Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
ANNOUNCEMENT
Case Report
Case Series
Clinicodermoscopic Challenge
Clinicopathologic Challenge
Correspondence
Editorial
Faculty’s Forum
Image Correspondence
Innovations and Ideas
Letter to Editor
Original Article
Post Graduate Thesis Section
Quiz
Research Methodology and Publishing
Resident’s Forum
Review Article
Reviewers 2023
Short Communication
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
ANNOUNCEMENT
Case Report
Case Series
Clinicodermoscopic Challenge
Clinicopathologic Challenge
Correspondence
Editorial
Faculty’s Forum
Image Correspondence
Innovations and Ideas
Letter to Editor
Original Article
Post Graduate Thesis Section
Quiz
Research Methodology and Publishing
Resident’s Forum
Review Article
Reviewers 2023
Short Communication
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
ANNOUNCEMENT
Case Report
Case Series
Clinicodermoscopic Challenge
Clinicopathologic Challenge
Correspondence
Editorial
Faculty’s Forum
Image Correspondence
Innovations and Ideas
Letter to Editor
Original Article
Post Graduate Thesis Section
Quiz
Research Methodology and Publishing
Resident’s Forum
Review Article
Reviewers 2023
Short Communication
View/Download PDF

Translate this page into:

Image Correspondence
1 (
1
); 63-64
doi:
10.25259/IJPGD_43_2022

Ehler Danlos syndrome

Department of Dermatology, Venereology and Leprosy, S P Medical College, Bikaner, Rajasthan, India

*Corresponding author: Madhvi Trivedi, Department of Dermatology, Venereology and Leprosy, S P Medical College, Bikaner, Rajasthan, India. trivedimadhvi24@gmail.com

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: Trivedi M, Mehta RD, Ghiya BC, Soni P. Ehler Danlos syndrome. Indian J Postgrad Dermatol 2023;1:63-4.

A 37-year-old male presented to skin department with complaint of multiple scars and lesion on chest. On dermatological examination, multiple well-defined and atrophic scars were present on forehead and cheeks. Epicanthal folds were also prominent [Figure 1a]. Multiple cigarette paper scars were present over both wrists, forehands, and knees [Figure 1b]. Multiple skin colored to white colored well-defined 1 cm × 2 cm papules identified as spheroids were present on the upper chest [Figure 1c]. Skin stretching revealed hyperextensible skin with normal recoil [Figure 1d]. Skin was soft and there was history of skin fragility. Joint mobility was normal. Systemic examination was normal. There was no history of bleeding tendency. Family history was also not significant.

(a) Scars on face and epicanthal folds. (b) Cigarette paper scars on wrist and forearm. (c) Subcutaneous spheroids. (d) Hyperextensible skin.
Figure 1:
(a) Scars on face and epicanthal folds. (b) Cigarette paper scars on wrist and forearm. (c) Subcutaneous spheroids. (d) Hyperextensible skin.

We considered a diagnosis of Ehler Danlos system and reviewed the crtieria for the same as mentioned below:

  • Major criteria

    1. Skin hyperextensibilty and atrophic scarring

    2. Generalized joint hypermobility.

  • Minor criteria

    1. Easy bruising

    2. Soft and doughy skin

    3. Skin fragility

    4. Molluscoid pseudotumors

    5. Subcutaneous spheroids

    6. Hernia (or history thereof)

    7. Epicanthal folds

    8. Complications of joint hypermobility

    9. Family history of first degree relative who meets clinical criteria.

Our patient fulfilled one major and four minor criteria. Confirmatory molecular testing for mutations in gene encoding type V collagen (COL5A1 and COL5A2) is necessary for the final diagnosis but it could not be done due to lack of availability of resources.

Declaration of patient consent

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

Conflicts of interest

There are no conflicts of interest.

Financial support and sponsorship

Nil.


Fulltext Views
857

PDF downloads
683
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections