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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 24-year-old male was referred to our hospital&#44; complaining of recalcitrant ulcers on the forehead and scalp&#46; Physical examination showed well-demarcated ulceration and depressed scars on the forehead&#44; scalp and upper arm &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#44; B&#41;&#46; In addition&#44; he had swollen and cold fingers with ulceration of the tip of the third finger with nail fold extension &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41;&#46; He noticed Raynaud&#8217;s phenomenon and morning stiffness one year previously&#46; Antinuclear antibodies &#40;1&#58;640&#44; nucleolar and homogeneous&#41;&#44; and positive anti-Scl-70 antibodies &#40;16 U&#47;mL&#59; normal &#60;10&#41; were detected in the serum&#44; whereas antibodies against centromere&#44; RNA polymerase III&#44; SS-A&#44; SS-B&#44; DNA&#44; ds-DNA&#44; cardiolipin&#44; and Sm were all within normal limits&#46; A biopsy specimen taken from the forehead revealed fibrosis of the lower dermis and subcutaneous tissues&#44; focal infiltration of mononuclear cells in the lower dermis and subcutaneous fat tissues&#44; and hyaline fat degeneration &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>D&#41;&#46; Furthermore&#44; perivascular mononuclear cell infiltration was observed in the muscle tissues &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>E&#41;&#46; Xanthomatous cells were detected in the dermis&#44; which were positive for CD68&#46; Alcian blue stain revealed mucin deposition in the dermis&#46; Of note&#44; foam cells were detected within the blood vessels in the lower dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>G&#41;&#46; Examination by direct immunofluorescence showed linear deposition of IgM in the epidermal basement membrane&#46; A second biopsy specimen from the finger showed thickened collagen bundles in the dermis&#46; A third biopsy from the forearm showed edematous dermis with slightly thickened collagen bundles in the lower dermis&#46; Although detailed examination denied pulmonary&#44; renal and esophageal involvement&#44; the symptoms of the patient fulfilled the 2013 classification criteria of systemic sclerosis proposed by American College of Rheumatology&#47;European League against Rheumatism&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Oral prednisolone &#40;PSL&#41; &#40;30&#160;mg&#47;day&#41; was started along with intravenous administration of alprostadil&#46; The ulcer was epithelialized six months later&#46; Thereafter&#44; PSL was gradually tapered&#44; and in turn&#44; dapson &#40;50&#160;mg&#47;d&#41; was started&#46; He is well-controlled under 8-10&#160;mg&#47;d oral PSL&#44; without recurrence of ulcers&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Lupus erythematosus profundus &#40;LEP&#41; is a subtype of chronic cutaneous LE with or without systemic lupus erythematosus &#40;SLE&#41;&#46; Additionally&#44; LEP is rarely seen in patients with connective tissue diseases other than SLE&#46; Association between LEP and systemic sclerosis &#40;SSc&#41; is extremely rare&#44; and only a few cases have been reported to date&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Association with LEP and SSc is extremely rare&#44; and thus the etiology of the present case is unclear&#46; The present case suffered from SSc&#44; and detailed examination denied SLE and mixed connective tissue disease &#40;MCTD&#41;&#46; However&#44; it was interesting to observe overlapping conditions of scleroderma and LEP in our case&#46; He developed sclerosis of the upper extremities&#44; but without organ involvement&#46; LEP was developed on the forehead&#44; scalp&#44; and upper extremity&#46; A recent report reviews 20 cases of lupus panniculitis of the scalp&#44; which involved the parietal &#40;70&#37;&#41;&#44; frontal &#40;45&#37;&#41;&#44; temporal &#40;40&#37;&#41;&#44; occipital &#40;30&#37;&#41;&#44; and vertex &#40;10&#37;&#41; regions&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Ulcer was observed in 10&#37;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">To date&#44; only a few cases of ulcerative LEP have been reported&#44;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;5</span></a> and microangiopathic processes such as segmental fibrinoid vascular necrosis&#44; small vessel thrombosis&#44; and dense angiocentric lymphocyte infiltrates have been suggested&#46; Ishiguro et al&#46; speculated that intractable ulcers occurred as a result of necrobiotic changes in the subcutaneous tissues caused by vascular changes&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> On the other hand&#44; xanthomatous reaction was detected in the fibrotic tissue of LEP&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In the present case&#44; vasculitis was not observed but foam cells were detected in the dermis as well as within the blood vessels&#46; It was speculated that xanthomatous cells were induced by phagocytosis of lipid peroxides caused by macrophage-derived oxygen radicals following destruction of sebaceous glands initiated by immune complex deposition&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> which may have induced circulation abnormalities and also tissue damages&#46; Moreover&#44; cellular infiltrates were observed not only in the subcutaneous tissues but also in the muscle layers&#44; and thus those tissue damages may have led to the ulceration of LEP in this case&#46;</p></span>"
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Case and Research Letters
Ulcerative Lupus Erythematosus Profundus in a Patient With Limited Cutaneous Systemic Sclerosis
Lupus eritematoso profundo ulcerativo en un paciente con esclerosis sistémica cutánea limitada
T. Mori
Corresponding author
mtatsu@fmu.ac.jp

Corresponding author.
, T. Ito, N. Kikuchi, T. Yamamoto
Department of Dermatology, Fukushima Medical University, Fukushima, Japan
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ulcerative lesions on the forehead &#40;A&#41; and scalp &#40;B&#41;&#44; along with swollen fingers and nailfold bleeding &#40;C&#41;&#46; Histological features showing focal lymphohistiocytic infiltration and hyaline fat necrosis in the lower dermis to the subcutis &#40;D&#44; E&#41;&#46; Lymphocytic infiltration was observed in the muscle layers &#40;F&#41;&#46; Foam cells were detected in the vessels &#40;G&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 24-year-old male was referred to our hospital&#44; complaining of recalcitrant ulcers on the forehead and scalp&#46; Physical examination showed well-demarcated ulceration and depressed scars on the forehead&#44; scalp and upper arm &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#44; B&#41;&#46; In addition&#44; he had swollen and cold fingers with ulceration of the tip of the third finger with nail fold extension &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41;&#46; He noticed Raynaud&#8217;s phenomenon and morning stiffness one year previously&#46; Antinuclear antibodies &#40;1&#58;640&#44; nucleolar and homogeneous&#41;&#44; and positive anti-Scl-70 antibodies &#40;16 U&#47;mL&#59; normal &#60;10&#41; were detected in the serum&#44; whereas antibodies against centromere&#44; RNA polymerase III&#44; SS-A&#44; SS-B&#44; DNA&#44; ds-DNA&#44; cardiolipin&#44; and Sm were all within normal limits&#46; A biopsy specimen taken from the forehead revealed fibrosis of the lower dermis and subcutaneous tissues&#44; focal infiltration of mononuclear cells in the lower dermis and subcutaneous fat tissues&#44; and hyaline fat degeneration &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>D&#41;&#46; Furthermore&#44; perivascular mononuclear cell infiltration was observed in the muscle tissues &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>E&#41;&#46; Xanthomatous cells were detected in the dermis&#44; which were positive for CD68&#46; Alcian blue stain revealed mucin deposition in the dermis&#46; Of note&#44; foam cells were detected within the blood vessels in the lower dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>G&#41;&#46; Examination by direct immunofluorescence showed linear deposition of IgM in the epidermal basement membrane&#46; A second biopsy specimen from the finger showed thickened collagen bundles in the dermis&#46; A third biopsy from the forearm showed edematous dermis with slightly thickened collagen bundles in the lower dermis&#46; Although detailed examination denied pulmonary&#44; renal and esophageal