Idiopathic CD4⁺ lymphocytopenia (ICL) is a rare immunodeficiency syndrome of unknown origin for which the increased risks of opportunistic infections and of malignancies have been well established; however, skin dysimmune diseases, including psoriasis, have been scarcely reported up to now. We report herein the severe course of psoriasis in four patients with ICL, and show evidence for a defect in the skin recruitment of regulatory CD4⁺FoxP3⁺ T cells. These data raise the apparent paradigm of the occurrence of a severe immunomediated disease together with a profound T‐cell defect, a model that might also apply to other immune deficiencies associated with psoriasis.