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

Translate this page into:

Case Report
2002:68:5;308-309
PMID: 17656984

Unilateral generalized hypertrichosis in proteus syndrome

N Asokan, K Abdul Latheef
 Department of Dermatology and Venereology TrichurMedical College, Trichur 680 596, India

Correspondence Address:
N Asokan
Department of Dermatology and Venereology TrichurMedical College, Trichur 680 596
India
How to cite this article:
Asokan N, Abdul Latheef K. Unilateral generalized hypertrichosis in proteus syndrome. Indian J Dermatol Venereol Leprol 2002;68:308-309
Copyright: (C)2002 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

Ayoung lady was found to have hemihype rtrophy, scoliosis, subcutaneous swellings, patchy dermal hypoplasia, plantar hyperplasia and macrodactyly. These suggested a diagnosis of Proteus syndrome. She was also having unilateral generalized hypertrichosis. The association of Proteus syndrome and unilateral generalized hypertrichosis has not been reported in the literature.
Keywords: Proteus syndrome, Unilateral generalized hypertric

Introduction

Proteus syndrome is a congenital hamartomatous syndrome characterised by a variety of malformations. In 1989 Samalaska et al,[1] identified the commonest anomalies in Proteus syndrome as hemihypertrophy, macrodactyly subcutaneous tumours, plantar or palmar masses, epidermal nevi, exostoses and scoliosis. Happle et al,[2] suggested patchy dermal hypoplasia as another common feature.

The first Indian report of Proteus syndrome was by George and Jacob in 1993.[3] More cases have subsequently been reported.[4],[5] Here we describe a patient with Proteus syndrome who also had unilateral generalized hypertrichosis.

Case Report

A 29 - year -old lady, mother of two children was seen for unilateral overgrowth of hair and asymmetry of the body. She was born of nonconsanguinous marriage of healthy parents. Mild facial asymmetry was noticed from early childhood. Other changes became prominent by puberty. There was no family history of any malformations. Except for a history suggestive of vascular headache and occasional low back ache, she did not have any other symptoms. On examination, the entire right half of the body was moderately hypertrophic, more marked on the limbs. The bulk of the upper limb and lower limb was more on right side by about 0.5 to 2 cm and 2 to 4 cm respectively. The right upper limb was longer by 1.5 cm and right lower limb by 3cm, with a proportional increase of proximal and distal parts. She had scoliosis of upper throracic spine to left side. A subcutaneous swelling suggestive of lipoma was present on right parietal region of scalp. Adjacent area on the left side was depressed and atrophic, suggestive of patchy dermal hypoplasia. There were lipoma - like subcutaneous masses on the right side of anterior abdominal wall. Margins of right heel and plantar, medial and dorsal aspects of right big toe showed a soft tissue hyperplasia with a verrucous appearance [Figure - 1]. This resulted in macrodactyly of the right big toe. Most of the right side of body was covered with thicker, longer and darker terminal hairs [Figure - 2]. Systemic examination and

haematological and biochemical investigations were normal. Radiological and ultrasonological investigations did not reveal any other abnormalities.

Discussion

Though the main concern of our patient was the striking, unilateral hypertrichosis, a careful examination revealed a number of malformations. These consisted of hemihypertrophy, scoliosis, subcutaneous swellings, patchy dermal hypoplasia, plantar hyperplasia and macrodactyly. This combination of findings suggested the diagnosis of Proteus syndrome.

The unique feature of our patient was the unilateral generalized hypertrichosis. The unilateral distribution ruled out hirsutism, endocrine anomalies and other systemic diseases. Nevoid hypertrichosis is typically localized and associated with a hyperpigmented nevus.[6] So it is best considered as a component of Proteus syndrome in this patient. To the best of our knowledge, unilateral generalized hypetrichosis has so far not been reported as a feature of Proteus syndrome.

Happle et al,[2] suggested ′twin spotting′, resulting in hyperplasia and hypoplasia of various tissues as the pathogenetic machanism in Proteus syndrome. Unilateral hypertrichosis in our patient could be due to the involvement of hair follicle structures in this process.

In our patient except for hypertrichosis, most of the features of Proteus syndrome were of a mild to moderate severity. On the other hand, most of the previously reported cases of Proteus syndrome had more prominent features. It could be assumed that many subtle forms of Proteus syndrome do exist and the condition may not be as rare as generally believed.

References
1.
Samalaska CP Levin SW, James WD, et al. Proteus syndrome. Arch Dermatol 1989;125:1109-14.
[Google Scholar]
2.
Happle R, Steijlen PM, Theile U, et al. Patchy dermal hypoplasia as a characteristic feature of Proteus syndrome. Arch Dermotol 1997; 133 :77 - 80.
[Google Scholar]
3.
George R, Jacob M. Proteus syndrome. Indian J Dermatol Venereol Leprol 1993; 59: 213 - 215.
[Google Scholar]
4.
Criton S. Asokan PU, Majeed PKA, Proteus syndrome. Indian J Dermatol Venereol Leprol 1995; 61: 152 - 154.
[Google Scholar]
5.
Mayekar AS, Chavan RG, Phadke VA, et al. Proteus syndrome.lndian J Dermatol Venereal Leprol 1997; 63:44 - 46.
[Google Scholar]
6.
Taskapan O, Dogan B, Cekmen S, et al. Nevoid hypertrichosis associated with duplication of the right thumb, J Am Acad Dermatol 1998; 39: 114 - 115.
[Google Scholar]
Show Sections