochemistry, 100x).
Cuboidal cells were positive for P63 (P63 Immunohistochemistry, 100x).
Figure 2f:
Cuboidal cells were positive for P63 (P63 Immunohistochemistry, 100x).
Plasma cells of the stroma were positive for CD38 (CD38 Immunohistochemistry, 100×).
Figure 2g:
Plasma cells of the stroma were positive for CD38 (CD38 Immunohistochemistry, 100×).

SCAP is a benign cutaneous neuroma derived from both apocrine and eccrine histogenesis and composed of undifferentiated pluripotential cells.2 Clinically, it typically presents as a dark-brown or grey papillary or rather warty, sometimes crusty, excrescence with a moist appearance that may manifest at birth or develop during childhood.2 Head and neck regions are most commonly involved sites.2 The following three clinical types of SCAP have been described so far: solitary nodular type, plaque type and linear type, of which, the linear type is rare.3 So far, there have been only four published cases of giant SCAP reported in the English literature,1,46 and none has been described to have presented on the thigh as a linear type. Based on the literature review, the age at the time of diagnosis, sex, the age of onset, location, appearance, clinical features, and any accompanying diseases are summarised in Table 1. Histologically, SCAP has a characteristic that makes it readily recognisable owing to its papillary projection consisting of two rows of cells – columnar and cuboidal, and the stroma contains numerous plasma cells.2 SCAP has been reported to be positive for carcinoembryonic antigen, cytokeratin AE1 (CK AE1), and epithelial membrane antigen (EMA) in columnar cells and positive for P63 in cuboidal cells.2 The clinical and histological features of the present case were found to be consistent with those of SCAP.

Table 1: The published reports on cases of giant syringocystadenoma papilliferum (SCAP)
Author (date) Age (year)/sex Age of onset (year) Location Appearance Clinical features Accompanying diseases
Kar et al.1 (2012) 12/M Since birth Lower back A 20-cm long and 7-cm wide fleshy cauliflower-like erythematous papulonodular lesion arranged in a linear array No No
Yaghoobi et al.4 (2009) 20/F 19 Left-sided occipital part of the scalp A linear lesion, highly elevated, moist, fetid, vegetated and pinkish, measuring 8 × 3 × 2 cm in diameter No Hypochromic, microcytic anaemia
Sardesai et al.5 (2009) 30/F 25 The medial aspect of the right thigh Two skin lesions: (large) 6 × 7 cm and (small) 1 × 3 cm No Condyloma acuminata
Kaehler et al.6 (2005) 43/M Since birth The left side of the ventral thorax A giant, 4 × 6 cm brownish, verrucous tumour with several scars No No
Our case (2023) 24/F Since birth Right thigh along Blaschko line Several red, dark-brown, moist, cauliflower-like papules, measuring 1–5 mm in diameter, and plaques of size 4 × 5 cm, 5 × 5 cm, and 9 × 3 cm, respectively, on the right thigh along the Blaschko lines Traumatic bleeding No

SCAP has recently been reported to be associated with somatic activating mutations in the HRAS, KRAS or BRAF oncogenes.7 Owing to financial reasons, the patient refused genetic testing. Surgical excision is the first-line treatment option for SCAP and CO2 laser may be considered for anatomic sites unfavourable to surgical excision.1 All lesions of the present patient were removed surgically.

In summary, we report a case of Blaschko-linear SCAP located on the thigh with a giant appearance. Our case findings highlight the important role played by histopathological examination to rule out other cutaneous tumours demonstrating similar characteristics.

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.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

References

  1. , , . Giant linear syringocystadenoma papilliferum of the back. Indian J Dermatol Venereol Leprol. 2012;78:123.
    [CrossRef] [PubMed] [Google Scholar]
  2. . Pathology of the skin with clinical correlations. Elsevier Limited; . p. :1510-11.
  3. , , , . Syringocystadenoma papilliferum in the scalp, with a linear presentation. An Bras Dermatol. 2023;98:406-9.
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]
  4. , , . Giant linear syringocystadenoma papilliferum on scalp. Indian J Dermatol Venereol Leprol. 2009;75:318-19.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , . Giant condyloma acuminata with syringocystadenoma papilliferum. Indian J Dermatol Venereol Leprol. 2009;75:330.
    [CrossRef] [PubMed] [Google Scholar]
  6. , , , . Giant naevoid syringocystadenoma papilliferum. Acta Derm Venereol. 2005;85:453-4.
    [CrossRef] [PubMed] [Google Scholar]
  7. , , , , , , et al. Activating mutations in the RAS/mitogen-activated protein kinase signaling pathway in sporadic trichoblastoma and syringocystadenoma papilliferum. Hum Pathol. 2015;46:272-6.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
338

PDF downloads
1,267
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections