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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a chronic&#44; recurrent and painful skin disease that usually affects the lower limbs&#46; It was first described by Bard and Winkelmann in 1967&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> as a vaso-occlusive disorder affecting the small vessels of the dermis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of an 82-year-old woman under dermatological follow-up for bullous pemphigoid&#44; which was stable on low doses of prednisone&#46; Of interest in her personal history was a prior diagnosis of primary Sj&#246;gren&#39;s syndrome with positive anti-Ro antibodies&#44; meeting the diagnostic criteria set established by Vitali et al&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">During a check-up visit&#44; coinciding with more xerophthalmia and xerostomia than usual&#44; reticulated erythematous macules of livedoid appearance together with painful ulcerated nodules and other areas of atrophie blanche were observed on both lower limbs bilaterally and symmetrically &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Due to a clinical suspicion of livedoid vasculopathy&#44; a diagnostic biopsy was undertaken showing thickening and hyalinisation of the vessel walls&#44; with no inflammatory component&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A complete blood test highlighted&#58; anaemia with a haemoglobin of 10&#46;4<span class="elsevierStyleHsp" style=""></span>g&#47;dl&#44; positive ANA 1&#47;1280 with speckled cytoplasmic patterns&#44; positive anti-SSA&#47;Ro antibodies &#40;&#62;240&#46;0<span class="elsevierStyleHsp" style=""></span>U&#47;ml&#41;&#44; reduced C3 and C4 &#40;43 and 3<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; respectively&#41;&#46; The remaining parameters showed no anomalies&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Therefore&#44; given the characteristic clinical skin symptoms and compatible histological findings&#44; the diagnosis of livedoid vasculopathy was confirmed&#44; and possible haematological alterations that would explain a prothrombotic condition were ruled out&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with pentoxifylline 400<span class="elsevierStyleHsp" style=""></span>mg every 8<span class="elsevierStyleHsp" style=""></span>h&#44; with 100<span class="elsevierStyleHsp" style=""></span>mg of aspirin daily&#44; achieving a good response and gradual resolution of the lesions&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a rare&#44; chronic and painful disease&#44; characterised by the presence of macules or purpuric papules and plaques with a tendency to form irregular ulcers that develop into star-shaped atrophic scars and peripheral hyperpigmentation&#44; described as atrophie blanche&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> It usually affects the lower limbs&#44; with a bilateral&#44; symmetrical distribution&#46; It is characteristically&#44; although not always&#44; associated with livedo reticularis&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">It can manifest at any time of life&#44; and is more frequent in young women&#44; with a 3&#58;1 ratio over males&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> However&#44; our patient was older than the average&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The most frequent histopathological finding is hyalinising vascular changes of the inner layers of the dermal vessels&#44; generally with little inflammation&#44; together with thrombosis inside the blood vessels&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> These signs enable the diagnosis to be confirmed&#44; and other processes that present with similar skin symptoms to be ruled out&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The condition&#39;s aetiopathogenesis remains unknown&#46; However&#44; the presence of thrombophilic alterations is considered increasingly relevant&#44; and complementary tests are needed to rule out prothrombotic conditions&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In turn&#44; it has been related to systemic diseases&#44; such as scleroderma&#44; systemic lupus erythematosus&#44; rheumatoid arthritis&#44; cryoglobulinaemia&#44; and mixed connective tissue disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">6&#44;7</span></a> However&#44; only one case relating to Sj&#246;gren&#39;s syndrome<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> has been published&#44; and its simultaneous coexistence with two autoimmune conditions has not been recorded to date&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">To conclude&#44; we present a case of livedoid vasculopathy in a patient with bullous pemphigoid and primary Sj&#246;gren&#39;s syndrome with positive anti-Ro antibodies and complement consumption&#46; The co-existence of this disease with both autoimmune conditions reinforces the hypothesis that there is an association with systemic diseases that can be aggressive to the endothelium&#44; triggering the onset of this vaso-occlusive disease&#46; However&#44; studies with a larger number of patients are necessary to obtain definitive results&#46;</p></span>"
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Letter to the Editor
Livedoid vasculopathy in a patient with bullous pemphigoid and primary Sjögren's syndrome
Vasculopatía livedoide en una paciente con penfigoide ampolloso y síndrome de Sjögren primario
María Isabel García Briz
Corresponding author
mariwelchy@gmail.com

Corresponding author.
