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

Oleh:

Wright, Adam C. ; Gibson, Lawrence E. ; Davis, Dawn Marie R.

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: JAAD: Journal of the American Academy of Dermatology (keterangan: ada di ClinicalKey) vol. 72 no. 05 (May 2015), page 859–867 .
Topik: acneiform lesions; antineutrophil cytoplasmic antibodies; cytoplasmic antineutrophil cytoplasmic antibody; granuloma; granulomatosis with polyangiitis; granulomatous inflammation; palpable purpura; proteinase 3 antineutrophil cytoplasmic antibody; vasculitis; Wegener granulomatosis

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Perpustakaan FK Nomor Panggil: J15.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Background 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. Objective We sought to better define and correlate the clinical, histopathologic, and immunopathologic characteristics of cutaneous GPA in pediatric patients. Methods 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. Results 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. Limitations Our study was limited because of its retrospective design. Conclusion Pediatric patients with cutaneous GPA most commonly have palpable purpura, leukocytoclastic vasculitis, and positive cytoplasmic ANCA or proteinase 3-ANCA serologic results. Cutaneous manifestations and histopathologic findings vary, but acneiform lesions may be a cutaneous manifestation of the disease unique to this age group.

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