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Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature

Polyarteritis nodosa is a rare vasculitis of childhood. Cutaneous PAN (cPAN) is limited to the skin, muscles, joints, and peripheral nerves. We describe a 7.5-year-old girl with cPAN presenting initially as massive cervical edema who later went on to develop subcutaneous nodules, livedo reticularis,...

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Autores principales: Bansal, Nina-Karen, Houghton, Kristin Michelle
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3200074/
https://www.ncbi.nlm.nih.gov/pubmed/22046510
http://dx.doi.org/10.1155/2010/687547
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author Bansal, Nina-Karen
Houghton, Kristin Michelle
author_facet Bansal, Nina-Karen
Houghton, Kristin Michelle
author_sort Bansal, Nina-Karen
collection PubMed
description Polyarteritis nodosa is a rare vasculitis of childhood. Cutaneous PAN (cPAN) is limited to the skin, muscles, joints, and peripheral nerves. We describe a 7.5-year-old girl with cPAN presenting initially as massive cervical edema who later went on to develop subcutaneous nodules, livedo reticularis, myositis, arthritis, and mononeuritis multiplex. The use of corticosteroids resulted in initial clinical improvement, but symptom recurrence necessitated disease modifying antirheumatic drugs and biologic therapy. We review a further 119 reports of biopsy proven cPAN in the literature. A majority of patients (96.6%) had cutaneous involvement; musculoskeletal involvement was common and included both articular (58.0%) and muscular (42.9%) symptoms, and nervous system involvement was least common (18.5%). Corticosteroids were used in the majority of patients (85.7%), followed by use of disease modifying antirheumatic drugs (33.0%), nonsteroidal anti-inflammatory drugs (10.7%), and intravenous immunoglobulin (9.8%). Therapy of cPAN with biologics has only been reported in 2 patients, and we report the first patient treated with Rituximab. A diagnosis of cPAN should be considered in a child with fever, vasculitic rash, and musculoskeletal symptoms. Most children respond to corticosteroids and have a benign course, but some require disease modifying antirheumatic drugs and biologic therapies.
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spelling pubmed-32000742011-11-01 Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature Bansal, Nina-Karen Houghton, Kristin Michelle Arthritis Clinical Study Polyarteritis nodosa is a rare vasculitis of childhood. Cutaneous PAN (cPAN) is limited to the skin, muscles, joints, and peripheral nerves. We describe a 7.5-year-old girl with cPAN presenting initially as massive cervical edema who later went on to develop subcutaneous nodules, livedo reticularis, myositis, arthritis, and mononeuritis multiplex. The use of corticosteroids resulted in initial clinical improvement, but symptom recurrence necessitated disease modifying antirheumatic drugs and biologic therapy. We review a further 119 reports of biopsy proven cPAN in the literature. A majority of patients (96.6%) had cutaneous involvement; musculoskeletal involvement was common and included both articular (58.0%) and muscular (42.9%) symptoms, and nervous system involvement was least common (18.5%). Corticosteroids were used in the majority of patients (85.7%), followed by use of disease modifying antirheumatic drugs (33.0%), nonsteroidal anti-inflammatory drugs (10.7%), and intravenous immunoglobulin (9.8%). Therapy of cPAN with biologics has only been reported in 2 patients, and we report the first patient treated with Rituximab. A diagnosis of cPAN should be considered in a child with fever, vasculitic rash, and musculoskeletal symptoms. Most children respond to corticosteroids and have a benign course, but some require disease modifying antirheumatic drugs and biologic therapies. Hindawi Publishing Corporation 2010 2010-11-24 /pmc/articles/PMC3200074/ /pubmed/22046510 http://dx.doi.org/10.1155/2010/687547 Text en Copyright © 2010 N.-K. Bansal and K. M. Houghton. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Clinical Study
Bansal, Nina-Karen
Houghton, Kristin Michelle
Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title_full Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title_fullStr Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title_full_unstemmed Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title_short Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature
title_sort cutaneous polyarteritis nodosa in childhood: a case report and review of the literature
topic Clinical Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3200074/
https://www.ncbi.nlm.nih.gov/pubmed/22046510
http://dx.doi.org/10.1155/2010/687547
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