Mikulicz disease is a rare chronic disease, manifested by a symmetrical increase in all salivary and lacrimal glands. Among the possible etiological factors, the role of viral infection, as well as autoimmune processes leading to dystrophic lesions of the salivary and lacrimal glands is considered. More often the disease (syndrome) Mikulicz occurs in patients with blood diseases. Diagnostics is based on the study of clinical examination data, laboratory and instrumental studies, including sialography. Symptomatic treatment: radiotherapy, novocaine blockades, galantamine.
Mikulicz disease (sarcoid sialosis) is a reactive–dystrophic lesion of the salivary and lacrimal glands, leading to their hypertrophy. The main factors contributing to the development of the disease are viral infection, allergic and autoimmune processes, diseases of the blood and lymphatic system. Mikulicz disease occurs exclusively in adults, and most often in women. The disease was first described in 1892 by the German surgeon I. Mikulicz. Currently, it is believed that the observed enlargement of the glands is not an independent disease, but a syndrome occurring in various diseases of the endocrine system and blood diseases.
The etiological factors leading to the development of Mikulicz disease are still unknown. The role of viral infection, allergies, autoimmune and endocrine disorders, and blood diseases are considered as possible causes. Systemic damage to organs and systems of the body causes a violation of the neurovegetative regulation of the salivary and lacrimal glands, a change in their secretory function. Allergic or autoimmune reactions in Mikulicz disease gradually lead to blockage of the excretory ducts of the glands by eosinophilic plugs and secretion retention, reduction of myoepithelial and smooth muscle cells of the ducts, proliferation of lymphoid and interstitial tissue squeezing the ducts and gradual progressive hypertrophy of the salivary and lacrimal glands.
Mikulicz disease is characterized by a gradual symmetrical increase in the salivary and lacrimal glands. First of all, hypertrophy of the parotid glands develops, which leads to a change in the oval of the face, then the pathological process spreads to other salivary and lacrimal glands. The skin at the same time retains its usual color, when probing the glands are dense, there may be a slight soreness.
With an increase in the submandibular salivary glands, the process of eating, as well as colloquial speech, is often difficult. Salivation is not disturbed at first, only in the later stages of Mikulicz disease there is a decrease in the secretory function of the salivary glands with the appearance of dry mouth, the development of taste disorders, difficulty swallowing. Enlarged lacrimal glands lead to swelling and drooping of the upper eyelids, narrowing of the eye slits. Vision is not impaired.
A long-lasting decrease in salivation or its complete cessation in Mikulicz disease is often complicated by the addition of ulcerative stomatitis, glossitis, gingivitis, frequent damage to the oral mucosa when eating, problems with the operation of removable prostheses.
Changes from the internal organs in Mikulicz disease, as a rule, are not noted. If Mikulicz syndrome is diagnosed, that is, an increase in the salivary and lacrimal glands due to chronic lymphoproliferative diseases (lymphomas, lymphogranulomatosis, etc.), symptoms characteristic of the underlying disease will be observed in the clinical picture.
Diagnosis of Mikulicz disease begins with a thorough collection of anamnesis (usually patients go to the doctor with complaints of dry mouth), a clinical examination, during which a symmetrical increase in salivary and lacrimal glands, dense consistency of hypertrophic areas, uneven contours, lumpiness, slight soreness, drooping of the upper eyelids, narrowing of the eye slits is detected.
With Mikulicz syndrome, itching of the skin, an increase in regional lymph nodes (more often axillary and inguinal), transient fever, general weakness may bother. In the process of diagnosis, the patient should be examined by specialists of various profiles – a dentist, a hematologist, an oncologist, an allergist, an immunologist, an endocrinologist, a rheumatologist.
Laboratory blood tests may show a pattern characteristic of lymphoproliferative diseases, a general urinalysis is usually without pathological changes. Sialography reveals signs of narrowing of the ducts of the salivary glands, dystrophic changes in glandular tissue with the appearance of small and large oval-shaped cavities in the form of grapes. Computed tomography helps to clarify the size and structure of the salivary glands, as well as to exclude the presence of a malignant neoplasm. A biopsy followed by histological examination of the biopsy of the salivary and lacrimal glands reveals the presence of pronounced lymphoid infiltration of the stroma, atrophic changes in the parenchyma and intra-flow proliferation.
To diagnose Mikulicz disease, an immunological and immunochemical study is carried out, followed by an examination by an allergist-immunologist, as well as an ophthalmologist’s consultation with the Schirmer test and a sample with fluorescein. It is necessary to conduct a differential diagnosis of Mikulicz disease with other sialadenoses, including hormonal, neurogenic, alimentary, Sjogren’s disease, tumors (lymphomas) of the salivary and lacrimal glands, sarcoidosis, amyloidosis, lymphogranulomatosis and other diseases.
Etiotropic treatment of Mikulicz disease has not been developed. Most often, X-ray therapy is used, which provides a temporary reduction in the size of the salivary glands, relief of the inflammatory process and restoration of the secretory function of the glands with the elimination of dryness of the oral cavity (remission can last 3-6 months). The often used therapeutic measures for Mikulicz disease include novocaine blockades in the salivary glands, as well as the use of galantamine in the form of injections.
If patients with Mikulicz disease have concomitant lymphoproliferative processes and systemic connective tissue diseases, the prognosis becomes unfavorable.