Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease process where the exact pathogenesis is not known. It is an independent immunological condition affecting multiple organ systems. Major manifestations include chronic urticarial vasculitis (UV) with complement defi...

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Main Authors: Ankit Agrawal, Sangeetha Pabolu, Shirisha Ale, Yesenia Galan
Format: Article
Language:English
Published: SAGE Publishing 2019-01-01
Series:Indian Journal of Rheumatology
Subjects:
Online Access:http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=4;spage=317;epage=320;aulast=Agrawal
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author Ankit Agrawal
Sangeetha Pabolu
Shirisha Ale
Yesenia Galan
author_facet Ankit Agrawal
Sangeetha Pabolu
Shirisha Ale
Yesenia Galan
author_sort Ankit Agrawal
collection DOAJ
description Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease process where the exact pathogenesis is not known. It is an independent immunological condition affecting multiple organ systems. Major manifestations include chronic urticarial vasculitis (UV) with complement deficiency and demonstration of serum C1q antibodies. We describe a case of a 60-year-old male who presented with a generalized rash and was eventually diagnosed with HUVS. Usually, HUVS and systemic lupus erythematosus (SLE) have overlapping features, and our case was unique as our patient did not have any SLE manifestations. This article emphasizes the importance of comprehensive review of systemic manifestations accompanying UV. Although rare, it raises the awareness among the physicians about its clinical presentation and diagnostic approach and also predicts the prognosis of the same.
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institution Kabale University
issn 0973-3698
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language English
publishDate 2019-01-01
publisher SAGE Publishing
record_format Article
series Indian Journal of Rheumatology
spelling doaj-art-53f1db1ab4d14f1a8e55d544de5eef4c2025-02-03T10:23:16ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012019-01-0114431732010.4103/injr.injr_65_19Hypocomplementemic urticarial vasculitis syndrome: A disease with many facesAnkit AgrawalSangeetha PaboluShirisha AleYesenia GalanHypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease process where the exact pathogenesis is not known. It is an independent immunological condition affecting multiple organ systems. Major manifestations include chronic urticarial vasculitis (UV) with complement deficiency and demonstration of serum C1q antibodies. We describe a case of a 60-year-old male who presented with a generalized rash and was eventually diagnosed with HUVS. Usually, HUVS and systemic lupus erythematosus (SLE) have overlapping features, and our case was unique as our patient did not have any SLE manifestations. This article emphasizes the importance of comprehensive review of systemic manifestations accompanying UV. Although rare, it raises the awareness among the physicians about its clinical presentation and diagnostic approach and also predicts the prognosis of the same.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=4;spage=317;epage=320;aulast=Agrawalautoimmunityhypocomplementemiamcduffie syndromeurticarial vasculitis
spellingShingle Ankit Agrawal
Sangeetha Pabolu
Shirisha Ale
Yesenia Galan
Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
Indian Journal of Rheumatology
autoimmunity
hypocomplementemia
mcduffie syndrome
urticarial vasculitis
title Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
title_full Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
title_fullStr Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
title_full_unstemmed Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
title_short Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
title_sort hypocomplementemic urticarial vasculitis syndrome a disease with many faces
topic autoimmunity
hypocomplementemia
mcduffie syndrome
urticarial vasculitis
url http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=4;spage=317;epage=320;aulast=Agrawal
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AT sangeethapabolu hypocomplementemicurticarialvasculitissyndromeadiseasewithmanyfaces
AT shirishaale hypocomplementemicurticarialvasculitissyndromeadiseasewithmanyfaces
AT yeseniagalan hypocomplementemicurticarialvasculitissyndromeadiseasewithmanyfaces