Introduction. -Necrobiotic xanthogranulomatosis is a rare cutaneous di
sorder usually associated with monoclonal gammopathy. Most frequently,
cutaneous lesions involve the face (periorbital region) and the trunk
, and are characterized by indurated xanthomatous plaques and nodules.
Exegesis. -We describe a patient presenting with necrobiotic xanthogr
anulomatosis that was unusual, as it was associated with IgM kappa mon
oclonal gammopathy of undetermined clinical significance and because n
o periorbital involvement was observed. Conclusions. -Necrobiotic xant
hogranulomatosis should be distinguished from other cutaneous manifest
ations associated,vith plasma cell dyscrasias, such as normolipemic pl
ane xanthoma. It can be associated with IgM gammopathy. (C) 1998, Else
vier, Paris.