What Is Mikulicz Disease?
Mikulicz's syndrome may be defined as the diffuse and bilateral enlargement of the salivary and lacrimal glands. This disease is named after the Polish surgeon Jan Mikulicz-Radecki (1850-1905), who first described its clinical features in 1892.
What Is the Etiology and Pathogenesis of Mikulicz Disease?
Mikulicz disease or syndrome can occur due to a variety of specific diseases or autoimmune disorders due to elevated IgG4 antibody concentrations, mainly affecting the salivary and lacrimal glands. The systemic conditions listed below that can cause this condition are: sarcoidosis, tuberculosis, syphilis, mumps, Graves disease, malignant lymphoma, leukemia, or associated with lymphocytic infiltration and formation of lymphoepithelial lesions historically known as Mikulicz disease.
However, as per research, the most common cause is lymphoepithelial sialadenitis which is why this term is coined LESA. It was formerly referred to as myoepithelial sialadenitis (MESA). LESA is the same autoimmune phenomenon and is now referred to as Mikulicz disease. It is termed an immunoglobulin G4 (IgG4) systemic disease. The specific characteristic distinguishing this disease is elevated IgG4 serum concentrations and infiltrations of plasmacyte cells that express IgG4 into the lacrimal and salivary glands. This condition is also very closely related or is in fact the other term for Sjögren syndrome.
In most cases, it is just a different form of the same condition characterized by the two features of dry eyes and dry mouth. Dry eyes can either lead to blurry vision or intolerance of bright light. Dry mouth is due to reduced salivary secretions that may result in a burning sensation of the mouth, sores, or alterations at the corners of the mouth (as saliva acts as a defense mechanism in the oral cavity, and its secretion is reduced here. The predilection is that people with Mikulicz syndrome and Sjögren syndrome are also at an increased risk of developing non-Hodgkin lymphoma. This disease commonly tends to affect females more than males and mainly occurs in the adult population over the age of 50 years).
What Are the Clinical Features of Mikulicz Disease?
Mucocutaneous symptoms are often the first clinical findings in patients with this disease as it is a systemic autoimmune disorder. It primarily affects the secretory glands. Dryness (xerosis) of the mucous membranes in context with the other diagnostic criteria is a key factor for the diagnosis of a multisystemic manifestation. Xerosis can involve mainly the mouth (xerostomia) and the eyes (leading to keratoconjunctivitis sicca). Vaginal dryness can also be observed in females. Regarding the oral cavity, patients complain of dryness, soreness, and burning sensations along with oral ulcerations that may be common. Vaginal xerosis can likewise result in dryness, burning, dyspareunia, or recurrent genital pain in that region. Because of this reduced salivary secretion, angular stomatitis of the mouth is another observable oral manifestation by the dental surgeon, and the patient may often report a candida infection. In addition to the mentioned signs in the mucous membranes, the skin manifestations that are the presenting or even preceding disease's features are extremely common and should prompt investigation for an earlier diagnosis. Xerosis cutis or simply dry skin is most common in almost 50 % of affected patients. It may lead to generalized uncomfortable itching or scratching sensation of the skin or pruritus. Changes in the walls of the blood vessels due to their inflammation are known as the phenomena of vasculitis that remains a predominant feature in this disease. The physician may thus observe either cutaneous or other forms of noncutaneous vasculitis as well. This results from the immune complex mechanisms in this disease. These manifestations include palpable and nonpalpable purpura (purple spots or tiny bleeding spots) on the legs and buttocks. If the vasculitis is of cutaneous type, this includes lymphocytic vasculitis and urticarial vasculitis. Though rarer, cases of urticarial lesions do occur, and in this type of vasculitis, the symptoms last longer than two days with an associated purpuric component. Patients frequently complain of burning and painful sensations in different body parts because of persisting vasculitis.
What Is the Histopathological Study of Mikulicz Disease?
In gross observation of the gland, it is found to be not only enlarged and whitish but also found with occasional cysts. On microscopic examination, the cardinal changes are marked with lymphoid infiltration and specific epimyoepithelial cell islands. This combination of findings was responsible for cloning previously used terminology, such as myoepithelial sialadenitis (MESA), recently modified to lymphoepithelial sialadenitis (LESA). The lymphoid tissue also contains well-formed multiple germinal centers and is composed of a mixed population of B and T lymphocytes. Further, it can be accompanied by scattered histiocytes and dendritic cells. The presence of epimyoepithelial islands appears as solid epithelial nests surrounded and infiltrated by lymphoid cells, which are mainly of monocytoid B type along with deposition of hyaline substance in between the cells.
What Are the Radiographic Findings of Mikulicz Disease?
CT (computed tomography) scan and MRI (magnetic resonance imaging) are recommended for diagnostic confirmation by the physician if needed. In a CT scan, a symmetrical but diffuse bilateral enlargement of the parotid gland, submandibular glands, or lacrimal glands is evident. The lymph node involvement is usually non-specific. In MRI, high cellularity and fibrosis are observable as low T2 signal intensity can give a confirmatory diagnosis of Mikulicz disease (in the region of either the lacrimal or the parotid glands).
How to Manage Mikulicz Disease?
Treatment usually consists of observation of the clinical symptoms by the physician initially unless the patient has concerns of eye-related or oral discomfort because of pain, dryness, irritation, or burning sensation. Surgical removal of the affected gland would be recommended in only those patients where the risks observed for systemic malfunction are higher.
Another treatment option by the physician or surgeon is aspiration from the affected gland, which can be repeated multiple times, especially in those who are debilitated or in those whose benefit from surgery would be outweighed by the risks.
Prognosis is usually good in the treatment of this disease. Therapies suggested or directed by the physician mainly alleviate the symptoms of dry eyes and mouth. Ophthalmic ointments, artificial tears, medicated sprays, medications that increase saliva production (salivary stimulants or supplements, mouthwashes), and for the production of tears may be recommended by the dental surgeon or the ophthalmologist accordingly based on the gland affected. A dermatologist can also be consulted regarding dry skin symptoms in mucocutaneous lesions. Clinicians also generally encourage lifestyle changes in persistent cases of the disease, and the patient should be advised and motivated to quit alcoholism, smoking, and caffeine. They can be detrimental and worsen the symptoms of dry eyes and dry mouth.
Conclusion:
The Mikulicz disease is generally a long-term and chronic disease because of its autoimmune nature, and careful observation of clinical symptoms by the physician along with monitoring the situation of dry eyes and dry mouth by the ophthalmologist, dental surgeon, and the dermatologist will aid in a good prognosis for the patient.
