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nucleolares y homog&#233;neos&#41; y anticuerpos anti-Scl-70 positivos &#40;16<span class="elsevierStyleHsp" style=""></span>U&#47;ml&#59; normalidad<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>10&#41; s&#233;ricos&#46; Los anticuerpos frente a centr&#243;meros&#44; ARN polimerasa <span class="elsevierStyleSmallCaps">iii</span>&#44; SS-A&#44; SS-B&#44; ADN&#44; ds-ADN&#44; cardiolipina y Sm se hallaban dentro de los l&#237;mites normales&#46; Una muestra de biopsia tomada de la frente revel&#243; fibrosis en la capa inferior de la dermis y tejidos subcut&#225;neos&#44; infiltraci&#243;n focal de c&#233;lulas mononucleares en la dermis inferior y tejidos grasos subcut&#225;neos y degeneraci&#243;n hialina &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>D&#41;&#46; Adem&#225;s&#44; se observ&#243; infiltraci&#243;n de c&#233;lulas mononucleares perivasculares en los tejidos musculares &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>E&#41;&#46; Se detectaron c&#233;lulas xantomatosas en la dermis&#44; positivas para CD68&#46; La tinci&#243;n de azul alciano revel&#243; dep&#243;sito de mucina en la dermis&#46; Cabe destacar que se detectaron c&#233;lulas espumosas dentro de los vasos sangu&#237;neos de la dermis inferior &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>G&#41;&#46; El examen mediante inmunofluorescencia directa reflej&#243; un dep&#243;sito lineal de IgM en la membrana basal epid&#233;rmica&#46; Una segunda muestra de biopsia tomada del dedo mostr&#243; haces engrosados de col&#225;geno en la dermis&#46; Una tercera biopsia de la frente reflej&#243; dermis edematosa con haces de col&#225;geno ligeramente engrosados en la dermis inferior&#46; Aunque el examen detallado descart&#243; el compromiso pulmonar&#44; renal y esof&#225;gico&#44; los s&#237;ntomas del paciente cumplieron los criterios de clasificaci&#243;n de esclerosis sist&#233;mica de 2013 propuestos por el <span class="elsevierStyleItalic">American College of Rheumatology&#47;European League against Rheumatism</span><a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a>&#46; Se inici&#243; tratamiento de prednisolona oral &#40;30<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#237;a&#41; junto con administraci&#243;n intravenosa de alprostadil&#46; Se epiteliz&#243; la &#250;lcera 6 meses despu&#233;s&#46; Posteriormente se redujo gradualmente la prednisolona y a su vez se inici&#243; tratamiento con dapsona &#40;50<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#41;&#46; El paciente se halla bien controlado con 8-10<span class="elsevierStyleHsp" style=""></span>mg&#47;d de prednisolona oral&#44; sin recidiva de &#250;lceras&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">El lupus eritematoso profundo &#40;LEP&#41; es un subtipo de LE cut&#225;neo cr&#243;nico con o sin LE sist&#233;mico &#40;LES&#41;&#46; Adem&#225;s&#44; el LEP se da raramente en pacientes con enfermedades del tejido conectivo que no sean LES&#46; La asociaci&#243;n entre LEP y esclerosis sist&#233;mica &#40;ES&#41; es extremadamente rara&#44; habi&#233;ndose reportado pocos casos hasta la fecha<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a>&#46; El caso presente padeci&#243; ES&#44; y el examen detallado descart&#243; LES y enfermedad mixta del tejido conectivo&#46; Sin embargo&#44; fue interesante observar condiciones de solapamiento de esclerodermia y LEP&#46; El paciente desarroll&#243; esclerosis en los brazos&#44; aunque sin compromiso org&#225;nico&#46; Se desarroll&#243; LEP en la frente&#44; el cuero cabelludo y el brazo&#46; Un informe reciente ha revisado 20 casos de paniculitis l&#250;pica en la frente&#44; con implicaci&#243;n de las regiones parietal &#40;70&#37;&#41;&#44; frontal &#40;45&#37;&#41;&#44; temporal &#40;40&#37;&#41;&#44; occipital &#40;30&#37;&#41; y coronal &#40;10&#37;&#41;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a>&#46; Se observaron &#250;lceras en el 10&#37; de los casos&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Hasta la fecha se han reportado pocos casos de LEP ulcerativo<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">4&#44;5</span></a>&#44; 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Cartas Científico Clínica
Lupus eritematoso profundo ulcerativo en un paciente con esclerosis sistémica cutánea limitada
Ulcerative Lupus Erythematosus Profundus in a Patient With Limited Cutaneous Systemic Sclerosis
T. Mori
Corresponding author
mtatsu@fmu.ac.jp

Autor para correspondencia.
