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:

Letter To Editor
2008:74:5;512-513
doi: 10.4103/0378-6323.44325
PMID: 19052426

Linear and whorled nevoid hypermelanosis

BK Brar, BB Mahajan, Neerja Puri
 Department of Dermatology and Venereology, GGS Medical College and Hospital, Faridkot-151 203, India

Correspondence Address:
B K Brar
C/o Brar Eye Hospital, Kotkopura, Faridkot, Punjab
India
How to cite this article:
Brar B K, Mahajan B B, Puri N. Linear and whorled nevoid hypermelanosis. Indian J Dermatol Venereol Leprol 2008;74:512-513
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Linear and whorled nevoid hypermelanosis is characterized by linear streaks of hyperpigmentation along the Blaschko′s lines. [1] It is also known as ′zebra-like pigmentation′. The pigmentary anomalies become apparent in infancy or early childhood and usually remain stable, but may either increase in extent or fade over time. Histopathology suggests melanocytic hypermelanosis in most cases, but pigmentary incontinence has also been reported.

Striking homologies exist between hypomelanosis of Ito and linear and whorled nevoid hypermelanosis (LWH). In addition, several patients have been reported who display bands of both hypopigmentation and hyperpigmentation, making the distinction between hypomelanosis of Ito and LWH somewhat blurred.

Emerging opinion is that a revised nomenclature should group hypomelanosis of Ito and LWH as a single entity including hypopigmentation or hyperpigmentation along the lines of Blaschko, possible association with extracutaneous anomalies seen in approximately 30% of patients, and somatic mosaicism in a yet undetermined but several different genes involved in human pigmentation.

A 4½ years old female child presented to the department of dermatology with multiple streaks of hyperpigmentation following the lines of Blaschko, all over the body. She was born of a full term vaginal delivery following an uneventful first pregnancy in the 27-year-old mother. The growth and development of the child was normal. Her parents and other family members were normal, and their was no history of consanguinity.

The child remained well for the first 6 weeks of life; but after 6 weeks, the mother noticed dark-colored streaks of linear and whorled pattern developing over the body of the child, involving almost the whole body; the streaks gradually became darker and darker. There was no history of erythema or vesiculobullous lesions. At about 1 year the lesions stabilized in color, and no new lesions developed after that.

Cutaneous examination revealed multiple, brown, hyperpigmented swirls and streaks along the lines of Blaschko, located symmetrically all over the body. The texture of the skin was normal over the streaks. In between the hyperpigmented streaks, the skin was normal. On the abdomen, a whorl type pattern was present; while the legs and arms had linear streaks. On the face, upper eyelids and ears were involved, but palms, soles, eyes, nails, and teeth of the child were normal. Skin biopsy showed diffuse basal cell hyperpigmentation with increase in the number of basal melanocytes. There was no incontinence of pigment, melanophages in dermis or giant melanosomes. The benign nature of the disorder was explained to the patient.

Somatic chromosomal mosaicism may present as isolated pigmentary abnormalities or multiple congenital anomalies with mental retardation. [2],[3],[4]

The differential diagnosis includes incontinentia pigmenti, epidermal nevi, and hypomelanosis of Ito. In incontinentia pigmenti, [5] the skin manifestations pass through four successive stages: vesicular, verrucous, whorl- or streak-like hyperpigmentation, and hypopigmented scars. Skin appendages, including hair and teeth, are commonly affected in incontinentia pigmenti with scarring alopecia and peg-shaped teeth. Lack of the above-mentioned features, absence of basal cell degeneration, incontinence of melanin pigment, tissue eosinophilia, and melanophages in the dermis in the skin histopathology ruled out the possibility of incontinentia pigmenti in the present case.

Epidermal nevi are often noticeable during infancy as hyperpigmented streaks along Blaschko′s line, which become papillomatous and hyperkeratotic with time. Extensive skin involvement is often associated with skeletal, ocular, and nervous system anomalies. In the absence of histopathologic evidence of hyperkeratosis, acanthosis, elongation of rete ridges, papillomatosis, and occasional evidence of vacuolization, the possibility of epidermolytic hyperkeratosis was not entertained.

The scarcity of the reports prompts the present communication; and to the best of our knowledge, this is the second case reported from India.

The importance of this report lies in the fact that this condition is not only rare but also, there are very few cases of LWH reported in the literature till date.

References
1.
Loomis CA. Linear hyperpigmentation including mosaicism. Semin Cutan Med Surg 1997;16:44.
[Google Scholar]
2.
Davis DG, Shaw MW. An unusual human mosaic for skin pigmentation. N Engl J Med 1964;270:1384-9.
[Google Scholar]
3.
Griffths WA. Reticulate pigmentary disorders: A review. Clin Exp Dermatol 1984;9:439-50.
[Google Scholar]
4.
Verghese S, Naevlin A, Miller M, Burton BK. Mosaic trisomy 7 in a patient with pigmentary abnormalities. Am J Med Genet 1999;87:372-4.
[Google Scholar]
5.
Landy SJ, Donnai D. Incontinentia pigmenti (Block-Sulzberger syndrome). J Med Genet 1993;30:53-9.
[Google Scholar]

Fulltext Views
510

PDF downloads
136
Show Sections