Syndrome (disease) Mikulich: causes, symptoms, treatment, prognosis

What is Mikulich syndrome?

Syndrome (disease) Mikulich Is a chronic disorder characterized by abnormal enlargement of glands in the head and neck, including near the ears (parotid glands) and around the eyes (lacrimal glands) and mouth (salivary glands). The tonsils and other glands in the soft tissues of the face and neck may also be involved. Although Mikulicz disease is almost always described as a benign condition, it always occurs in association with another underlying disorder such as tuberculosis, leukemia, syphilis, Hodgkin's lymphoma, lymphosarcoma (non-Hodgkin lymphoma), Sjogren's syndrome or lupus erythematosus (SLE). People with Mikulicz syndrome are at increased risk of developing lymphomas.

Some people with Mikulicz syndrome may experience recurring fevers. Fever can be accompanied by dry eyes, decreased lacrimation, and inflammation of various parts of the eyes (uveitis). The classic signs are an enlarged lacrimal gland, an enlarged parotid gland, dry mouth, and dry eyes.

The exact cause of Mikulich's syndrome is not known. Some scientists believe that Mikulich's disease should be considered a form of Sjogren's syndrome.

Signs and symptoms

Mikulich disease is characterized by the sudden onset of extreme dry mouth (xerostomia), which can lead to difficulty swallowing (dysphagia) and tooth decay. Other symptoms include:

• enlargement of the lacrimal glands, which leads to the absence or decrease in tears;
• Enlarged glands in the neck (parotid glands)
• painless inflammation of the salivary glands.

The glands near the jaw (submandibular) can also swell. Symptoms can persist for long periods of time or come and go with frequent relapses. The symptoms of Mikulicz's disease are very similar to those of Sjogren's syndrome, and some researchers suspect that they may be the same disorder (see below). (See section "Related Disorders" in the article).

Causes and risk factors

The exact cause of Mikulich's syndrome is unknown, although it is suspected that it is autoimmune disease. Autoimmune disorders occur when the body's natural defenses against "foreign" or invading organisms (for example, viruses, bacteria) begins to attack healthy tissues by unknown reasons.

Symptoms of Mikulicz syndrome can occur due to an excessive accumulation of certain white blood cells (lymphocytes) in many glands of the face, mouth and / or neck.

Affected populations

Mikulicz disease affects more women than men and is most often seen in middle age.

The disorder often occurs in conjunction with Sjogren's syndrome. Some scientists have suggested that Mikulicz syndrome and Sjogren's syndrome may in fact be the same disorder.

Related disorders

Symptoms of the following disorders may be similar to those of Mikulich's disease. Comparisons can be useful for differential diagnosis:

• Piggy (parotitis) Is an acute viral disease that causes painful inflammation and swelling of the salivary glands, including the parotid, submandibular, sublingual, and buccal glands. At one time, mumps was a common infectious disease in children. However, in 1967 a vaccine was developed against the disease. The onset of the disease is characterized by headache, loss of appetite, a general feeling of not feeling well (malaise) and high or low fever. Within 24 hours, the temperature rises to about 40 degrees and usually occurs with pain and swelling of the glands in the front of the ears (parotid and under the jaw (submandibular).
• Heerfordt's Syndrome (uveoparotid fever), similar to Mikulich's syndrome and is sometimes the first symptom of another disease, sarcoidosis of the lungs. Heerfordt syndrome is characterized by swelling of the glands in the front of the ears (parotid glands) and paralysis of one or more cranial nerves. The nerves of the face are most often affected.
• Sjogren's Syndrome - an autoimmune disease characterized by progressive degeneration of the mucous secreting glands, especially the mouth (salivary) and eyes (lacrimal). Symptoms of Sjogren's Syndrome usually onset suddenly and may include inflammation of the membranes surrounding the eyes and cornea (keratoconjunctivitis). Various degrees of dry mouth (xerostomia) can cause difficulty swallowing and / or dental disease.
• Swelling of the glands in the parotid region can occur for many different reasons. Painless swelling on both sides of the face can occur without fever and in combination with other disorders such as Laenneck's cirrhosis, chronic alcoholism, malnutrition, diabetes, pregnancy, lactation and / or hypertriglyceridemia. Malignant and benign tumors of the salivary glands can also cause swelling of the salivary glands. Parotid enlargement can also be associated with the use of certain medications (such as guanethidine or iodine). Obstruction of the duct from the parotid gland to the orifice (Stensen's duct) of calcium phosphates (calcification) can also cause parotid edema.

Standard treatments

A biopsy of one of the swollen glands is the key to diagnosing Mikulicz syndrome. Ultrasound examination of the area can help rule out other causes of the swelling of the gland. Treatment for this disorder is symptomatic. Medical therapy is more productively directed at treating any underlying medical condition. Artificial tears can be used to maintain moisture in the eyes, and artificial saliva can be used to treat oral symptoms.

Some people with Mikulicz disease may be prescribed a soft, wet diet. It can help relieve pain caused by chewing and swallowing. Other treatments are symptomatic and supportive.

Andrey Zakirov/ article author