Mikulicz syndrome 4.5/5 (4)

Share Button
Clinical feature and histopathological examination of the patients (a) Swelling of bilateral upper eyelids. (b) Erythematous papular skin lesions on the right cheek. HE histopathological examination of skin lesion (c) salivary gland (d) fibrosis and infiltration of lymphocytes and plasma cells. Immunohistochemical examination of skin lesion (s) salivary gland (f) infiltration of IgG4 positive plasma cells
Mikulicz syndrome with salivary and tear glands. Takahashi H, 2018. CC BY SA 3.0

Mikulicz syndrome (Sclerosing sialo-dacryoadenite)

Definition

Mikulicz syndrome is a chronic benign disease in which both saliva glands located in front of the ears (glandula parotis) and the tear glands above the eyelids (glandula lacrimalis) swell. Most cases are flushed IgG4 related disease. One detects when elevated IgG4 is found in a tissue sample and sometimes in the blood. Similar symptoms can be seen in unusual cases of sarcoidosis (Rao D, 2017). The cause of the disease's localization is unknown, but an autoimmune process is likely. The result is that large amounts of hard, scar-like tissue can displace healthy tissue and reduce salivary and lacrimal gland function. Women are attacked more frequently than men.

Symptoms

Swollen glands and dry mouth. Swollen salivary glands in front of the ears (parotid glands) and under the lower jaws (submandibularis glands). Some suddenly notice extreme dryness in the mouth and swallowing problems because saliva production is reduced.

Symmetrical swelling above the eye (lacrimal glands) and dry eyes

Küttner's tumor caused by sclerosing sialo-adenitis, most often in a submandibular gland (benign, IgG4 related condition)-

Examinations

PET / CT, MRI and biopsy. Marginal zone B cell lymphoma that clinically mimics Mikulicz syndrome Affiliation: Section of Oral and Maxillofacial Oncology, Kyushu University Japan. CC BY 4.0

Medical history covers current symptoms such as swelling in the glands and dryness in the mouth and eyes. At the same time, symptoms from other organs that can be attacked in IgG4-related disease, such as lymph nodes, are sought.

Clinical noticeably hard but well-defined swellings are felt in the affected glands. Lymph nodes on the neck, under the arms and in the groin are also assessed. A general examination of the lungs, heart and stomach area is also done.

Laboratory tests may be inconspicuous, but for investigation one can consider measuring hemoglobin, white blood cells (leukocytes with differential counts), thrombocytes, CRP, kidney-liver-bile-pancreas and metabolism tests, immunoglobulins with sub-classes of IgG (including IgG4). Hormone tests (TSH, growth hormone/somatotropin, anti-diuretic hormone/ADH) for possible pituitary manifestations can also be considered. Antinuclear factors (ANA) are usually normal. Urine sting.

Imaging with ultrasound, alternatively an MRI examination can show the size of the swellings. PET/CT can be useful if you want to assess disease spread in the case of IgG4-related disease in arteries, salivary glands, lymph nodes and other organs. The method can thus be used for stage classification and monitoring (reference: Ebbo M, 2014).

Tissue sample (biopsy) is important to ensure the diagnosis (Deshpande V, 2012). 

Similar diseases / differential diagnoses

Mikulicz syndrome: Difference from Sjögren's syndrome:

  1. The incidence is the same for men and women (Sjøgren's is much more common among women (>10:1)
  2. Not noticeable fatigue
  3. Significantly enlarged tear and salivary glands but not chronic dry mouth (xerostomy) or dry eyes (xerophthalmia)
  4. Persistent gland enlargement
  5. At the same time other IgG4 manifestations such as autoimmune pancreatitis
  6. Increased level of IgG4 in the blood and IgG4+ plasma cell infiltration in tissues
  7. No occurrence of SSA / B antibody in blood sample
  8. Better response to corticosteroids treatment (prednisolone) compared to Sjögren's syndrome
  9. Tissue sample (biopsy) shows differences: IgG4-positive plasma cell infiltration is not typical for Sjøgren's syndrome.

Treatment

Literature


This page has had 1 visits today

Please rate this page