, T. Ito, N. Kikuchi, T. Yamamoto
Departamento de Dermatología, Fukushima Medical University, Fukushima, Japón
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nucleolares y homog&#233;neos&#41; y anticuerpos anti-Scl-70 positivos &#40;16<span class="elsevierStyleHsp" style=""></span>U&#47;ml&#59; normalidad<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>10&#41; s&#233;ricos&#46; Los anticuerpos frente a centr&#243;meros&#44; ARN polimerasa <span class="elsevierStyleSmallCaps">iii</span>&#44; SS-A&#44; SS-B&#44; ADN&#44; ds-ADN&#44; cardiolipina y Sm se hallaban dentro de los l&#237;mites normales&#46; Una muestra de biopsia tomada de la frente revel&#243; fibrosis en la capa inferior de la dermis y tejidos subcut&#225;neos&#44; infiltraci&#243;n focal de c&#233;lulas mononucleares en la dermis inferior y tejidos grasos subcut&#225;neos y degeneraci&#243;n hialina &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>D&#41;&#46; Adem&#225;s&#44; se observ&#243; infiltraci&#243;n de c&#233;lulas mononucleares perivasculares en los tejidos musculares &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>E&#41;&#46; Se detectaron c&#233;lulas xantomatosas en la dermis&#44; positivas para CD68&#46; La tinci&#243;n de azul alciano revel&#243; dep&#243;sito de mucina en la dermis&#46; Cabe destacar que se detectaron c&#233;lulas espumosas dentro de los vasos sangu&#237;neos de la dermis inferior &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>G&#41;&#46; El examen mediante inmunofluorescencia directa reflej&#243; un dep&#243;sito lineal de IgM en la membrana basal epid&#233;rmica&#46; Una segunda muestra de biopsia tomada del dedo mostr&#243; haces engrosados de col&#225;geno en la dermis&#46; Una tercera biopsia de la frente reflej&#243; dermis edematosa con haces de col&#225;geno ligeramente engrosados en la dermis inferior&#46; Aunque el examen detallado descart&#243; el compromiso pulmonar&#44; renal y esof&#225;gico&#44; los s&#237;ntomas del paciente cumplieron los criterios de clasificaci&#243;n de esclerosis sist&#233;mica de 2013 propuestos por el <span class="elsevierStyleItalic">American College of Rheumatology&#47;European League against Rheumatism</span><a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a>&#46; Se inici&#243; tratamiento de prednisolona oral &#40;30<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#237;a&#41; junto con administraci&#243;n intravenosa de alprostadil&#46; Se epiteliz&#243; la &#250;lcera 6 meses despu&#233;s&#46; Posteriormente se redujo gradualmente la prednisolona y a su vez se inici&#243; tratamiento con dapsona &#40;50<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#41;&#46; El paciente se halla bien controlado con 8-10<span class="elsevierStyleHsp" style=""></span>mg&#47;d de prednisolona oral&#44; sin recidiva de &#250;lceras&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">El lupus eritematoso profundo &#40;LEP&#41; es un subtipo de LE cut&#225;neo cr&#243;nico con o sin LE sist&#233;mico &#40;LES&#41;&#46; Adem&#225;s&#44; el LEP se da raramente en pacientes con enfermedades del tejido conectivo que no sean LES&#46; La asociaci&#243;n entre LEP y esclerosis sist&#233;mica &#40;ES&#41; es extremadamente rara&#44; habi&#233;ndose reportado pocos casos hasta la fecha<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a>&#46; El caso presente padeci&#243; ES&#44; y el examen detallado descart&#243; LES y enfermedad mixta del tejido conectivo&#46; Sin embargo&#44; fue interesante observar condiciones de solapamiento de esclerodermia y LEP&#46; El paciente desarroll&#243; esclerosis en los brazos&#44; aunque sin compromiso org&#225;nico&#46; Se desarroll&#243; LEP en la frente&#44; el cuero cabelludo y el brazo&#46; Un informe reciente ha revisado 20 casos de paniculitis l&#250;pica en la frente&#44; con implicaci&#243;n de las regiones parietal &#40;70&#37;&#41;&#44; frontal &#40;45&#37;&#41;&#44; temporal &#40;40&#37;&#41;&#44; occipital &#40;30&#37;&#41; y coronal &#40;10&#37;&#41;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a>&#46; Se observaron &#250;lceras en el 10&#37; de los casos&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Hasta la fecha se han reportado pocos casos de LEP ulcerativo<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">4&#44;5</span></a>&#44; habi&#233;ndose sugerido procesos microangiop&#225;ticos tales como necrosis vascular fibrinoide segmentaria&#44; trombosis de peque&#241;os vasos e infiltrados linfocitarios angioc&#233;ntricos densos&#46; Ishiguro et al&#46; sugirieron que las &#250;lceras intratables se produc&#237;an como resultado de los cambios necrobi&#243;ticos en los tejidos subcut&#225;neos causados por cambios vasculares<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a>&#46; Por otro lado&#44; se detect&#243; una reacci&#243;n xantomatosa en el tejido fibr&#243;tico del LEP<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a>&#46; En el presente caso no se observ&#243; vasculitis&#44; pero se detectaron c&#233;lulas espumosas en la dermis y en los vasos sangu&#237;neos&#46; Se sugiri&#243; que las c&#233;lulas xantomatosas estaban inducidas por la fagocitosis de la peroxidaci&#243;n lip&#237;dica causada por los radicales del ox&#237;geno derivados de los macr&#243;fagos&#44; tras la destrucci&#243;n de las gl&#225;ndulas seb&#225;ceas iniciada por el dep&#243;sito de complejos inmunes<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a>&#44; que pudo haber inducido alteraciones circulatorias y tambi&#233;n da&#241;os tisulares&#46; Adem&#225;s&#44; se observaron infiltrados celulares no solo en los tejidos subcut&#225;neos&#44; sino tambi&#233;n en las capas musculares &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a><span class="elsevierStyleHsp" style=""></span>F&#41;&#44; y consecuentemente los da&#241;os tisulares pudieron haber conducido a la ulceraci&#243;n en LEP en este caso&#46;</p></span>"
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Article information
ISSN: 00017310
Original language: Spanish
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