TRUE Mikulicz's disease is rare. Few surgeons have ever had a case. A search of 10 standard surgical textbooks revealed no mention of the disease, yet its cure may require surgical intervention. Less than 100 authentic cases were found in the literature, although the latter is replete with cases that do not fit the classical description of the disease given by Mikulicz before the society for scientific medicine at Königsberg in 1888.
Mikulicz said:
Both lacrymal glands and all the salivary glands were changed in a symmetrical manner into tumors, which pushed themselves markedly forward from the normal position of these organs and thereby distorted the physiognomy of the patient in a remarkable way. The tumors had arisen gradually; they were at the time of examination of a hard consistency, painless, without trace of inflammatory signs. Moreover, elsewhere there were present no pathological changes in the bearer of these tumors....