involvement&#44; the symptoms of the patient fulfilled the 2013 classification criteria of systemic sclerosis proposed by American College of Rheumatology&#47;European League against Rheumatism&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Oral prednisolone &#40;PSL&#41; &#40;30&#160;mg&#47;day&#41; was started along with intravenous administration of alprostadil&#46; The ulcer was epithelialized six months later&#46; Thereafter&#44; PSL was gradually tapered&#44; and in turn&#44; dapson &#40;50&#160;mg&#47;d&#41; was started&#46; He is well-controlled under 8-10&#160;mg&#47;d oral PSL&#44; without recurrence of ulcers&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Lupus erythematosus profundus &#40;LEP&#41; is a subtype of chronic cutaneous LE with or without systemic lupus erythematosus &#40;SLE&#41;&#46; Additionally&#44; LEP is rarely seen in patients with connective tissue diseases other than SLE&#46; Association between LEP and systemic sclerosis &#40;SSc&#41; is extremely rare&#44; and only a few cases have been reported to date&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Association with LEP and SSc is extremely rare&#44; and thus the etiology of the present case is unclear&#46; The present case suffered from SSc&#44; and detailed examination denied SLE and mixed connective tissue disease &#40;MCTD&#41;&#46; However&#44; it was interesting to observe overlapping conditions of scleroderma and LEP in our case&#46; He developed sclerosis of the upper extremities&#44; but without organ involvement&#46; LEP was developed on the forehead&#44; scalp&#44; and upper extremity&#46; A recent report reviews 20 cases of lupus panniculitis of the scalp&#44; which involved the parietal &#40;70&#37;&#41;&#44; frontal &#40;45&#37;&#41;&#44; temporal &#40;40&#37;&#41;&#44; occipital &#40;30&#37;&#41;&#44; and vertex &#40;10&#37;&#41; regions&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Ulcer was observed in 10&#37;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">To date&#44; only a few cases of ulcerative LEP have been reported&#44;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;5</span></a> and microangiopathic processes such as segmental fibrinoid vascular necrosis&#44; small vessel thrombosis&#44; and dense angiocentric lymphocyte infiltrates have been suggested&#46; Ishiguro et al&#46; speculated that intractable ulcers occurred as a result of necrobiotic changes in the subcutaneous tissues caused by vascular changes&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> On the other hand&#44; xanthomatous reaction was detected in the fibrotic tissue of LEP&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In the present case&#44; vasculitis was not observed but foam cells were detected in the dermis as well as within the blood vessels&#46; It was speculated that xanthomatous cells were induced by phagocytosis of lipid peroxides caused by macrophage-derived oxygen radicals following destruction of sebaceous glands initiated by immune complex deposition&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> which may have induced circulation abnormalities and also tissue damages&#46; Moreover&#44; cellular infiltrates were observed not only in the subcutaneous tissues but also in the muscle layers&#44; and thus those tissue damages may have led to the ulceration of LEP in this case&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Mori T&#44; Ito T&#44; Kikuchi N&#44; Yamamoto T&#46; Lupus eritematoso profundo ulcerativo en un paciente con esclerosis sist&#233;mica cut&#225;nea limitada&#46; Actas Dermosifiliogr&#46; 2021&#46; <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.ad.2019.10.016">https&#58;&#47;&#47;doi&#46;org&#47;10&#46;1016&#47;j&#46;ad&#46;2019&#46;10&#46;016</span></p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ulcerative lesions on the forehead &#40;A&#41; and scalp &#40;B&#41;&#44; along with swollen fingers and nailfold bleeding &#40;C&#41;&#46; Histological features showing focal lymphohistiocytic infiltration and hyaline fat necrosis in the lower dermis to the subcutis &#40;D&#44; E&#41;&#46; Lymphocytic infiltration was observed in the muscle layers &#40;F&#41;&#46; Foam cells were detected in the vessels &#40;G&#41;&#46;</p>"
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