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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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Yélamos" "autores" => array:1 [ 0 => array:3 [ "nombre" => "O." "apellidos" => "Yélamos" "email" => array:1 [ 0 => "oyelamos@santpau.cat" ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain" "identificador" => "aff0005" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "RF-Perspectiva dermatológica de la enfermedad relacionada con la inmunoglobulina G4 (IgG4)" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by lymphoplasmacytic inflammation, fibrosis, and elevated levels of IgG4 in tissues and eventually in the blood. IgG4-RD is a recently described disease and encompasses classic entities such as Riedel's thyroiditis, Mikulicz syndrome, and Küttner's tumor.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> It is a rare disease that affects middle-aged men, especially Asians.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The etiology of IgG4-RD is poorly understood. Several potential triggers have been proposed, including genetic susceptibility, malignant tumors, and even some microorganisms such as <span class="elsevierStyleItalic">Helicobacter pylori</span>.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Pathophysiologically, an increase in the Th2 and Treg responses has been described. This induces the release of cytokines such as IL-10 and TGF-β, which are responsible for the inflammation and the fibrosis characteristic of this disease.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">IgG4-RD predominantly affects the pancreas, presenting with autoimmune pancreatitis, but can affect virtually every organ, including the salivary glands, the lacrimal glands, the orbits, the lungs, the kidneys, the liver, the thyroid, the hypophysis, the retroperitoneum, and the prostate.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> Skin involvement is less common, and usually occurs after the onset of systemic symptoms. However, cutaneous involvement prior to the appearance of systemic symptoms has been described, as well as cases with exclusive involvement of the skin.<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, plaques, and particularly nodules, predominantly located on the head and neck.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4,5</span></a> The differential diagnosis includes cutaneous lymphomas and pseudolymphomas, sarcoidosis, lupus, cutaneous metastases, and even deep skin infections such as syphilis and micobacteriosis.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Histologically, the lesions are characterized by an infiltrate rich in lymphocytes and plasma cells, storiform fibrosis, obliterative phlebitis, and less frequently an infiltrate with few eosinophils.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1,4,5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Various diagnostic criteria for IgG4-RD have been proposed. These include the presence of compatible signs (mass or swelling in one or more organs) and a biopsy compatible with elevated levels of IgG4+ plasma cells (IgG4/IgG ratio <span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>40%;<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>10 IgG4+ cells per high-power field), associated with elevated serum levels of IgG4 (><span class="elsevierStyleHsp" style=""></span>135<span class="elsevierStyleHsp" style=""></span>mg/dL).<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> However, these criteria remain under discussion, as normal serum IgG4 levels can be found in patients with exclusive skin or single organ involvement.<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, atopic dermatitis, some parasitic infections,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> and recently in marginal zone lymphomas.<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, azathioprine, thalidomide, and even biologics such as rituximab and infliximab.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1,3,4</span></a> The prognosis depends on the degree of dysfunction caused by the inflammation and fibrosis in the organs affected by the disease.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In summary, it is important that dermatologists consider IgG4-RD in patients presenting with a nodule or mass with an infiltrate rich in plasma cells. However, as IgG4-RD is a recently described and little-known disease, the diagnostic limitations should be borne in mind. Further characterization of this disease is necessary to establish definitive diagnostic criteria.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "PalabrasClave" => array:1 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1558320" "palabras" => array:2 [ 0 => "Immunoglobulin G4 (IgG4)" 1 => "IgG4-related disease" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Yélamos O. RF-Perspectiva dermatológica de la enfermedad relacionada con la inmunoglobulina G4 (IgG4). Actas Dermosifiliogr. 2015;106:231–232.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "IgG4-related disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.H. Stone" 1 => "Y. Zen" 2 => "V. 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| 2018 October | 4 | 0 | 4 |
| 2018 September | 4 | 1 | 5 |
| 2018 August | 0 | 4 | 4 |
| 2018 July | 0 | 5 | 5 |
| 2018 June | 0 | 1 | 1 |
| 2018 May | 0 | 7 | 7 |
| 2018 April | 0 | 9 | 9 |
| 2018 February | 29 | 3 | 32 |
| 2018 January | 37 | 10 | 47 |
| 2017 December | 33 | 9 | 42 |
| 2017 November | 19 | 8 | 27 |
| 2017 October | 19 | 10 | 29 |
| 2017 September | 20 | 7 | 27 |
| 2017 August | 16 | 7 | 23 |
| 2017 July | 7 | 11 | 18 |
| 2017 June | 30 | 19 | 49 |
| 2017 May | 15 | 14 | 29 |
| 2017 April | 20 | 17 | 37 |
| 2017 March | 16 | 16 | 32 |
| 2017 February | 13 | 15 | 28 |
| 2017 January | 16 | 14 | 30 |
| 2016 December | 23 | 8 | 31 |
| 2016 November | 43 | 14 | 57 |
| 2016 October | 28 | 15 | 43 |
| 2016 September | 0 | 10 | 10 |
| 2016 August | 0 | 2 | 2 |
| 2016 July | 7 | 1 | 8 |
| 2016 June | 11 | 6 | 17 |
| 2016 May | 9 | 19 | 28 |
| 2016 April | 11 | 30 | 41 |
| 2016 March | 11 | 17 | 28 |
| 2016 February | 8 | 1 | 9 |
| 2016 January | 5 | 13 | 18 |
| 2015 December | 6 | 7 | 13 |
| 2015 November | 5 | 1 | 6 |
| 2015 October | 8 | 7 | 15 |
| 2015 September | 0 | 6 | 6 |
| 2015 August | 0 | 1 | 1 |
| 2015 July | 6 | 2 | 8 |
| 2015 June | 2 | 2 | 4 |
| 2015 May | 2 | 4 | 6 |
| 2015 April | 0 | 12 | 12 |
Show all
