Generic selectors
Exact matches only
Search in title
Search in content
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 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:

Net Letter
2014:80:5;484-484
doi: 10.4103/0378-6323.140352
PMID: 25201870

Capillary malformation associated with multiple accessory tragi

Pravesh Yadav, Vibhu Mendiratta, Saurabh Mittal, Ram Chander
 Departments of Dermatology and Sexually Transmitted Diseases, Lady Hardinge Medical College and Suchita Kriplani Hospital, Delhi, India

Correspondence Address:
Pravesh Yadav
RZ-97, Phase-III, Prem Nagar, Najafgarh, New Delhi - 110 043
India
How to cite this article:
Yadav P, Mendiratta V, Mittal S, Chander R. Capillary malformation associated with multiple accessory tragi . Indian J Dermatol Venereol Leprol 2014;80:484
Copyright: (C)2014 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

A 5-year-old boy, born of an uneventful pregnancy, was brought to our outpatient department with complaints of reddish discoloration over the left side of face and chin along with two skin-colored swellings in front of the left ear since birth which increased in size in proportion to the body size. The child was developmentally normal and had normal intelligence. There was no history of seizures. There was no family history of capillary malformation or branchial arch anomalies. Examination revealed multiple erythematous, non-blanchable patches present along the mandibular distribution of trigeminal nerve and the lower lip along with a diffuse swelling of the lower lip. There was no thrill or bruit. In addition, there were two skin-colored fleshy papules present just anterior to the left pinna [Figure - 1]. Examination of the oral cavity was unremarkable. Ophthalmic, neurological, and systemic examination was normal. Imaging of the head and neck region, chest radiograph, ultrasound of the abdomen and pelvis, and echocardiograph were unremarkable. Hematological and biochemical investigations were normal. The parents of the child refused biopsy or treatment of both the accessory tragus and capillary malformation.

Figure 1: Capillary malformation present along the mandibular distribution of trigeminal nerve associated with diffuse swelling of the lower lip and multiple tragi present just anterior to the left pinna

Capillary malformation is a low-flow vascular malformation commonly affecting the face in the distribution of the trigeminal nerve (V1, V2, and V3). Lesions affecting the face may extend into the mucosal surfaces such as the lips and gingiva. They are sometimes associated with an underlying disorder in the form of various syndromes such as Sturge-Weber syndrome. Immunohistochemical and confocal microscopic studies of capillary malformations reveal a significantly decreased density of perivascular nervous tissue in lesional skin suggesting that inadequate innervation may be responsible for decreased vascular tone and progressive vascular dilatation. [1],[2] A potential role of vascular endothelial growth factor (VEGF)-A and its most active receptor VEGF-R2 expression, [3] as well as RASA1 gene on chromosome 5q encoding a protein (p120-rasGAP) [4] has been elucidated probably by inducing vessel proliferation and/or vasodilatation.

A normal tragus is derived from the dorsal portion of the first branchial arch as it grows ventrally to join in the midline during embryonic life. Isolated accessory auricles may be inherited as an autosomal dominant trait, mapped to chromosome 14q11.2-12. [5] Accessory tragi may also occur with other malformations and syndromes of the first branchial arch, such as Treacher Collins, Goldenhar, Nager′s acrofacial dysostosis, Wolf-Hirschhorn syndrome (4p-), oculocerebrocutaneous syndrome, Townes′ syndrome, and Down′s syndrome.

The mandibular and maxillary branches of the trigeminal nerve are also derived from the first branchial arch and innervate structures derived from the first arch. A defect in the first branchial arch possibly associated with a linked mutation potentially offers an explanation for this concurrence. The associated mutation may lead to defective trigeminal nerve innervation of blood vessels in the mandibular region leading to progressive vascular dilatation manifesting as a capillary malformation and the first branchial defect may lead to defective closure ventrally leading to accessory tragi. Also, both capillary malformation and accessory tragi have been known to be autosomal dominantly inherited.

We were unable to find previous reports of an association of capillary malformation with accessory tragus. The association in our patient may be conicidental, but such associations provide an opportunity to explore possible genetic or pathogenetic links between the two conditions. Unfortunately, we were unable to carry out genetic studies in our patient. Though our patient did not have any systemic involvement, patients with these pathologies should be screened for other local and systemic abnormalities.

References
1.
Smoller BR, Rosen S. Port-wine stains. A disease of altered neural modulation of blood vessels? Arch Dermatol 1986;122:177-9.
[Google Scholar]
2.
Selim MM, Kelly KM, Nelson JS, Wendelschafer-Crabb G, Kennedy WR, Zelickson BD. Confocal microscopy study of nerves and blood vessels in untreated and treated port wine stains: Preliminary observations. Dermatol Surg 2004;30:892-7.
[Google Scholar]
3.
Vural E, Ramakrishnan J, Cetin N, Buckmiller L, Suen JY, Fan CY. The expression of vascular endothelial growth factor and its receptors in port-wine stains. Otolaryngol Head Neck Surg 2008;139:560-4.
[Google Scholar]
4.
Eerola I, Boon LM, Mulliken JB, Burrows PE, Dompmartin A, Watanabe S, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet 2003;73:1240-9.
[Google Scholar]
5.
Yang Y, Guo J, Liu Z, Tang S, Li N, Yang M, et al. A locus for autosomal dominant accessory auricular anomaly maps to 14q11.2-q12. Hum Genet 2006;120:144-7.
[Google Scholar]

Fulltext Views
162

PDF downloads
142
Show Sections