There is sometimes lymphocytic infiltrative disease in parenchymal organs, but autoimmunity is minimal and lymphoma has not been observed in the CEDS patients.

Cell death counters proliferation of lymphocytes, allowing the immune system to achieve dynamic homeostasis whereby it can defend against pathogens and avoid autoimmunity.

The clinical phenotype of CEDS patients represented a paradox because caspase-8 was considered to be chiefly a pro-apoptotic protease, that was mainly involved in signal transduction.

[5] Clinical features suggesting CEDS should be investigated by immunologic studies assessing serum immunoglobulin levels, antibody function, and lymphocyte activation.

Patients who have CEDS have done well while maintained on intravenous immunoglobulin and prophylactic acyclovir to decrease sinopulmonary infections and mucocutaneous herpes virus outbreaks.