Kikuchi's disease (KD) is a benign self-limiting febrile illness usual
ly affecting young women, which is manifested clinically by fever and
cervical lymphadenopathy. Skin involvement in Ka is very rare and is e
vident clinically in the form of skin rashes and nodules. We describe
one such case of KD in a 33-year-old Bulgarian woman who presented wit
h cervical and axillary lymphadenopathy and who developed a transient
facial rash. Biopsy of axillary lymph nodes showed the characteristic
features of KD with infiltration of the lymph node paracortex by apopt
otic plasmacytoid monocytes. Biopsies of the facial skin showed two fe
atures: (1) dermal infiltration by apoptotic plasmacytoid monocytes; (
2) on immunofluorescence studies of frozen sections prepared from invo
lved and uninvolved facial skin, deposition of immunoglobulins and com
plement at the dermoepidermal junction and in the walls of dermal bloo
d vessels. Such immunofluorescence findings in the skin of patients wi
th KD have never been described. These findings suggest the presence o
f an autoimmune reaction as a component of KD.