The syndrome defined as 'idiopathic CD4 lymphocytopenia' (ICL) is a ra
re disease of unknown aetiology, often associated with severe depressi
on of immune defences and the occurrence of opportunistic infections.
A case is reported wherein a severe immunodeficiency syndrome with per
sistent idiopathic CD4+ lymphopenia developed in a woman suffering fro
m systemic microscopic polyarteritis; no signs of HIV 1/2 or HTLV I/II
infection were evident. The patient died of widespread opportunistic
infections. The association of ICL with vasculitis has never been repo
rted until now. A link between the two diseases cannot be ruled out.