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2</a>B&#41;&#46; Fungi and AFB screening using Grocott and Faraco stains&#44; respectively&#44; were negative&#46; Chest and sinuses tomography showed no alterations&#44; as well as whole blood count&#44; renal function&#44; and measurement of complement fractions&#46; As for ANCA&#44; it was positive for a-ANCA 1&#47;20 &#40;negative for c-ANCA and p-ANCA&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Based on the histopathological findings&#44; the diagnosis was granulomatous vasculitis&#46; The term granulomatous vasculitis can be confusing&#44; as it is used in two different contexts&#58; &#40;1&#41; granulomatous inflammation in the vessel wall and &#40;2&#41; extravascular granulomatous inflammation associated with necrotizing vasculitis&#46; The latter&#44; better termed &#8220;vasculitis with granulomatosis&#8221;&#44; has as prototypes granulomatosis with polyangiitis &#40;GPA&#41; and eosinophilic granulomatosis with polyangiitis &#40;EGPA&#41;&#44; which rarely have true granulomatous arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Granulomatous vasculitis is a common finding in the skin lesions of large-vessel vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> In temporal arteritis&#44; there is usually a history of headache and muscle weakness&#44; with the skin being unusually affected&#46; Takayasu&#8217;s arteritis&#44; which mainly affects the aorta and its branches&#44; may also unusually affect the skin&#44; with the histopathological finding of giant cell arteritis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The patient described in the present case report had no symptoms consistent with these conditions&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Rarely&#44; granulomatous arteritis is a cutaneous manifestation of systemic diseases such as sarcoidosis&#44; inflammatory bowel disease&#44; hepatitis C and post-herpes zoster&#46; Moreover&#44; there are cases associated with medications such as montelukast&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> These possibilities were excluded through detailed anamnesis and complementary tests&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">GPA and EGPA are ANCA-associated vasculitis&#44; where histopathological analysis of the skin lesions usually discloses extravascular granulomas and small and medium - size vessels necrotizing vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> More rarely&#44; these vasculitis may present with granulomatous inflammation of the vessel wall and they are associated with systemic involvement&#44; which was not seen in this patient&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Atypical ANCA is characterized on indirect immunofluorescence by concomitant perinuclear and cytoplasmic staining&#44; and it is mainly associated with drug-induced vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Although positive&#44; it was not considered relevant to the clinical context&#44; as&#44; in addition to showing low titers&#44; there was no history of medication or illicit drug use&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Cutaneous polyarteritis nodosa &#40;PAN&#41; manifests histopathologically as necrotizing arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The literature has only one mention of the possibility of this condition manifesting granulomatous inflammation with multinucleated giant cells in the vascular lumen in 2 of 20 cases of PAN&#44; which clinically had the presence of a nodule in 90&#37;&#44; livedo reticularis in 80&#37;&#44; and ulceration in 35&#37; of the cases&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">This case report describes a case of granulomatous vasculitis limited to the skin&#46; Despite extensive research&#44; no causal factor was found&#44; and clinical-laboratory follow-up as an outpatient was chosen&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0045" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#39; contributions</span><p id="par0050" class="elsevierStylePara elsevierViewall">Diego Henrique Morais Silva&#58; Design and planning of the study&#59; drafting and editing of the manuscript&#59; collection&#44; analysis&#44; and interpretation of data&#59; critical review of the literature&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Neusa Yuriko Sakai Valente&#58; Approval of the final version of the manuscript&#59; effective participation in research orientation&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the manuscript&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Agatha Ramos Oppenheimer&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Anna Karoline Gouveia de Oliveira&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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Case Letter
Granulomatous arteritis limited to the skin: case report and etiologic differential diagnosis
Diego Henrique Morais Silva
Autor para correspondência
dhenriquems@gmail.com

Corresponding author.
