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
2011:77:2;219-221
doi: 10.4103/0378-6323.77477
PMID: 21393965

Degos disease-like presentation in systemic lupus erythematosus

Najeeba Riyaz, Rahima Saleem, Roshni Shafeeq
 Department of Dermatology and Venereology, Medical College, Calicut, India

Correspondence Address:
Najeeba Riyaz
Department of Dermatology and Venereology, Medical College, Calicut
India
How to cite this article:
Riyaz N, Saleem R, Shafeeq R. Degos disease-like presentation in systemic lupus erythematosus. Indian J Dermatol Venereol Leprol 2011;77:219-221
Copyright: (C)2011 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Degos disease (DD) or malignant atrophic papulosis or Kohlmeier-Degos-Delort-Tricort syndrome was first described in 1941 by Kohlmeier, and Robert Degos recognized it as a distinct entity in 1942. [1],[2] This rare disorder is probably an endovasculitis affecting small- and medium-sized arteries leading to tissue infarction mainly in the skin, gastrointestinal system, and central nervous system. It is characterized by porcelain white atrophic papules with infarctive lesions. Variants of DD with only cutaneous involvement and association with collagen vascular disease have been described. [1],[3] Hence, DD is considered as a distinctive reaction pattern rather than a specific disease.[4] We report a patient with the benign cutaneous form of DD whose immunological profile was suggestive of systemic lupus erythematosus (SLE).

A 32-year-old male presented with multiple erythematous papules and nodules mainly over the back of trunk and proximal extremities of 2 weeks duration. These lesions rapidly developed necrosis at the center which eventually healed leaving behind porcelain-white, atrophic scars within a week. He gave history of photosensitivity and erythema over the malar area.

Cutaneous examination revealed a faint malar rash with multiple skin color and erythematous papules and nodules of size 0.5-1 cm over the trunk. Some papules had central necrosis. Multiple round and linear atrophic porcelain white scars were seen over both arms and forearms, some having a rim of erythema [Figure - 1]. Atrophic telangiectatic lesions were seen on the tips of thumb, index, and middle fingers.

Figure 1: Papule with porcelain white atrophic centre

Histopathology from an erythematous papule with central depression revealed dermal edema, mucin deposition and lymphocytic vasculitis consistent with DD [Figure - 2]. A biopsy from a nodule showed basal cell degeneration, interface dermatitis, and periappendageal and perivascular infiltrate suggestive of SLE. The immunological profile revealed positive ANA, RA factor, anti-Sm, anti-u 1 -RNP, anti-SS-A, and antiplatelet antibodies. With these findings we came to a diagnosis of benign cutaneous form of DD with SLE. He was treated with hydroxychloroquine and low dose aspirin.

Figure 2: Dermal edema and mucin deposition with lymphocytic vasculitis, consistent with Degos disease (H and E, ×40)

DD is an uncommon condition characterized by asymptomatic papules which over a few days to weeks develop porcelain white depressed scars with rosy rim partly due to delicate telangiectases. A fatal outcome can occur within 1-2 years due to gastrointestinal and central nervous systems involvement.

Our patient had only cutaneous features suggestive of the benign cutaneous form of DD. Harvell et al have reported cases with only cutaneous involvement. About 4-15% of patients could have a benign course. Our patient also had malar rash, photosensitivity, positive ANA, and anti-Sm antibody, and past history of thrombocytopenia, suggestive of SLE. A review of the literature showed that until 2000, 16 patients with typical lesions of DD seemed to have other diseases concurrent with it, mainly collagen vascular diseases,[4] of which 11 had systemic lupus erythematosus and one each had rheumatoid arthritis, dermatomyositis, progressive systemic sclerosis, drug-induced immunosuppression, and AIDS. Dubin [5] et al described a 29-year-old man with clinical and histological features of DD and immunological features suggestive of SLE similar to our case. Black reported two females with SLE who developed atrophic papules of DD.[1] Histopathology of the early papule had shown abundant mucin which was similar to our finding. A biopsy from another lesion was suggestive of SLE. Doutre et al[3] have also reported similar findings.

According to Ball et al,[4] DD is a distinctive morphologic pattern induced by many different factors, rather than a disease with a single specific cause.

Since our patient had only cutaneous lesions and no systemic involvement, he was treated with hydroxychloroquine.

This case is reported for its exceptionally interesting and rare presentation with a varied immunological profile. Patients with skin lesions typical of DD should be assessed for other associated diseases like SLE, other collagen vascular diseases, and immunosuppressive disorders.

References
1.
Black MM. Malignant atrophic papulosis (Degos disease). Int J Dermatol 1976;5:405-11
[Google Scholar]
2.
Scheinfeld NS. Malignant atrophic papulosis. Clin Exp Dermatol 2007;32:483-7.
[Google Scholar]
3.
Doutre MS, Beylot C, Bioulac P, Busquet M, Conte M. Skin lesions resembling malignant atrophic papulosis in lupus erythematosus. Dermatologica 1987;175:45-6.
[Google Scholar]
4.
Ball E, Newburger A, Ackerman AB. Degos' disease: A distinctive pattern of disease chiefly of lupus erythematosus and not a specific disease per se. Am J Dermatopathol 2003;25:308-20.
[Google Scholar]
5.
Dubin HV, Stawiski MA. Systemic lupus erythematosus resembling malignant atrophic papulosis. Arch Int Med 1974;134:321-3.
[Google Scholar]

Fulltext Views
3,155

PDF downloads
1,444
Show Sections