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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Macular arteritis was first described in 2003 by Fein et al&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Since then&#44; fewer than 15 cases have been reported&#46; The disease mainly affects women with a mean age of 40 years<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and usually presents as vaguely oval and pigmented macules on the legs&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Macular arteritis is confirmed by microscopy&#44; which shows lymphocytic vasculitis selectively affecting the arterioles at the dermal subcutaneous junction&#46; Macular arteritis is also known as lymphocytic thrombophilic arteritis because of the induction of endothelial swelling&#44; luminal stenosis&#44; and&#44; occasionally&#44; concentric fibrin deposition&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> Laboratory analyses reveal antinuclear antibodies in 30&#37; of cases and anticardiolipin antibodies in up to 60&#37;&#44; although no apparent correlation with other manifestations of antiphospholid syndrome has been found&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a 61-year-old woman with no past history of interest who consulted for slowly progressive pigmented macules with poorly defined borders that had begun to appear 3 years earlier &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Biopsy revealed lymphocytic vasculitis selectively affecting the arterioles at the dermal subcutaneous junction &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; with very noticeable lymphocytic cuffing&#44; marked endothelial swelling&#44; and narrowing of the vascular lumen&#44; in which concentric fibrin deposition was visible &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The workup comprised a complete coagulation study&#44; serology for autoimmune diseases&#44; hepatotropic viruses&#44; human immunodeficiency virus&#44; and syphilis&#44; erythrocyte sedimentation rate&#44; antineutrophilic cytoplasmic antibody&#44; and protein electrophoresis&#44; all of which yielded normal or negative results&#46; These findings led us to make a diagnosis of macular arteritis&#46; The lesions stabilized or abated very slightly without treatment during the summer months&#44; and no other cutaneous or systemic manifestations were observed after 1 year of follow-up&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Macular arteritis may be considered a minor form of antiphospholipid syndrome&#44; although its only aspect in common with this condition is the occasional slight increase in anticardiolipin antibody titer&#44; since the patient does not present the characteristic manifestations of thrombophilia or underlying thrombotic vasculopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It seems that increased anticardiolipin antibody titer is associated with the vascular insult&#44; although it has also been recorded in 7&#37; of healthy patients and after episodes of infection&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Potentially associated conditions include thrombophilic and lymphocytic vasculitis with systemic involvement&#44; such as Degos disease and livedo vasculitis&#44; or Sneddon syndrome<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a>&#59; however&#44; none of the reported cases of macular arteritis involved extracutaneous manifestations&#44; and the lesions observed in this condition are completely different from the porcelain-like atrophic or livedoid lesions that are characteristic of the first 2 conditions&#46; The fibrin ring observed under microscopy seems to be a focal phenomenon<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and is in no way comparable to the intense multisystem thrombosis observed in Degos disease and Sneddon syndrome&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Differentiation of macular arteritis from cutaneous polyarteritis nodosa &#40;PAN&#41; is more complicated&#46; Despite its name&#44; cutaneous PAN does not include the multisystem manifestations of classic or microscopic PAN&#46; In addition&#44; the disease selectively affects the arterioles of the dermal subcutaneous junction&#44; as was the case in our patient&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> There are characteristic features that help to differentiate macular arteritis from cutaneous PAN&#44; namely&#44; the clinical manifestations&#44; with the presence of livedo reticularis&#44; nodules&#44; and ulceration in PAN&#46; The changes in the microscopic findings over time also appear to differ between macular arteritis and PAN&#46; The initial infiltrate in PAN has abundant polymorphonuclear cells&#44; but this changes to a lymphohistiocytic infiltrate&#44; and there are also differences in the sites of the lesions&#44; which are localized to the vascular bifurcations in PAN&#44; with intense focal necrosis that can manifest as a rosette pattern with the formation of microaneurysms&#46; None of these findings have been reported in macular arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However&#44; there have been cases reported as lymphocytic thrombophilic arteritis that&#44; despite presenting clinical features &#40;livedo rash&#41; and histological features &#40;residual nuclear dust together with a lymphohistiocytic infiltrate and a vascular rosette pattern&#41; of PAN&#44; were very probably genuine cutaneous PAN&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In the case we report&#44; the cutaneous manifestation&#44; namely&#44; pigmented macules on the thighs&#44; suggests a diagnosis of macular arteritis&#46; These lesions are characteristic of macular arteritis and clearly differ from the habitual manifestations of PAN&#46; In addition&#44; histopathology in our patient revealed the absence of an acute phase with abundant polymorphonuclear cells&#46; It is questionable whether macular arteritis has sufficient defining features to be considered a separate entity from cutaneous PAN&#59; however&#44; given our currently limited knowledge of the pathogenic and immunologic mechanisms underlying these conditions&#44; we cannot rule out the possibility that macular arteritis is at one end of the spectrum<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> that includes systemic PAN as its most relevant disease&#46; The range of potential manifestations is affected by immune complex size&#44; localization to venous&#47;arterial endothelium&#44; selectivity for skin or other organs&#44; chemotactic potential for different types of cells&#44; and persistence of the antigen &#40;eg&#44; drugs&#44; hepatitis B or C virus&#44; and neoplasms&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> as well as the ability to eliminate it&#46;</p></span>"
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Case and Research Letters
Macular Arteritis: A Pole of the Spectrum of Cutaneous Polyarteritis Nodosa?
