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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Immunoglobulin G4-related disease &#40;IgG4-RD&#41; is a systemic disease characterized by lymphoplasmacytic inflammation&#44; fibrosis&#44; and elevated levels of IgG4 in tissues and eventually in the blood&#46; IgG4-RD is a recently described disease and encompasses classic entities such as Riedel&#39;s thyroiditis&#44; Mikulicz syndrome&#44; and K&#252;ttner&#39;s tumor&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> It is a rare disease that affects middle-aged men&#44; especially Asians&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The etiology of IgG4-RD is poorly understood&#46; Several potential triggers have been proposed&#44; including genetic susceptibility&#44; malignant tumors&#44; and even some microorganisms such as <span class="elsevierStyleItalic">Helicobacter pylori</span>&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Pathophysiologically&#44; an increase in the Th2 and Treg responses has been described&#46; This induces the release of cytokines such as IL-10 and TGF-&#946;&#44; which are responsible for the inflammation and the fibrosis characteristic of this disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">IgG4-RD predominantly affects the pancreas&#44; presenting with autoimmune pancreatitis&#44; but can affect virtually every organ&#44; including the salivary glands&#44; the lacrimal glands&#44; the orbits&#44; the lungs&#44; the kidneys&#44; the liver&#44; the thyroid&#44; the hypophysis&#44; the retroperitoneum&#44; and the prostate&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Skin involvement is less common&#44; and usually occurs after the onset of systemic symptoms&#46; However&#44; cutaneous involvement prior to the appearance of systemic symptoms has been described&#44; as well as cases with exclusive involvement of the skin&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Cutaneous IgG4-RD presents with erythematous papules&#44; plaques&#44; and particularly nodules&#44; predominantly located on the head and neck&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;5</span></a> The differential diagnosis includes cutaneous lymphomas and pseudolymphomas&#44; sarcoidosis&#44; lupus&#44; cutaneous metastases&#44; and even deep skin infections such as syphilis and micobacteriosis&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Histologically&#44; the lesions are characterized by an infiltrate rich in lymphocytes and plasma cells&#44; storiform fibrosis&#44; obliterative phlebitis&#44; and less frequently an infiltrate with few eosinophils&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4&#44;5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Various diagnostic criteria for IgG4-RD have been proposed&#46; These include the presence of compatible signs &#40;mass or swelling in one or more organs&#41; and a biopsy compatible with elevated levels of IgG4&#43; plasma cells &#40;IgG4&#47;IgG ratio <span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>40&#37;&#59;<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>10 IgG4&#43; cells per high-power field&#41;&#44; associated with elevated serum levels of IgG4 &#40;&#62;<span class="elsevierStyleHsp" style=""></span>135<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> However&#44; these criteria remain under discussion&#44; as normal serum IgG4 levels can be found in patients with exclusive skin or single organ involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> It is also important to note that an increase in serum IgG4 levels is not a pathognomonic finding in IgG4-RD as such increases have been described in other dermatoses such as pemphigus vulgaris&#44; atopic dermatitis&#44; some parasitic infections&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> and recently in marginal zone lymphomas&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">IgG4-RD has been treated using systemic corticosteroids&#44; azathioprine&#44; thalidomide&#44; and even biologics such as rituximab and infliximab&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> The prognosis depends on the degree of dysfunction caused by the inflammation and fibrosis in the organs affected by the disease&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">In summary&#44; it is important that dermatologists consider IgG4-RD in patients presenting with a nodule or mass with an infiltrate rich in plasma cells&#46; However&#44; as IgG4-RD is a recently described and little-known disease&#44; the diagnostic limitations should be borne in mind&#46; Further characterization of this disease is necessary to establish definitive diagnostic criteria&#46;</p></span>"
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Resident's Forum
Dermatological Aspects of immunoglobulin G4 (IgG4)-Related Disease
