Table 1

Summary of the main clinical and biological features of patients

Patient 1Patient 2Patient 3
Dyskeratosis+++
Arthritis+++
Recurrent fever+++
Chronic infection+−+
Recurrent elevated CRP+++
ANA−++
Vitamin A deficiency+++
High transitional B cells+−+

  • −, negative; +, positive; ANA, antinuclear antibody; CRP, C reactive protein.