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Immunodeficiency syndromes:
| • | heterogeneous group of diseases in which the immune system does not develop normally |
| • | many of these patients have an increased susceptibility to malignancy |
| • | in addition, many have an increased incidence of eczema for reasons that are not clear |
Clinical differential diagnosis (all 3 show chronic eczematous dermatitis):
| • | Wiskott-Aldrich syndrome |
Other:
| • | epidermodysplasia verruciformis |
Treatment:
Prognosis:
| CGD chronic granulomatous disease | normal life span |
SUMMARY:
| Disease: | Defect in immunity: |
| epidermodysplasia verruciformis | natural killer cell activity (i.e. cell mediated immunity) |
| Leiner disease | C5 dysfunction (though normal levels) |
| chronic mucocutaneous candidiasis | cell-mediated immunity |
| Wiskott-Aldrich syndrome | WASP gene (impairs cell-mediated and humoral immunity) |
| Job syndrome | diminished neutrophil chemotaxis |
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