Cutaneous manifestations of pediatric granulomatosis with polyangiitis: a clinicopathologic and immunopathologic analysis

Journal of the American Academy of Dermatology
Adam C WrightDawn Marie R Davis

Abstract

Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis associated with variable cutaneous manifestations and histopathologic findings. It is less frequent in children than adults and is often positive for cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or proteinase 3-ANCA. We sought to better define and correlate the clinical, histopathologic, and immunopathologic characteristics of cutaneous GPA in pediatric patients. We retrospectively reviewed clinical records and cutaneous histopathologic specimens of patients 17 years or younger with cutaneous manifestations of GPA who were seen at our institution from 1990 to 2013. Of the 52 patients identified with GPA, cutaneous involvement occurred in 36.5% and was the initial manifestation of disease in 7.7%. Of the 19 patients with cutaneous involvement, 26.3% developed acneiform and folliculitis-like papules; 84.2% were cytoplasmic ANCA positive; and 78.9% were proteinase 3-ANCA positive. Histopathologic features included leukocytoclastic vasculitis, granulomatous inflammation, acneiform and perifollicular inflammation, granulomatous vasculitis, and palisading granulomas. Our study was limited because of its retrospective design. Pediatric patients with cutaneo...Continue Reading

References

Feb 1, 1977·Archives of Dermatology·C H HuR K Winkelmann
Mar 15, 1992·Annals of Internal Medicine·G S HoffmanA S Fauci
Jul 1, 1989·Annals of Internal Medicine·B NölleW L Gross
May 1, 1985·The Journal of Pediatrics·S L HallS Hellerstein
Dec 1, 1974·The Laryngoscope·T J McDonaldE G Harrison
Jun 1, 1984·Australian and New Zealand Journal of Medicine·J B HallW R Adam
Aug 1, 1997·Mayo Clinic Proceedings·L E GibsonH O Perry
Sep 1, 1999·Pediatric Dermatology·V BrazzelliG Borroni
Feb 29, 2000·Arthritis and Rheumatism·R A WattsD G Scott
Jan 13, 2001·Annals of the Rheumatic Diseases·R A WattsD G Scott
Nov 26, 2003·International Journal of Dermatology·Lawrence E GibsonHenry Homburger
Jan 31, 2012·Current Rheumatology Reports·Marinka TwiltDavid Cabral
Jun 1, 2012·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Ekaterini SiomouDavid V Milford
Oct 10, 2012·Arthritis and Rheumatism·J C JennetteR A Watts
Sep 11, 2013·Pediatric Dermatology·Jacquelyn DosalElizabeth Alvarez-Connelly

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Citations

Jun 24, 2016·Pediatric Rheumatology Online Journal·Lampros FotisAndrew J White
Sep 7, 2016·Pediatric Dermatology·Allison S ClearyKlaus F Helm
Jul 3, 2020·Clinical Rheumatology·Lampros FotisVassiliki Papaevangelou
May 4, 2018·Rheumatology International·Giovanni GenoveseAngelo Valerio Marzano
May 27, 2020·Journal of Advanced Research·Gaafar RagabXavier Puéchal
Jun 7, 2021·Pediatric Rheumatology Online Journal·Rotem Semo OzLinda Wagner-Weiner

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