PRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 101 No. 8 August 2008
Three Cases of Mikulicz's Disease as an
IgG4-related Disease
Sei-ichi Nakano
(Tokushima Red Cross Hospital)
Motohisa Yamamoto
(Sapporo Medical University)
Teiji Takemura
(Sakai Municipal Hospital)
Naozumi Ishimaru and Yoshio Hayashi
(University of Tokushima School of Dentistry)
Katsushi Miyazaki, Koichi Tamura and Noriaki Takeda
(University of Tokushima)
Tetsuo Himi
(Sapporo Medical University)
We reported three cases of Mikulicz's disease. Mikulicz's disease was diagnosed by the following criteria: 1) symmetrical enlargement of the lacrimal and salivary glands lasting at least 3 months', 2) the lacrimal and salivary glands were microscopically involved in the infiltration of inflammatory cells, 3) sarcoidosis and other lymphoproliferative diseases were excluded. Three patients with Mikulicz's disease were all characterized by elevated IgG4 concentration in the serum and prominent infiltration of IgG4-positive plasmacytes in the minor salivary glands. On the contrary, there were no findings indicating sicca syndrome or anti-SS-A and SS-B antibodies, which characterize Sjogren syndrome. It is suggested that Mikulicz's disease is an IgG4-related plasmacytic disease, with a pathogenesis differing from that of Sjogren syndrome.
Key words :Mikukicz's disease, IgG4, lacrimal gland, salivary gland, Sjogren syndrome