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1</a>&#41;&#46; There were also multiple papules that coalesced to form brownish plaques with irregular borders on the lateral aspects of both feet &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; These lesions extended onto the lateral surface of both ankles&#44; where discrete ulceration was observed&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histology showed fibrinoid necrosis of the small caliber vessels&#44; with a polymorphonuclear infiltrate and karyorrhexis&#46; The early stages of concentric perivascular fibrosis were also present &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Immunofluorescence was negative&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Blood tests&#44; liver function&#44; kidney function&#44; serum electrolytes&#44; protein analysis and serum protein electrophoresis&#44; antinuclear antibodies&#44; and cryoglobulin were all normal&#46; Urinary sediment was normal&#46; Serology for human immunodeficiency virus &#40;HIV&#41; and hepatitis A&#44; B&#44; and C viruses was negative&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Erythema elevatum diutinum &#40;EED&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Blood tests confirmed normal glucose-6-phosphate dehydrogenase levels&#44; and treatment was then initiated with dapsone 100<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#44; achieving a good clinical response&#46; After 6 weeks of treatment with no recurrence of the symptoms&#44; the dose of dapsone was reduced to 50<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#46; At the time of writing&#44; only a residual postinflammatory hyperpigmentation remained&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">EED is a rare form of leukocytoclastic vasculitis characterized by a chronic clinical course&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> It affects adults aged between 30 and 70 years&#44; although cases have been reported in other age ranges&#46; It presents clinically as erythematous-purpuric and yellowish papules that can develop into infiltrated plaques with irregular borders&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The lesions are initially soft&#44; but later acquire a firm&#44; doughy consistency&#46; Some patients enter a chronic phase of fibrosis in which the initial lesions are replaced by nodules that are hard and can grow to a large size&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Histologically&#44; EED is characterized by leukocytoclastic vasculitis of small-caliber vessels&#44; with fibrinoid necrosis and a neutrophilic infiltrate&#46; The epidermis shows acanthosis and parakeratosis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The most distinctive feature of the disease is the appearance of progressive concentric perivascular fibrosis around the affected vessels&#46; Histological findings are very similar to those of granuloma faciale in an extrafacial location and differentiation of the 2 conditions is difficult for single lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">EED has been described in association with many systemic conditions&#44; including autoimmune and hematological diseases and infections&#44; in particular HIV infection&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> in which EED may be the first manifestation&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Hepatitis B or C virus infection must also be ruled out&#44; and a complete blood count and protein electrophoresis are needed to exclude IgA and IgG gammopathy&#44; IgA multiple myeloma&#44; polycythemia vera&#44; hairy cell leukemia&#44; mixed cryoglobulinemia&#44; and myelodysplasia&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The group of diseases associated with EED includes inflammatory bowel disease&#44; rheumatoid arthritis&#44; systemic lupus erythematosus&#44; and relapsing polychondritis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The treatment of choice for EED is dapsone&#44; 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Cases for Diagnosis
Grouped Papules and Plaques on the Lower Limbs
Pápulas y placas agrupadas en extremidades inferiores
J.A. Suárez-Pérez
Corresponding author
jasuape@hotmail.com

Corresponding author.
, N. López-Navarro, E. Herrera-Acosta
Servicio de Dermatología, Hospital Clínico Universitario Virgen de la Victoria, Málaga, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 33 year-old man with no relevant medical history who was seen for skin lesions that had been present on both pretibial areas and lateral borders of the feet for 2 years&#46; The lesions were slightly pruritic and followed an intermittent course with periods of improvement but never healed completely&#46; No improvement was seen with the application of topical corticosteroids&#46; The patient reported no extracutaneous symptoms&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed multiple brownish papules and plaques distributed over both pretibial areas &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; There were also multiple papules that coalesced to form brownish plaques with irregular borders on the lateral aspects of both feet &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; These lesions extended onto the lateral surface of both ankles&#44; where discrete ulceration was observed&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histology showed fibrinoid necrosis of the small caliber vessels&#44; with a polymorphonuclear infiltrate and karyorrhexis&#46; The early stages of concentric perivascular fibrosis were also present &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Immunofluorescence was negative&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Blood tests&#44; liver function&#44; kidney function&#44; serum electrolytes&#44; protein analysis and serum protein electrophoresis&#44; antinuclear antibodies&#44; and cryoglobulin were all normal&#46; Urinary sediment was normal&#46; Serology for human immunodeficiency virus &#40;HIV&#41; and hepatitis A&#44; B&#44; and C viruses was negative&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Erythema elevatum diutinum &#40;EED&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Blood tests confirmed normal glucose-6-phosphate dehydrogenase levels&#44; and treatment was then initiated with dapsone 100<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#44; achieving a good clinical response&#46; After 6 weeks of treatment with no recurrence of the symptoms&#44; the dose of dapsone was reduced to 50<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#46; At the time of writing&#44; only a residual postinflammatory hyperpigmentation remained&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">EED is a rare form of leukocytoclastic vasculitis characterized by a chronic clinical course&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> It affects adults aged between 30 and 70 years&#44; although cases have been reported in other age ranges&#46; It presents clinically as erythematous-purpuric and yellowish papules that can develop into infiltrated plaques with irregular borders&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The lesions are initially soft&#44; but later acquire a firm&#44; doughy consistency&#46; Some patients enter a chronic phase of fibrosis in which the initial lesions are replaced by nodules that are hard and can grow to a large size&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Histologically&#44; EED is characterized by leukocytoclastic vasculitis of small-caliber vessels&#44; with fibrinoid necrosis and a neutrophilic infiltrate&#46; The epidermis shows acanthosis and parakeratosis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The most distinctive feature of the disease is the appearance of progressive concentric perivascular fibrosis around the affected vessels&#46; Histological findings are very similar to those of granuloma faciale in an extrafacial location and differentiation of the 2 conditions is difficult for single lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">EED has been described in association with many systemic conditions&#44; including autoimmune and hematological diseases and infections&#44; in particular HIV infection&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> in which EED may be the first manifestation&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Hepatitis B or C virus infection must also be ruled out&#44; and a complete blood count and protein electrophoresis are needed to exclude IgA and IgG gammopathy&#44; IgA multiple myeloma&#44; polycythemia vera&#44; hairy cell leukemia&#44; mixed cryoglobulinemia&#44; and myelodysplasia&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> The group of diseases associated with EED includes inflammatory bowel disease&#44; rheumatoid arthritis&#44; systemic lupus erythematosus&#44; and relapsing polychondritis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The treatment of choice for EED is dapsone&#44; which reduces disease severity but is not curative&#59; recurrence is common when treatment is stopped&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Control of associated diseases&#44; when present&#44; is essential&#46; Other therapeutic options include niacinamide&#44; colchicine&#44; and sulfapyridine&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Treatment with intralesional corticosteroids and surgical excision are only indicated in cases of localized disease&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p></span></span>"
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ISSN: 15782190
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Idiomas
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