, Neusa Yuriko Sakai Valente, Agatha Ramos Oppenheimer, Anna Karoline Gouveia de Oliveira
Department of Dermatology, Hospital do Servidor Público Estadual de São Paulo, São Paulo, SP, Brazil
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Detail of the infiltrate&#44; consisting predominantly of epithelioid histiocytes &#40;Hematoxylin &#38; eosin&#44; &#215;400&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Vasculitis constitutes a heterogeneous group of conditions characterized by inflammation in the blood vessel wall&#44; with narrowing or occlusion of the vascular lumen&#46; A 43-year-old man&#44; without comorbidities&#44; had presented multiple erythematous nodules on the anterior surface of the lower limbs for the past ten years&#44; with outbreaks and remissions &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; He had no local or systemic symptoms&#46; A skin biopsy was performed and histopathology disclosed a nodular infiltrate with epithelioid histiocytes and multinucleated giant cells&#44; on the wall and around a medium-caliber vessel&#44; with occlusion of the lumen by a fibrinoid thrombus &#40;<a class="elsevierStyleCrossRefs" href="#fig0010">Figs&#46; 2 and 3</a>&#41;&#46; Verhoeff-van Gieson staining demonstrated the presence of an internal elastic lamina in the wall of the affected vessel &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B&#41;&#46; Fungi and AFB screening using Grocott and Faraco stains&#44; respectively&#44; were negative&#46; Chest and sinuses tomography showed no alterations&#44; as well as whole blood count&#44; renal function&#44; and measurement of complement fractions&#46; As for ANCA&#44; it was positive for a-ANCA 1&#47;20 &#40;negative for c-ANCA and p-ANCA&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Based on the histopathological findings&#44; the diagnosis was granulomatous vasculitis&#46; The term granulomatous vasculitis can be confusing&#44; as it is used in two different contexts&#58; &#40;1&#41; granulomatous inflammation in the vessel wall and &#40;2&#41; extravascular granulomatous inflammation associated with necrotizing vasculitis&#46; The latter&#44; better termed &#8220;vasculitis with granulomatosis&#8221;&#44; has as prototypes granulomatosis with polyangiitis &#40;GPA&#41; and eosinophilic granulomatosis with polyangiitis &#40;EGPA&#41;&#44; which rarely have true granulomatous arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Granulomatous vasculitis is a common finding in the skin lesions of large-vessel vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> In temporal arteritis&#44; there is usually a history of headache and muscle weakness&#44; with the skin being unusually affected&#46; Takayasu&#8217;s arteritis&#44; which mainly affects the aorta and its branches&#44; may also unusually affect the skin&#44; with the histopathological finding of giant cell arteritis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The patient described in the present case report had no symptoms consistent with these conditions&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Rarely&#44; granulomatous arteritis is a cutaneous manifestation of systemic diseases such as sarcoidosis&#44; inflammatory bowel disease&#44; hepatitis C and post-herpes zoster&#46; Moreover&#44; there are cases associated with medications such as montelukast&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> These possibilities were excluded through detailed anamnesis and complementary tests&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">GPA and EGPA are ANCA-associated vasculitis&#44; where histopathological analysis of the skin lesions usually discloses extravascular granulomas and small and medium - size vessels necrotizing vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> More rarely&#44; these vasculitis may present with granulomatous inflammation of the vessel wall and they are associated with systemic involvement&#44; which was not seen in this patient&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Atypical ANCA is characterized on indirect immunofluorescence by concomitant perinuclear and cytoplasmic staining&#44; and it is mainly associated with drug-induced vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Although positive&#44; it was not considered relevant to the clinical context&#44; as&#44; in addition to showing low titers&#44; there was no history of medication or illicit drug use&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Cutaneous polyarteritis nodosa &#40;PAN&#41; manifests histopathologically as necrotizing arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The literature has only one mention of the possibility of this condition manifesting granulomatous inflammation with multinucleated giant cells in the vascular lumen in 2 of 20 cases of PAN&#44; which clinically had the presence of a nodule in 90&#37;&#44; livedo reticularis in 80&#37;&#44; and ulceration in 35&#37; of the cases&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">This case report describes a case of granulomatous vasculitis limited to the skin&#46; Despite extensive research&#44; no causal factor was found&#44; and clinical-laboratory follow-up as an outpatient was chosen&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0045" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#39; contributions</span><p id="par0050" class="elsevierStylePara elsevierViewall">Diego Henrique Morais Silva&#58; Design and planning of the study&#59; drafting and editing of the manuscript&#59; collection&#44; analysis&#44; and interpretation of data&#59; critical review of the literature&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Neusa Yuriko Sakai Valente&#58; Approval of the final version of the manuscript&#59; effective participation in research orientation&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the manuscript&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Agatha Ramos Oppenheimer&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Anna Karoline Gouveia de Oliveira&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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