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Cutaneous Granulomas in Ataxia Telangiectasia and Other Primary Immunodeficiencies: Reflection of Inappropriate Immune Regulation?

2011; Karger Publishers; Volume: 223; Issue: 1 Linguagem: Inglês

10.1159/000330335

1421-9832

Lynn Yuun Tirng Chiam, M.M.M. Verhagen, Ásgeir Haraldsson, N. M. Wulffraat, Gertjan J. Driessen, M.G. Netea, Corry M.R. Weemaes, Marieke M.B. Seyger, Marcel van Deuren,

Parvovirus B19 Infection Studies

<i>Background:</i> Non-infective cutaneous granulomas with unknown pathogenesis occur in various primary immunodeficiencies (PIDs) including ataxia telangiectasia (A-T). <i>Objective:</i> To find a common immunological denominator in these cutaneous granulomas. <i>Methods:</i> 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. <i>Results:</i> 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 naïve 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. <i>Conclusions:</i> We hypothesize that the pathogenesis of these skin granulomas is related to immune dysregulation of macrophages due to the absence of naïve T cells with an appropriate T-cell receptor repertoire and the unopposed activity of γδ T cells and/or natural killer cells.