{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T21:53:18Z","timestamp":1775771598593,"version":"3.50.1"},"reference-count":39,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatology"],"published-print":{"date-parts":[[2011]]},"abstract":"<jats:p>&lt;i&gt;Background:&lt;\/i&gt; Non-infective cutaneous granulomas with unknown pathogenesis occur in various primary immunodeficiencies (PIDs) including ataxia telangiectasia (A-T). &lt;i&gt;Objective:&lt;\/i&gt; To find a common immunological denominator in these cutaneous granulomas. &lt;i&gt;Methods:&lt;\/i&gt; The dermatological and immunological features of 4 patients with A-T and cutaneous granulomas were described. The literature on skin granulomas in A-T and in other PIDs is reviewed. &lt;i&gt;Results:&lt;\/i&gt; All 4 A-T patients had progressive granulomas on their limbs and showed decreased IgG and IgA concentrations with normal IgM levels. They had a marked decrease in B cells and na\u00efve T cells coinciding with the appearance of the cutaneous granulomas. Similar B- and T-cell abnormalities were described in patients with other PIDs with skin granulomas. &lt;i&gt;Conclusions:&lt;\/i&gt; We hypothesize that the pathogenesis of these skin granulomas is related to immune dysregulation of macrophages due to the absence of na\u00efve T cells with an appropriate T-cell receptor repertoire and the unopposed activity of \u03b3\u03b4 T cells and\/or natural killer cells.<\/jats:p>","DOI":"10.1159\/000330335","type":"journal-article","created":{"date-parts":[[2011,8,29]],"date-time":"2011-08-29T08:29:57Z","timestamp":1314606597000},"page":"13-19","source":"Crossref","is-referenced-by-count":60,"title":["Cutaneous Granulomas in Ataxia Telangiectasia and Other Primary Immunodeficiencies: Reflection of Inappropriate Immune Regulation?"],"prefix":"10.1159","volume":"223","author":[{"given":"L.Y.T.","family":"Chiam","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.M.M.","family":"Verhagen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Haraldsson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Wulffraat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.-J.","family":"Driessen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.G.","family":"Netea","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.M.R.","family":"Weemaes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.M.B.","family":"Seyger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"van 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