2011-10-01
Cutaneous granulomas in ataxia telangiectasia and other primary immunodeficiencies: Reflection of inappropriate immune regulation?
Publication
Publication
Dermatology: international journal for clinical and investigative dermatology , Volume 223 - Issue 1 p. 13- 19
Background: Non-infective cutaneous granulomas with unknown pathogenesis occur in various primary immunodeficiencies (PIDs) including ataxia telangiectasia (A-T). Objective: To find a common immunological denominator in these cutaneous granulomas. Methods: 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. Results: 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. Conclusions: 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.
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| doi.org/10.1159/000330335, hdl.handle.net/1765/73336 | |
| Dermatology: international journal for clinical and investigative dermatology | |
| Organisation | Department of Pediatrics |
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Chiam, L. Y. T., Verhagen, M., Haraldsson, Á., Wulffraat, N., Driessen, G., Netea, M., … van Deuren, M. (2011). Cutaneous granulomas in ataxia telangiectasia and other primary immunodeficiencies: Reflection of inappropriate immune regulation?. Dermatology: international journal for clinical and investigative dermatology (Vol. 223, pp. 13–19). doi:10.1159/000330335 |
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