Primary immunodeficiencies: The term "primary immunodeficiencies, synonym: PID/immune deficiency syndromes/immune deficiency diseases" covers more than 400 different diseases of the immune system, which are characterized by a temporary or permanent disturbance of the immune function, in which the immune deficiency is congenital, occurs in families and/or can be inherited (see under primary immunodeficiencies/overview and classification).
Clinically, primary immunodeficiencies are usually, but not always (e.g. fever syndromes), manifested by a pathological susceptibility to infections. PGDs can manifest in all organs, with surface epithelia in the skin, lungs, intestines, and genitourinary tract most commonly affected.
Among PIDs,common variable immunodeficiency, or CVID, is the most common. Typical warning signs are hidden behind the acronyms:
- ELVIS (pathogen: unusual; localization: atypical; course: long; intensity: severe; total: very common)
and
- GARFIELD (granulomas, autoimmunity, recurrent fever, eczema, lymphoproliferation, intestinal inflammation).
The following table gives an alphabetically listed overview of skin changes that can occur in connection with primary immunodeficiencies: