Mikulicz Syndrome Research Articles

Kaposi's Sarcoma of the Eyelids of an African Child

Lerman and Pinsky (1959) recently, in this registry, briefly reviewed the literature on Kaposi's sarcoma, made special reference to the few cases in which the primary lesion has been situated in the orbital region, and described a case of their own of a white woman aged 50 with a nodule of Kaposi's sarcoma in one eyelid. The present report of a similar case has the additional unusual features of bilateral occurrence of the tumors as part of the Mikulicz syndrome and the extreme youth of the patient. Attention is also drawn to the frequency of this tumor in the African. The patient, a boy aged 2 years, of the Sukuma tribe of Tanganyika, is shown in Figure 1 soon after admission to hospital. The grandmother who accompanied him stated that the condition had been present for about three months. Both eyes were entirely hidden by the everted and edematous upper

Possible sites of macroglobulin synthesis: a study made with fluorescent antibody.

SummaryThis paper describes experiments on the localization of macroglobulins by the fluorescent antibody technique in the tissues of two patients, one showing Mikulicz's syndrome and the other suffering from myeloma.The first patient had the macroglobulin in large quantities in mature and immature plasma cells in the bone marrow. It was considered that the macroglobulin was a product of a plasmacytosis reactive to the pathological process in the patient's parotid gland.The second patient had both a macroglobulin and a myeloma globulin in his serum. The fluorescent antibody studies suggested that these two abnormal proteins originated in different plasma cells in the bone marrow, which were, however, morphologically identical with orthodox staining. Neither protein could be demonstrated in frozen sections of other tissues obtained at autopsy.

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

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.

HAIR LOSS AND PIGMENTATION DUE TO THIOURACIL DERIVATIVES

Among the side reactions associated with the administration of the therapeutic thiouracil derivatives, propylthiouracil and methylthiouracil, in the treatment of hyperthyroidism have been leucopenia and granulocytopenia, 1 agranulocytosis, 2 anemia, 3 thrombocytopenic purpura, 4 dermatoses including pruritis and urticaria, 5 fever, 6 menstrual changes, 7 and others such as nausea, headache, arthralgia, numbness in the extremities, jaundice, Mikulicz's syndrome, and lesions resembling periarteritis nodosa. However, with the exception of a recent inquiry 8 in the Queries and Minor Notes section ofThe Journalrelative to hair loss, there appears to be no report of hair loss or pigmentation directly attributable to methylthiouracil or propylthiouracil. The following is a rather striking instance of hair loss and, of lesser importance, yellowish pigmentation of the hair, the former of such pronounced degree as to necessitate discontinuance of these drugs. REPORT OF A CASE K. W., 67, a white woman, was first seen April

The Osseous Lesions of Sarcoidosis

In the fifty years which have passed since Caesar Boeck (1) first described an apparently rare dermatologic condition which he chose to call “multiple benign sarkoid of the skin,” this disease has come to be recognized as a generalized systemic disorder of interest and importance not only to dermatologists but to physicians in virtually all branches of medical practice. On occasion sarcoid lesions apparently confined to the skin are encountered, but the consistency with which deep-seated sarcoidosis occurs with or without skin alterations warrants thorough investigation of all organ-systems of patients in whom the disease is suspected. The evolution of sarcoidosis as we now recognize it has been carefully documented by many writers, notably Hunter (2) and Longcope and Pierson (3). Their accounts of Hutchinson's “Mortimer's malady,” Besnier's lupus pernio, Boeck's multiple benign sarcoid, Heerfordt's uveoparotid fever, Schaumann's lymphogranuloma benignum, Jüngling's osteitis tuberculosa multiplex cystica, and certain instances of the Mikulicz syndrome, and how all these apparently specific diseases were gradually recognized as different manifestations of a single clinicopathologic entity constitute one of the most fascinating chapters in the history of medicine. Nor has the chapter yet ended, for not only do each few years bring reports of sarcoidosis involving portions of the body in which it has not hitherto been described and new tests for the determination of its presence (4, 5), but the etiology of the condition never has been satisfactorily established. Schaumann (6, 7), who made the first real attempt to correlate the various clinical and pathological manifestations of sarcoidosis, insists that the disease is primarily one of the lymphohematopoietic system, with or without involvement of the skin, and that the reporting of lesions in other portions of the body represents a failure to distinguish “what is essential and what is accidental.” Like Besnier (8), Boeck (1), and Jüngling (9), as well as the majority of modern authorities, Schaumann has consistently maintained that sarcoidosis is a peculiar manifestation of tuberculosis. One can find considerable support for this theory in the contributions of various laboratory research workers. For example, Jadassohn (10) has produced sarcoid-like lesions in the skin of rats by injection of tubercle bacilli, and Florence Sabin (11) and her co-workers have obtained non-caseating “hard” tubercles typical of sarcoid by injecting only the lipoid fraction of tubercle bacilli into laboratory animals. On the other hand, it has been found that sarcoid-like lesions can be produced locally by the injection of other organisms (12) as well as by numerous inert substances such as grass (12) and silica (13).

MIKULICZ'S SYNDROME WITH FEVER AS AN UNUSUAL COMPLICATION OF THIOURACIL THERAPY

“IODINE MUMPS” has long been known to the medical profession as a rare, toxic reaction to iodine (1). Indeed iodine has been reported as an etiologic factor in some cases of Mikulicz's syndrome (3). An analogous phenomenon has been reported to occur in patients receiving thiouracil in the treatment of thyrotoxicosis (4). The present report deals with the detailed description of a patient who developed fever and a generalized swelling of the salivary and lacrimal glands while taking thiouracil. Following recovery, a sialogram showed evidence of structural damage to at least one of the salivary glands. CASE REPORT A. L., Lab. #25523, a white female aged 50, was admitted to the medical service of the Massachusetts General Hospital on February 23, 1945. She complained of a goiter, weight loss and nervousness. In 1942 she first noticed in her neck a lump which produced no symptoms. At about the same time she was treated in another hospital with x-ray and radium for epidermoid cancer of the cervix.

Mikulicz's syndrome: Alexander J. Schaffer and A. Wilmot Jacobsen. Am. Jour. Dis. Child., September, 1927, xxxiv, 3

Mikulicz's syndrome: Alexander J. Schaffer and A. Wilmot Jacobsen. Am. Jour. Dis. Child., September, 1927, xxxiv, 3