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:

Case Report
2001:67:6;334-335
PMID: 17664795

Letterer siwe disease

Anuja E George, K Yogirajan
 Department of Dermato-Venereology, Medical College, Thiruvananthapuram-695011, Kerala, India

Correspondence Address:
Anuja E George
Department of Dermato-Venereology, Medical College, Thiruvananthapuram-695011, Kerala
India
How to cite this article:
George AE, Yogirajan K. Letterer siwe disease. Indian J Dermatol Venereol Leprol 2001;67:334-335
Copyright: (C)2001 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

A 1-year-old boy presented with recurrent pyoderma-like lesions and purulent ear discharge of 6 months duration. The biopsy helped to confirm Letterer Siwe disease. Purpuric lesion on palms and soles which is a reputedly lethal sign of the disease was also present in the child who died within a few days.
Keywords: Purpura, Letterer Siwe disease, Langerhan cell histiocytosis

Introduction

Langerhan cell histiocytosis (LCH) is the name proposed by Risdall et al,[1] in 1983, for a group of disorders previously called Histiocytosis X, where Langerhan cells play a central role in the pathogenesis. Letterer Siwe (LS) disease is an acute, disseminated, multisystem, often fatal type of LCH. The other types are Hand Schuller Christian (HSC) disease which is chronic and disseminated; eosinophilic granuloma which is chronic, benign and solitary and the mixed type.[2]

Case Report

A 1-year-old boy was admitted with history of recurrent pyoderma-like lesions especially over the scalp, face, palms and soles with purulent discharge from both ears since the age of 4 months. There was history of high fever with cough and dyspnoea at the age of 7 months, but there was never polyuria or polydypsia.

He was of moderate build and nourishment, was toxic, febrile and anaemic with pedal oedema. There was erythema, fissuring, scaling and crusting with vesicles and pustules over scalp; face especially over the forehead, periocular and retroauricuar areas [Figure - 1]; palms; soles; neck; groin folds and trunk. The palms and soles in addition had extensive haemorrhagic spots [Figure - 2] and there was a congenital haemangioma on the right palm. Multiple post inflammatory hypopigmented scars were present on the trunk; the teeth and nails were normal. There was also generalised lymphadenopathy, alopecia, blepharoc-onjunctivitis, oral candidiasis and bilateral purulent foul smelling ear discharge with a CSOM on the left side. There was gross hepatosplenomegaly but other systems were within normal limits.

He was born as the second child of a non-consanguinous marriage, was fully immunised for age and the developmental milestones were normal. The family history was noncontributory.

With a clinical diagnosis of Histiocytosis-X, the following investigations were done: Urine-normal; blood-Hb: 6gms%,TC: 7200/cmm, P-71 L-25 E-4, ESR: 104mm/first hour; Blood picture-normocytic hypochromic anaemia, platelets scattered and reduced in number. Gram stain of smear from intact pustule-sterile; culture of pus showed contaminant growth of Staph aureus. Elisa for HIV-negative, VDRL-nonreactive. X-rays of chest (PA) and skull (lateral) were normal. Skin biopsy showed extensive infiltration of dermis by histiocytes mainly in the upper zone and invading into the epidermis. A few atypical histiocytes with hyperchromatic nuclei and extravasated erythrocytes were also seen in the infiltrate.

The child was started on antipyretics, systemic antibiotics and topical agents. He developed acute abdominal distension and expired within a few days.

Discussion

In Letterer Siwe disease recurrent pyoderma -like lesions with crusting and scaling, vesicopustular and purpuric eruption occurs in crops over the face, scalpfand trunk, resembling seborrhoeic dermatitis. Nodular lesions in body flexures and intraoral haemorrhage, gingivitis, loose teeth, precocious eruption of teeth and ectopic teeth also may be seen.[3] The pustules are sterile and Tzanck smear of the vesicopustules shows pale histiocytes which can be used as a rapid screening test. Skin biopsy in LS disease shows a proliferative reaction with extensive upper dermal infiltration and epidermal invasion with histiocytes, some of which are atypical, along with erythrocytes.

This case had typical skin lesions and the biopsy confirmed LS disease. He also had recurrent otitis media which is the most frequent clinical symptom of HSC disease, thus suggesting that there can be an overlap of the two diseases, being manifestations of the same disease process. However, there was no polyuria or polydypsia characteristic of ′ HSC disease. Oral candidiasis may have been due to immune deficiency due to the repeated and long term therapy with antibiotics and steroids.

Chemotherapy coupled with radiation to localised bony lesions and supportive measures are useful in treatment, there being no unified therapeutic measure.[4] Thymic extracts have been successfully used to treat LS disease.[5]

Poor prognostic features include younger age; dissemination of lesions; involvement of lungs, liver, CNS and RE system; associated infection; anaemia, thrombocytopenia and purpura.[6] The child had extensive purpuric lesions on palms and soles which is a reputedly lethal sign,[6] as well as hepatosplenomegaly, thrombocytopenia and severe anaemia. He died within a few days proving again that the presence of purpura in LS disease is a lethal marker.

References
1.
Risdall AJ, Dehner LP, Duray Pet al. Histiocytosis X (LC histiocytosis) Prognostic role of histopathology. Arch Pathol Lab Med 1983; 107-109.
[Google Scholar]
2.
Berry DH, Becton DL. Natural history of Histiocytosis X. Hematol Oncol Clin North Am 1987;1 :23.
[Google Scholar]
3.
Hartmann KS. Histiocytosis X. A review of 114 cases with oral involvement. Oral Surg, Oral Med, Oral Pathol 1980;49: 38.
[Google Scholar]
4.
McClelland J, Pritchard J, Chu AC. Current controversies. Hematol Oncol. Clin North Am 1987;1:147.
[Google Scholar]
5.
Osband ME, Lipton JM, Lavin Pet al. Histiocytosis X: Demonstration of abnormal immunity, T-cell histamin H2 receptor deficiency and successful treatment with thymic extract.N Eng 3 Med 1981;304:146
[Google Scholar]
6.
Nezelof C, Frileux - herbet F Cronier-Sachot J. Disseminated histiocytosis X: Analysis of Analysis of prognostic factors based on a retrospective study of 50 casecs. Cancer 1979; 44: 1824.
[Google Scholar]

Fulltext Views
7,923

PDF downloads
1,537
Show Sections