Mikulicz syndrome and disease

Miculicz's disease (synonyms: sarcoid sialosis, Miculicz's allergic reticuloepithelial sialosis, lymphomyeloid sialosis, lymphocytic tumor) is named after the physician J. Miculicz, who in 1892 described an enlargement of all major and some minor salivary glands, as well as lacrimal glands, which he observed over 14 months in a 42-year-old peasant.

The author wrote that the disease began approximately six months before the observation began with swelling of the lacrimal glands. The palpebral fissure narrowed, making it difficult to see with a halo. There were no other subjective sensations. Soon the submandibular glands enlarged, which interfered with talking and eating, and later the parotid glands did the same. Vision was not impaired. The enlarged salivary glands had a dense-elastic consistency, were painless, and moderately mobile. There was a lot of saliva in the oral cavity, and the mucous membrane was not changed. The author performed a partial resection of the lacrimal glands, which soon increased again to their previous size. Only after complete removal of the lacrimal and submandibular glands could the peasant return to his work and feel well. However, after 2 months he fell ill and died of peritonitis on the 9th day. The parotid and minor salivary glands, which had enlarged somewhat after the operation, began to quickly decrease before death and then completely disappeared. During the pathohistological examination of the submandibular glands, I. Mikulich established that the entire gland had a normal structure; it could be divided into lobes and lobes. On the section, the tissue of the gland differed from the normal by the poverty of vessels, had a pale reddish-yellow color. The gland was of a soft consistency, had a greasy transparent surface. Microscopically unchanged acini were moved apart by round-cell tissue, the cells of which were of different sizes. Between them there was a thin endoplasmic reticulum. In larger cells, mitoses could be recognized. A similar picture was observed in the lacrimal glands.

Epidemiology of Mikulicz disease

This is a rare disease, observed in adults, mainly in women. In childhood, it is not noted. It is often diagnosed in patients with blood diseases (lymphogranulomatosis) or is their precursor.

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Causes of Mikulicz's disease

The causes of Mikulicz's disease are unknown, but a viral infection and a blood disease (lymphogranulomatosis) are suspected.

Symptoms of Mikulicz disease

The clinical picture is characterized by a slow and painless significant enlargement of all salivary and lacrimal glands (as described by I. Mikulich in 1892). The skin over the glands does not change in color. When palpated, the glands are of a dense, elastic consistency. The function of the salivary and lacrimal glands, unlike Sjogren's syndrome, does not change. The mucous membrane of the oral cavity does not change in color. Saliva is released freely when massaging the salivary glands. No signs of damage to internal organs are detected.

Diagnosis of Mikulicz disease

In Mikulicz syndrome, lymphoproliferative changes in the blood system may be detected. Laboratory parameters of urine remain within normal limits. Sialograms reveal signs of interstitial sialadenitis with narrowing of the gland ducts. Biopsies of the salivary and lacrimal glands reveal a pronounced lymphoid infiltrate, which compresses the ducts without destroying the basement membranes and without replacing the acinar tissue.

Treatment of Mikulicz's disease

Treatment of Mikulicz's disease (syndrome) is mainly symptomatic. X-ray therapy is used, which gives a temporary effect, after which the salivary glands increase again, and long-term dryness of the oral cavity may occur. Novocaine external blockades in the area of the salivary glands, galantamine injections, etc. are also used for treatment. Treatment is considered effective in the case of a significant reduction in salivary glands over a long period, the absence of xerostomia and exacerbation of sialadenitis.

Prognosis of Mikulicz's disease

The prognosis is unfavorable. Relapse is quite common. During the observation dynamics, various blood diseases or other severe pathological processes in the body are detected.