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 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 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
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter to the Editor - Case Letter
2015:81:5;518-519
doi: 10.4103/0378-6323.162319
PMID: 26261133

Unilateral cutaneous vasculitis: An uncommon presentation and a possible explanation

Burak Tekin1 , Andac Salman1 , Serhan Tuglular2 , Derya Guler2 , Gulsen Ozen3 , Haner Direskeneli3 , Fatma Gulcicek Ayranci4 , Leyla Cinel4 , Tulin Ergun1
1 Department of Dermatology, School of Medicine, Marmara University, Istanbul, Turkey
2 Department of Nephrology, School of Medicine, Marmara University, Istanbul, Turkey
3 Department of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey
4 Department of Pathology, School of Medicine, Marmara University, Istanbul, Turkey

Correspondence Address:
Burak Tekin
Department of Dermatology, Marmara University Pendik Training and Research Hospital, Mimar Sinan Street, No: 41, Istanbul
Turkey
How to cite this article:
Tekin B, Salman A, Tuglular S, Guler D, Ozen G, Direskeneli H, Ayranci FG, Cinel L, Ergun T. Unilateral cutaneous vasculitis: An uncommon presentation and a possible explanation. Indian J Dermatol Venereol Leprol 2015;81:518-519
Copyright: (C)2015 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

A 41-year-old man with ankylosing spondylitis, familial Mediterranean fever (homozygous M694V mutation) and hypertension was admitted for evaluation of purpuric skin lesions which had appeared on his left ankle 5 days before admission, and rapidly progressed proximally. There was no history of infection or exposure to a new medication as a trigger. His medical history was significant for chronic deep vein thrombosis of the left lower limb diagnosed 3 years ago and treated with oral warfarin. Physical examination showed purpuric papules and plaques covering a large area on the left lower limb, with tense bullae on the lateral aspect of the left leg and severe pitting edema. The right lower limb was normal except for a few dusky macules [Figure - 1]a. On admission, his vital signs were normal except for mild hypertension.

Figure 1: (a) Purpuric papules and plaques with tense bullae on the left lower limb with marked edema (b) Lesions are less pronounced on the 6 th day of treatment

Laboratory work-up showed elevated serum C-reactive protein (32 mg/l; normal: 0-5), creatinine (2 mg/dl; normal: 0-1.2) and proteinuria (705 mg/day; normal: 50-80). Evaluation for hypercoagulability revealed reduced protein S activity (49%, normal: 89-129) and a heterozygote mutation (C677T) of the MTHFR gene. Signs of intraluminal echogenicity and recanalization were noted in the left deep femoral vein on Doppler ultrasound. Findings on a skin biopsy from a purpuric papule were consistent with leukocytoclastic vasculitis [Figure - 2]. Histopathological examination of a kidney biopsy was consistent with renal amyloidosis and did not show vasculitis. The patient was diagnosed as having cutaneous vasculitis, most probably due to familial Mediterranean fever, and treatment with oral methylprednisolone (0.5 mg/kg/day) was commenced. This dose was maintained for 1 week and then tapered over the next 3 weeks. A progressive resolution of the rash was observed [Figure - 1]b.

Figure 2: Vessel wall infiltrated with neutrophils, and leukocytoclasia. Endothelial cells are swollen and neutrophils extend into the perivascular area (H and E, ×400)

Cutaneous small vessel vasculitis classically manifests as palpable purpura due to intense neutrophilic inflammation of the post-capillary venules. Dependent areas of the body such as the lower extremities and sites covered by tight clothing are preferentially involved, indicating the role of hydrostatic pressure and stasis in the pathogenesis. [1] Although symmetrical distribution of lesions is the rule, at least two cases of cutaneous small vessel vasculitis with predominantly unilateral involvement have been reported. In both of these, the relatively spared limb had been kept horizontal due to infection or immobilization, while the more prominently affected limb was in a relatively dependent position. The resulting higher hydrostatic pressure in the latter could explain the asymmetrical involvement. [2],[3]

Familial Mediterranean fever, a hereditary periodic fever syndrome, can influence blood vessels via multiple mechanisms. The association between this disease and the vasculitides can be explained by endothelial cell dysfunction and damage mediated by pro-inflammatory cytokines. Further, an increased incidence of hypercoagulopathy and thrombosis has been observed in familial Mediterranean fever, presumably linked to persistent subclinical inflammation. [4]

Familial Mediterranean fever may have contributed to both the thrombosis and vasculitis in our patient. We hypothesize that the chronic deep vein thrombosis increased the hydrostatic pressure in the left lower limb, leading to unilateral cutaneous vasculitis. This is a presentation clinicians should be aware of, though it remains to be seen if unilateral cutaneous vasculitis can also occur in other conditions affecting local hemodynamics, such as chronic lymphedema or vascular malformations.

References
1.
Shinkai K, Fox LP. Cutaneous vasculitis. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology. 3 rd ed. China: Elsevier; 2012. p. 385-410.
[Google Scholar]
2.
Schattner A, Cohen J. Curious cutaneous vasculitis. Rheumatol Int 2008;28:1281-2.
[Google Scholar]
3.
Gershinsky Y, Levi R, Heyman SN. Unilateral purpuric rash in a patient with acute renal failure. Isr Med Assoc J 2011;13:515.
[Google Scholar]
4.
Aksu K, Keser G. Coexistence of vasculitides with familial Mediterranean fever. Rheumatol Int 2011;31:1263-74.
[Google Scholar]

Fulltext Views
95

PDF downloads
12
Show Sections