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

Translate this page into:
[gtranslate]

Case Report
2002:68:2;105-106
PMID: 17656897

Tuberous xanthomas in type IIA hyperlipoproteinemia

K Krishna Mohan, K Dileep Kumar, BV Ramachandra
 Department of Dermatology, Andhra Medical College, Visakhapatnam, India

Correspondence Address:
B V Ramachandra
Door No. 14 -24-1, Bharathi Hospital, Maharanipeta, Visakhapatnam - 530 002, Andhra Pradesh
India
How to cite this article:
Mohan K K, Kumar K D, Ramachandra B V. Tuberous xanthomas in type IIA hyperlipoproteinemia. Indian J Dermatol Venereol Leprol 2002;68:105-106
Copyright: (C)2002 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

A 4 - year -old obese girl with multiple yellowish, plaques and nodular lesions showed features of tuberous xanthomato in Type IIa hyperlipoproteinemia.
Keywords: Hypercholesterolemia, Xanthoma

Introduction

Xanthomas are commonly caused by a disturbance of lipoprotein metabolism.[1],[2] Tuberous xanthomas present as yellow or reddish nodules located mainly on the extensor surface of the extremities and buttocks. They may be confused with eruptive xanthomas.[3] They indicate a systemic alteration of cholesterol and/or triglyceride metabolism. When they do occur in children and adolescents, a more severe form of hyperlipidemia should be suspected. Prompt diagnosis and treatment may help to prevent side effects such as early coronary artery disease and pancreatitis. Recently Type II a hyperlipoproteinemia was diagnosed in a 4-year-old obese girl who presented with tuberous xanthoma. The presentation was atypical and henceforth being reported.

Case Report

A 4-year-old-obese girl presented with multiple waxy nodules over the skin for the past 2 years. The initial lesions started on the trunk and on the bony prominences and in the skin folds and were persistent, slowly progressive and asymptomatic. Her parents were asymptomatic. The case presented with multiple yellowish plaques and nodular lesions distributed bilaterally and symmetrically over the front and back of the trunk which extended in to the skin folds like bands. Skin was folded on the thighs, wrist, natal cleft and at the interdigital areas which showed similar type of lesions. There was no involvement of palmar creases, arcus juvenalis, lymphadenopathy or organomegaly. No clinical evidence of systemic involvement was observed. A diagnosis of xanthomatosis was considered, and the patient was subjected for investigations. The lipid profile [Figure - 1] and the lipoprotein electrophoresis showed a rise in the LDL cholesterol. Blood sugar, liver function tests, thyroid profile, X - ray chest, ECG. GTT and ultra sound scan abdomen were normal. Biopsy from the nodule revealed infiltration of the dermis with admixture of foam cells, histiocytes and lymphocytes.[4] The lipid profile of her father showed a rise in the LDL cholesterol and triglycerides.

Discussion

The hyperlipoproteinemias which manifest in early childhood are type -1 and type II a.[3] Type I is autosomal recessive and rarest. Familial hypercholesterolemia is a common autosomal dominant disorder affecting approximately one in 500 of general population.The primary defect is due to a reduction in L D L catabolism because of an abnormality in the L D L receptors. Heterozygotes express half the number of LDL receptors and homozygotes have between o and 25%. Homozygotes for familial hypercholesterolemia have markedly elevated cholesterol and LDL at birth. In early years a unique yellowish xanthoma[2] may develop in the interdigital webs of the hands and in the cleft between the buttocks and tuberous xanthomas on the elbows, knees and buttocks. These xanthomas do not appear in the heterozygous adult with familial hypercholesterolemia. The case was presented for its rarity in its clinical presentation. The clinical importance in the present homozygous patient was to foresee a very early development of severe coronary artery disease, and myocardial infarction which can occur in the first and second decades of life.

References
1.
Maher - Wiese VL, Marmer EL, Grant - Kels JM. Xanthomas and inherited hyperlipoproteinemias in children and adolescents. Pediatr Dermatol 1990;7:166.
[Google Scholar]
2.
Parker F. Xanthomas and hyperlipidemias. J Am Acad Dermatol 1985;13:1.
[Google Scholar]
3.
Mallory SB. Infiltrative Diseases. In: Pediatric Dermatology, Edited by Lawrence A. Schanchner, Ronald C, Hansen, Chuchill Livingstone Publishing, New York 1995;859-864.
[Google Scholar]
4.
Bulkley BH, Buja M, Ferrans VJ, et al. Tuberous xanthomas in homozygous Type- II hyperlipoproteinemia: A histologic, histochemical and electron microscopical study. Arch Pathol 1975;99:293-300.
[Google Scholar]
Show Sections