Mikulicz disease is characterized by the chronic and benign enlargement of the glandular structures situated in the head and neck, with the parotid, lacrimal and other salivary glands amongst the most commonly affected structures.
This condition, if primary, has been historically designated as Mikulicz disease. Although benign, this condition is almost always found along with certain other underlying diseases, such as tuberculosis, leukemia, syphilis, hodgkin's disease, sarcoidosis, systemic lupus erythematosus or Sjogren's syndrome. The appropriate terminology for this condition then becomes Mikulicz syndrome [1]. Such designations are now considered outdated and ambiguous. Some authors believe that Mikulicz disease, Mikulicz syndrome as well as Sjogren's syndrome all refer to the same condition- an autoimmune lymphocytic inflammation of the salivary and lacrimal glands due to a hyperactive immune response [2] [3] [4].
Owing to the inflammatory processes occurring in the enlarged parotid and other salivary glands, Mikulicz disease patients are usually affected by xerostomia of a sudden onset that may present as dysphagia or dental problems. The enlargement of the lacrimal glands, on the other hand, may lead to a reduction or absence of tear secretion.
Painless swellings of the parotid and other salivary glands may be found in these patients. Other glands, such as the submaxillary and the buccal glands, may also be involved. Uveitis is another commonly seen clinical sign. This disease is usually long-lasting and recurring fevers may be observed in some patients [5] [6].
Mikulicz disease may also be known by a variety of names including Dacryosialoadenopathia, Mikulicz-Radecki syndrome, Mikulicz-Sjogren's syndrome. Several other conditions, having similar clinical manifestations, should be considered in the differential diagnosis of Mikulicz disease.
Mumps, an acute viral disease of childhood was a leading cause of enlargement of the salivary glands before the advent of prophylactic mass vaccinations. Unlike Mikulicz disease, the inflammation of the glands is usually painful in mumps. The first symptoms may further include malaise, loss of appetite and a low-grade temperature that rises quickly within the first 24 hours.
Heerfordt’s syndrome, characterized by the enlargement of the parotid glands accompanied by the paralysis of the facial nerve should also be excluded. It is an early feature of sarcoidosis.
Salivary gland duct obstruction due to stones may also be a cause for glandular enlargement and should be considered in the differential diagnosis. Other reasons for salivary gland swelling include malnutrition, chronic alcoholism, pregnancy and the use of certain medications such as iodine. Salivary gland tumors, both benign and malignant, should also be ruled out while making a diagnosis [7] [8].
Mikulicz disease is suspected in patients presenting with a simultaneous enlargement of the parotid and lacrimal glands.
Biopsy may be required to establish the reason for the enlargement of these glands. Histological findings in Mikulicz disease include lymphocytic infiltration in the salivary and lacrimal glands. Autoantibodies, such as anti-Ro, anti-La, antinuclear antibodies (ANA) and rheumatoid factor may also be detected in the serum.
Dry eye may be objectively diagnosed by certain tear flow dynamic tests, including the schirmer test, cotton thread test, clearance test or the tear break-up time test. Changes in the ocular surface may be detected via staining with the rose bengal or fluorescein stains [9] [10].