{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:19:57Z","timestamp":1747181997634,"version":"3.40.5"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T00:00:00Z","timestamp":1615161600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of neoplastic mast cells in the skin and\/or other extracutaneous tissues. Most patients with skin involvement can be subclassified into one of the three subtypes of cutaneous mastocytosis currently recognized by the World Health Organization (i.e., mastocytoma, maculopapular cutaneous mastocytosis and diffuse cutaneous mastocytosis); however, some patients may occasionally present with atypical skin lesions that cannot be ascribed to any of these disease subtypes. Here, we report three patients diagnosed with mastocytosis and an unusual cutaneous involvement mimicking Kaposi\u2019s sarcoma. Skin biopsies showed neoplastic mast cell infiltrates together with features commonly seen in acroangiodermatitis, and immunohistochemistry for human herpesvirus 8 was negative. One patient fulfilled the criteria for aggressive systemic mastocytosis, showed no response to cytoreductive therapy, and died because of disease progression. The remaining two patients had indolent and smoldering systemic mastocytosis, respectively, but they showed several features associated with an unfavorable prognosis such as extensive involvement of the hematopoiesis by the <jats:italic>KIT<\/jats:italic> D816V mutation, increased serum \u03b22\u2010microglobulin, and decreased serum lactate dehydrogenase. The presence of pseudo\u2010Kaposi\u2019s sarcoma skin lesions is an uncommon finding in mastocytosis which may alert physicians to the possible existence of underlying features indicative of a poor prognosis.<\/jats:p>","DOI":"10.1111\/1346-8138.15734","type":"journal-article","created":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T17:03:56Z","timestamp":1615223036000},"page":"657-660","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Mastocytosis in the skin accompanied by pseudo\u2010Kaposi\u2019s sarcoma"],"prefix":"10.1111","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8646-3636","authenticated-orcid":false,"given":"Laura","family":"Vergara\u2010de\u2010la\u2010Campa","sequence":"first","affiliation":[{"name":"Dermatology Department Complejo Hospitalario de Toledo Toledo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"In\u00e9s","family":"Torrado","sequence":"additional","affiliation":[{"name":"Spanish Reference Center of Mastocytosis Instituto de Estudios de Mastocitosis de Castilla\u2010La Mancha (CLMast) Hospital Virgen del Valle Complejo Hospitalario de Toledo Toledo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda","family":"Recuero\u2010Pradillo","sequence":"additional","affiliation":[{"name":"Pathology Department Complejo Hospitalario de Toledo Toledo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Torrelo","sequence":"additional","affiliation":[{"name":"Dermatology Department Hospital Infantil Universitario Ni\u00f1o Jes\u00fas Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mercedes","family":"Gironella","sequence":"additional","affiliation":[{"name":"Hematology Department Hospital Universitario Vall d\u2019Hebron Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emilia","family":"Pardal","sequence":"additional","affiliation":[{"name":"Hematology Department Hospital Virgen del Puerto Plasencia Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Cristina","family":"Godoy","sequence":"additional","affiliation":[{"name":"Hematology Department Hospital Universitario Miguel Servet Zaragoza Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Almudena","family":"Matito","sequence":"additional","affiliation":[{"name":"Spanish Reference Center of Mastocytosis Instituto de Estudios de Mastocitosis de Castilla\u2010La Mancha (CLMast) Hospital Virgen del Valle Complejo Hospitalario de Toledo Toledo Spain"},{"name":"Spanish Network on Mastocytosis (REMA) Toledo Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"P\u00e9rez\u2010Hortet","sequence":"additional","affiliation":[{"name":"Dermatology 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Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,3,8]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000101711"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.2174\/1573396315666181120163952"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2015.08.034"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2012-0101-RS"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.4103\/2229-5178.137791"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(86)90442-3"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1590\/abd1806-4841.20143036"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0463.2002.100501.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.05.003"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2011.84"}],"container-title":["The 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