, Amelia Prats Máñez, Ramón García Ruiz, Almudena Mateu Puchades
Servicio de Dermatología, Hospital Universitario Doctor Peset, Valencia, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a chronic&#44; recurrent and painful skin disease that usually affects the lower limbs&#46; It was first described by Bard and Winkelmann in 1967&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> as a vaso-occlusive disorder affecting the small vessels of the dermis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of an 82-year-old woman under dermatological follow-up for bullous pemphigoid&#44; which was stable on low doses of prednisone&#46; Of interest in her personal history was a prior diagnosis of primary Sj&#246;gren&#39;s syndrome with positive anti-Ro antibodies&#44; meeting the diagnostic criteria set established by Vitali et al&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">During a check-up visit&#44; coinciding with more xerophthalmia and xerostomia than usual&#44; reticulated erythematous macules of livedoid appearance together with painful ulcerated nodules and other areas of atrophie blanche were observed on both lower limbs bilaterally and symmetrically &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Due to a clinical suspicion of livedoid vasculopathy&#44; a diagnostic biopsy was undertaken showing thickening and hyalinisation of the vessel walls&#44; with no inflammatory component&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A complete blood test highlighted&#58; anaemia with a haemoglobin of 10&#46;4<span class="elsevierStyleHsp" style=""></span>g&#47;dl&#44; positive ANA 1&#47;1280 with speckled cytoplasmic patterns&#44; positive anti-SSA&#47;Ro antibodies &#40;&#62;240&#46;0<span class="elsevierStyleHsp" style=""></span>U&#47;ml&#41;&#44; reduced C3 and C4 &#40;43 and 3<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; respectively&#41;&#46; The remaining parameters showed no anomalies&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Therefore&#44; given the characteristic clinical skin symptoms and compatible histological findings&#44; the diagnosis of livedoid vasculopathy was confirmed&#44; and possible haematological alterations that would explain a prothrombotic condition were ruled out&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with pentoxifylline 400<span class="elsevierStyleHsp" style=""></span>mg every 8<span class="elsevierStyleHsp" style=""></span>h&#44; with 100<span class="elsevierStyleHsp" style=""></span>mg of aspirin daily&#44; achieving a good response and gradual resolution of the lesions&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a rare&#44; chronic and painful disease&#44; characterised by the presence of macules or purpuric papules and plaques with a tendency to form irregular ulcers that develop into star-shaped atrophic scars and peripheral hyperpigmentation&#44; described as atrophie blanche&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> It usually affects the lower limbs&#44; with a bilateral&#44; symmetrical distribution&#46; It is characteristically&#44; although not always&#44; associated with livedo reticularis&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">It can manifest at any time of life&#44; and is more frequent in young women&#44; with a 3&#58;1 ratio over males&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> However&#44; our patient was older than the average&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The most frequent histopathological finding is hyalinising vascular changes of the inner layers of the dermal vessels&#44; generally with little inflammation&#44; together with thrombosis inside the blood vessels&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> These signs enable the diagnosis to be confirmed&#44; and other processes that present with similar skin symptoms to be ruled out&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The condition&#39;s aetiopathogenesis remains unknown&#46; However&#44; the presence of thrombophilic alterations is considered increasingly relevant&#44; and complementary tests are needed to rule out prothrombotic conditions&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In turn&#44; it has been related to systemic diseases&#44; such as scleroderma&#44; systemic lupus erythematosus&#44; rheumatoid arthritis&#44; cryoglobulinaemia&#44; and mixed connective tissue disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">6&#44;7</span></a> However&#44; only one case relating to Sj&#246;gren&#39;s syndrome<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> has been published&#44; and its simultaneous coexistence with two autoimmune conditions has not been recorded to date&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">To conclude&#44; we present a case of livedoid vasculopathy in a patient with bullous pemphigoid and primary Sj&#246;gren&#39;s syndrome with positive anti-Ro antibodies and complement consumption&#46; The co-existence of this disease with both autoimmune conditions reinforces the hypothesis that there is an association with systemic diseases that can be aggressive to the endothelium&#44; triggering the onset of this vaso-occlusive disease&#46; However&#44; studies with a larger number of patients are necessary to obtain definitive results&#46;</p></span>"
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