Arteritis macular: ¿en el espectro de la poliarteritis nudosa cutánea?
R. Valverdea,
Corresponding author
, C. Garridoa, V. Leisa, E. Ruiz-Bravob
a Sección de Dermatología, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Universitario La Paz, Madrid, Spain
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of cases and anticardiolipin antibodies in up to 60&#37;&#44; although no apparent correlation with other manifestations of antiphospholid syndrome has been found&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a 61-year-old woman with no past history of interest who consulted for slowly progressive pigmented macules with poorly defined borders that had begun to appear 3 years earlier &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Biopsy revealed lymphocytic vasculitis selectively affecting the arterioles at the dermal subcutaneous junction &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; with very noticeable lymphocytic cuffing&#44; marked endothelial swelling&#44; and narrowing of the vascular lumen&#44; in which concentric fibrin deposition was visible &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The workup comprised a complete coagulation study&#44; serology for autoimmune diseases&#44; hepatotropic viruses&#44; human immunodeficiency virus&#44; and syphilis&#44; erythrocyte sedimentation rate&#44; antineutrophilic cytoplasmic antibody&#44; and protein electrophoresis&#44; all of which yielded normal or negative results&#46; These findings led us to make a diagnosis of macular arteritis&#46; The lesions stabilized or abated very slightly without treatment during the summer months&#44; and no other cutaneous or systemic manifestations were observed after 1 year of follow-up&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Macular arteritis may be considered a minor form of antiphospholipid syndrome&#44; although its only aspect in common with this condition is the occasional slight increase in anticardiolipin antibody titer&#44; since the patient does not present the characteristic manifestations of thrombophilia or underlying thrombotic vasculopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It seems that increased anticardiolipin antibody titer is associated with the vascular insult&#44; although it has also been recorded in 7&#37; of healthy patients and after episodes of infection&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Potentially associated conditions include thrombophilic and lymphocytic vasculitis with systemic involvement&#44; such as Degos disease and livedo vasculitis&#44; or Sneddon syndrome<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a>&#59; however&#44; none of the reported cases of macular arteritis involved extracutaneous manifestations&#44; and the lesions observed in this condition are completely different from the porcelain-like atrophic or livedoid lesions that are characteristic of the first 2 conditions&#46; The fibrin ring observed under microscopy seems to be a focal phenomenon<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and is in no way comparable to the intense multisystem thrombosis observed in Degos disease and Sneddon syndrome&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Differentiation of macular arteritis from cutaneous polyarteritis nodosa &#40;PAN&#41; is more complicated&#46; Despite its name&#44; cutaneous PAN does not include the multisystem manifestations of classic or microscopic PAN&#46; In addition&#44; the disease selectively affects the arterioles of the dermal subcutaneous junction&#44; as was the case in our patient&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> There are characteristic features that help to differentiate macular arteritis from cutaneous PAN&#44; namely&#44; the clinical manifestations&#44; with the presence of livedo reticularis&#44; nodules&#44; and ulceration in PAN&#46; The changes in the microscopic findings over time also appear to differ between macular arteritis and PAN&#46; The initial infiltrate in PAN has abundant polymorphonuclear cells&#44; but this changes to a lymphohistiocytic infiltrate&#44; and there are also differences in the sites of the lesions&#44; which are localized to the vascular bifurcations in PAN&#44; with intense focal necrosis that can manifest as a rosette pattern with the formation of microaneurysms&#46; None of these findings have been reported in macular arteritis&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However&#44; there have been cases reported as lymphocytic thrombophilic arteritis that&#44; despite presenting clinical features &#40;livedo rash&#41; and histological features &#40;residual nuclear dust together with a lymphohistiocytic infiltrate and a vascular rosette pattern&#41; of PAN&#44; were very probably genuine cutaneous PAN&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In the case we report&#44; the cutaneous manifestation&#44; namely&#44; pigmented macules on the thighs&#44; suggests a diagnosis of macular arteritis&#46; These lesions are characteristic of macular arteritis and clearly differ from the habitual manifestations of PAN&#46; In addition&#44; histopathology in our patient revealed the absence of an acute phase with abundant polymorphonuclear cells&#46; It is questionable whether macular arteritis has sufficient defining features to be considered a separate entity from cutaneous PAN&#59; however&#44; given our currently limited knowledge of the pathogenic and immunologic mechanisms underlying these conditions&#44; we cannot rule out the possibility that macular arteritis is at one end of the spectrum<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> that includes systemic PAN as its most relevant disease&#46; The range of potential manifestations is affected by immune complex size&#44; localization to venous&#47;arterial endothelium&#44; selectivity for skin or other organs&#44; chemotactic potential for different types of cells&#44; and persistence of the antigen &#40;eg&#44; drugs&#44; hepatitis B or C virus&#44; and neoplasms&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> as well as the ability to eliminate it&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Valverde R&#44; et al&#46; Arteritis macular&#58; &#191;en el espectro de la poliarteritis nudosa cut&#225;nea&#63; Actas Dermosifiliogr&#46; 2013&#59;104&#58;263&#8211;5&#46;</p>"
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ISSN: 15782190
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