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.
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-Tpatients 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.
Authors: Heather Minto; Kofi A Mensah; Paul R Reynolds; Eric Meffre; Kira Rubtsova; Erwin W Gelfand Journal: Clin Immunol Date: 2019-01-09 Impact factor: 3.969
Authors: Elena Deripapa; Dmitry Balashov; Yulia Rodina; Alexandra Laberko; Natalya Myakova; Nataliia V Davydova; Maria A Gordukova; Dmitrii S Abramov; Galina V Pay; Larisa Shelikhova; Andrey P Prodeus; Mikhail A Maschan; Alexey A Maschan; Anna Shcherbina Journal: Front Immunol Date: 2017-07-24 Impact factor: 7.561
Authors: Oyinade M Aderibigbe; Debra Long Priel; Chyi-Chia Richard Lee; Michael J Ombrello; Vimal H Prajapati; Marilyn G Liang; Jonathan J Lyons; Douglas B Kuhns; Edward W Cowen; Joshua D Milner Journal: JAMA Dermatol Date: 2015-06 Impact factor: 10.282