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
Images in Dermatology
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
Media and news
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
Images in Dermatology
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
Media and news
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:

Letter to the Editor
2012:78:1;105-106
doi: 10.4103/0378-6323.90961
PMID: 22199073

A case of Parry-Romberg syndrome with congenital heart disease

Supriya P Deshmukh1 , Yugal K Sharma1 , Bharat B Dogra2 , Nitin C Chaudhari1
1 Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India
2 Department of Surgery, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India

Correspondence Address:
Supriya P Deshmukh
401- Gaurangi CHS, Opposite Damani Estate Post Office, L.B.S. Marg, Thane - 400 601, Maharashtra
India
How to cite this article:
Deshmukh SP, Sharma YK, Dogra BB, Chaudhari NC. A case of Parry-Romberg syndrome with congenital heart disease. Indian J Dermatol Venereol Leprol 2012;78:105-106
Copyright: (C)2012 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

A 19-year-old female presented to our outpatient department with an asymptomatic, right-sided facial atrophy and alopecia involving frontal region of scalp and right eyebrow since four years [Figure - 1]. Skin over the involved areas was smooth in texture, non-dyspigmented, and could be easily pinched up on palpation. In addition to this, right eye showed retraction of upper-lid margin with loss of cilia and same-sided ear pinna showed atrophy of the skin and cartilage [Figure - 2]. She had a history of being operated for a congenital heart disease, i.e., Shone′s complex (subaortic stenosis and coarctation of aorta and additional finding of patent ductus arteriosus, as evidenced by the transthoracic echocardiographic report of 2006) diagnosed almost 6 years back [Figure - 3]a and b. Chest examination revealed sternotomy scar and cardiovascular system examination was normal. Dental examination was normal.

Figure 1: Right-sided facial atrophy and alopecia involving frontal area of scalp and eyebrow. This image also shows atrophied ear pinna
Figure 2: Right eye upper lid margin is retracted with partial loss of cilia
Figure 3: Transthoracic echocardiography image showing Shone's complex, (a) Subaortic membrane (b) Coarctation of aorta

Complete hemogram, urine analysis, antinuclear antibody were within normal limits. X-ray chest showed bilateral cervical ribs, right rib being more prominent than left and old sternal sutures. Orthopantogram X-ray, X-ray of skull, CT scan of brain, Electroencephalogram, Electrocardiogram, and Transthoracic echocardiography were normal. A punch biopsy from scalp was taken and a light microscopic evaluation of the hematoxylin and eosin (H and E)-stained histopathological slide showed atrophied epidermis, thick collagen bundles surrounding eccrine glands, and mild mononuclear cell infiltrate in reticular dermis. There was considerable loss of hair follicles and atrophy of appendages. Our clinical and histopathological findings made us keep both Parry-Romberg syndrome and linear localized scleroderma (LSc) "en coup de sabre" as the dermatological differential diagnoses, since both these conditions tend to overlap.

Parry-Romberg Syndrome is a rare disorder characterized by progressive hemifacial atrophy of the skin, adipose tissue, and in some cases involving muscle, cartilage, and underlying bony structures. The incidence and cause of this alteration is unknown. [1] Hyperactivity of the brain stem sympathetic centers, possibly caused by an autoimmune process, may be the primary cause for cutaneous and subcutaneous atrophy in this condition. [2] Trauma, viral infections, endocrine disturbances, and heredity are also believed to be associated with the disease. [1] It begins in childhood, preferentially involves the upper face, and is usually unilateral. It may start with hyper- or hypopigmentation, followed by slowly progressing atrophy which tends to remain limited to the distribution of the trigeminal nerve or may involve the entire face unilaterally or bilaterally. [3] A number of conditions have been reported in association with Parry-Romberg syndrome, [3] e.g., (1) Ocular changes such as loss of eyebrows and eyelashes, iris atrophy, ocular muscle palsies, uveitis, and enophthalmos; (2) Central nervous system disorders like migraine, headaches, hemiparesis, trigeminal neuralgia, and unilateral epilepsy. There may be localized areas of alopecia as seen in our case. The affected side of oral cavity can present with some deficiency in dental root development detectable on X-ray Orthopantogram and also unilateral atrophy of tongue. [3]

Linear LSc "en coup de sabre" is an autoimmune connective tissue disorder which involves the face unilaterally, usually the frontoparietal area, but may extend downward. Most cases begin before 10 years of age. The involved skin is depressed, hard, hyperpigmented, shiny, and devoid of hair. Ocular, neurological, and oral cavity alterations described above may be present with linear LSc "en coup de sabre" as well. [4]

The relationship between Parry-Romberg syndrome and linear LSc "en coup de sabre" is not clearly understood. Tollefson and Witman, [5] found in their study that both entities frequently co-exist and both are likely variants of morphea. However, Orozco-Covarrubias et al. [4] pointed out that in most cases, it is possible to differentiate between scleroderma "en coup de sabre" and Parry-Romberg syndrome. They found that most important clinical feature that differentiated both conditions was "cutaneous sclerosis," absence of which favors diagnosis of Parry-Romberg syndrome. Statistically significant histopathological features were connective tissue fibrosis, adnexal atrophy, and mononuclear cell infiltrate (superficial and deep), which favors a diagnosis of LSc "en coup de sabre."

Shone′s complex is a rare congenital heart disease, described by Shone et al. in 1963. The complete form typically consists of four obstructive lesions namely parachute-like mitral valve, supravalvar mitral ring, subaortic stenosis, and coarctation of aorta. [6] However, incomplete forms with two or three lesions are also described as seen in our case.

Sharma et al. [7] reported coexistence of LSc "en coup de sabre morphea" and aortic regurgitation in a single case of systemic sclerosis. However, association of Parry-Romberg syndrome or LSc "en coup de sabre morphea" with congenital heart disease has not been reported in the literature, and whether it is merely a chance occurrence or an actual association remains to be seen.

References
1.
Da Silva Pinheiro TP, Camarinha-da Silva C, Limeira-da Silveira CS, Ereno-Botelho PC, Rodrigues Pinheiro MG, Viana-Pinheiro JJ. Progressive hemifacial atrophy-Case report. Med Oral Patol Oral Cir Bucal 2006;11:E112-4.
[Google Scholar]
2.
Lonchampt P, Emile J, Pélier-Cady MC, Cadou B, Barthelaix A. Central sympathetic dysregulation and immunological abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease). Clin Auton Res 1995;5:199-204.
[Google Scholar]
3.
Sandhu K, Handa S. Subdural hygroma in a patient with Parry-Romberg Syndrome. Pediatr Dermatol 2004;21:48-50.
[Google Scholar]
4.
Orozco-Covarrubias L, Guzmán-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R. Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 2002;16:361-6.
[Google Scholar]
5.
Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-63.
[Google Scholar]
6.
Narvencar KP, Jaquese-Costa AK, Patil VR. Shone's complex. J Assoc Physicians India 2009;57:415-6.
[Google Scholar]
7.
Sharma YK, Sawhney MP, Srivastava S. Systemic sclerosis, localized morphea, en coup de sabre and aortic regurgitation: A rare association. Indian J Dermatol Venereol Leprol 2004;70:99-101.
[Google Scholar]

Fulltext Views
3,676

PDF downloads
2,936
Show Sections