The sialographic differentiation of Mikulicz's disease and Mikulicz's syndrome.

Abstract

IN THE SEVEN decades since Mikulicz described the disease entity which bears his name, there has been much confusion between Mikulicz's disease and Mikulicz's syndrome. The literature records attempts to differentiate the two by means of biopsy, but histopathology, particularly in Mikulicz's disease, is uncertain. Most of these reports (3, 15, 17, 20) were written before the advent of sialography, and more recently this diagnostic approach has been employed only sporadically, and then with conflicting observations (2, 30, 46, 47). It was for this reason that sialographic studies were undertaken in a group of patients with salivary gland swellings in which histologic proof of Mikulicz's disease or Mikulicz's syndrome had been obtained. The resulting roentgenograms have shown a pattern of variations indicating that sialography can be used for the differential diagnosis of the two conditions. Historically, the disease was first identifled in 1888 by Johann von MikuliczRadecki (23), who described it as a distinct clinical and pathological entity having as its chief characteristic symmetrical tumefactions of the lacrimal and salivary glands which develop gradually, are hard in consistency, painless, and unassociated with other organic involvement. Microscopic examinations of the excised portions of the gland disclosed atrophy of the acinar parenchyma and diffuse replacement by lymphoid tissues. In the following years numerous reports attempted to classify the variations found in what appeared to be cases of Mikulicz's disease. Von Brunn (3) in 1906, and Howard (15) in 1909, distinguished between the disease and pseudoleukemia and true lymphatic leukemia, but this simple classification failed to take into account the varied causes of lacrimal and salivary gland swellings. A more elaborate classification on etiological grounds was made by Thursfield (53) in 1914, and finally Schaffer and Jacobsen (45), in 1927, distinguished between Mikulicz's disease and Mikulicz's syndrome. This remains the accepted classification today and contrasts Mikulicz's disease with its uncertain histopathology against Mikulicz's syndrome, of more clearly defined pathologic nature, including such entities as leukemia, the lymphomas, sarcoidosis, tuberculosis, and syphilis. The sialographic studies comprising this series were initiated at the University of Michigan and continued at the Nat ional Institutes of Health, Bethesda, Md. Fortythree patients have been examined. More than one hundred sialograms have been completed in which reflex stimulation of the salivary glands has been employed. There were 29 cases of Mikulicz's disease (see Table I) and 14of Mikulicz's syndrome, the latter group including 3 cases of leu kemia, 5 of lymphoma, 5 of sarcoidosis, and 1 of tuberculosis. Mikulicz's Disease There is no consistent clinical pattern of symptoms in so-called Mikulicz's disease.