RF-Perspectiva dermatológica de la enfermedad relacionada con la inmunoglobulina G4 (IgG4)
O. Yélamos
Servicio de Dermatología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Immunoglobulin G4-related disease &#40;IgG4-RD&#41; is a systemic disease characterized by lymphoplasmacytic inflammation&#44; fibrosis&#44; and elevated levels of IgG4 in tissues and eventually in the blood&#46; IgG4-RD is a recently described disease and encompasses classic entities such as Riedel&#39;s thyroiditis&#44; Mikulicz syndrome&#44; and K&#252;ttner&#39;s tumor&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> It is a rare disease that affects middle-aged men&#44; especially Asians&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The etiology of IgG4-RD is poorly understood&#46; Several potential triggers have been proposed&#44; including genetic susceptibility&#44; malignant tumors&#44; and even some microorganisms such as <span class="elsevierStyleItalic">Helicobacter pylori</span>&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Pathophysiologically&#44; an increase in the Th2 and Treg responses has been described&#46; This induces the release of cytokines such as IL-10 and TGF-&#946;&#44; which are responsible for the inflammation and the fibrosis characteristic of this disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">IgG4-RD predominantly affects the pancreas&#44; presenting with autoimmune pancreatitis&#44; but can affect virtually every organ&#44; including the salivary glands&#44; the lacrimal glands&#44; the orbits&#44; the lungs&#44; the kidneys&#44; the liver&#44; the thyroid&#44; the hypophysis&#44; the retroperitoneum&#44; and the prostate&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Skin involvement is less common&#44; and usually occurs after the onset of systemic symptoms&#46; However&#44; cutaneous involvement prior to the appearance of systemic symptoms has been described&#44; as well as cases with exclusive involvement of the skin&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Cutaneous IgG4-RD presents with erythematous papules&#44; plaques&#44; and particularly nodules&#44; predominantly located on the head and neck&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;5</span></a> The differential diagnosis includes cutaneous lymphomas and pseudolymphomas&#44; sarcoidosis&#44; lupus&#44; cutaneous metastases&#44; and even deep skin infections such as syphilis and micobacteriosis&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Histologically&#44; the lesions are characterized by an infiltrate rich in lymphocytes and plasma cells&#44; storiform fibrosis&#44; obliterative phlebitis&#44; and less frequently an infiltrate with few eosinophils&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4&#44;5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Various diagnostic criteria for IgG4-RD have been proposed&#46; These include the presence of compatible signs &#40;mass or swelling in one or more organs&#41; and a biopsy compatible with elevated levels of IgG4&#43; plasma cells &#40;IgG4&#47;IgG ratio <span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>40&#37;&#59;<span class="elsevierStyleHsp" style=""></span>&#62;<span class="elsevierStyleHsp" style=""></span>10 IgG4&#43; cells per high-power field&#41;&#44; associated with elevated serum levels of IgG4 &#40;&#62;<span class="elsevierStyleHsp" style=""></span>135<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> However&#44; these criteria remain under discussion&#44; as normal serum IgG4 levels can be found in patients with exclusive skin or single organ involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> It is also important to note that an increase in serum IgG4 levels is not a pathognomonic finding in IgG4-RD as such increases have been described in other dermatoses such as pemphigus vulgaris&#44; atopic dermatitis&#44; some parasitic infections&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> and recently in marginal zone lymphomas&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">IgG4-RD has been treated using systemic corticosteroids&#44; azathioprine&#44; thalidomide&#44; and even biologics such as rituximab and infliximab&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> The prognosis depends on the degree of dysfunction caused by the inflammation and fibrosis in the organs affected by the disease&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">In summary&#44; it is important that dermatologists consider IgG4-RD in patients presenting with a nodule or mass with an infiltrate rich in plasma cells&#46; However&#44; as IgG4-RD is a recently described and little-known disease&#44; the diagnostic limitations should be borne in mind&#46; Further characterization of this disease is necessary to establish definitive diagnostic criteria&#46;</p></span>"
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