Cutaneous granulomas in ataxia telangiectasia and other primary immunodeficiencies: reflection of inappropriate immune regulation?
Dermatology
; 223(1): 13-9, 2011.
Article
en En
| MEDLINE
| ID: mdl-21876338
ABSTRACT
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.
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Enfermedades de la Piel
/
Ataxia Telangiectasia
/
Granuloma
Límite:
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Dermatology
Asunto de la revista:
DERMATOLOGIA
Año:
2011
Tipo del documento:
Article
País de afiliación:
Países Bajos