SIALODOCHITIS FIBRINOSA OF KUSSMAUL: A CASE PRESENTATION

Abstract

<h3>Objective</h3> Sialodochitis fibrinosa of Kussmaul is a rare phenomenon that was first reported in 1879. It is characterized by recurrent major salivary gland swelling in patients with atopic background. Treatment includes antihistamines, systemic glucocorticoids, massage, mucolytic agents, sialendoscopy, and surgery. To date, less than 70 cases have been reported in the literature. <h3>Case Summary</h3> A 42-year-old woman was referred from the emergency room to the Oral Medicine unit at Sheba Medical Center with the chief complaint of severe xerostomia and asphyxia due to submandibular swelling. Her medical history was significant only for asthma. The patient reported facial and submandibular swelling accompanied by severe pain, 3 weeks before her presentation. Two months earlier, she had developed urticaria, which was treated with antihistamines. The patient denied any symptoms associated with xerophthalmia. Complete blood count as well as lipase, IgG4, and angiotensin converting enzyme (ACE) levels were within normal limits. Blood levels of amylase and C-reactive protein were elevated. Virology (hepatitis B virus, cytomegalovirus, hepatitis C virus, human immunodeficiency virus [HIV], measles, mumps) and autoimmune panel were negative. Salivary glands ultrasound failed to demonstrate any pathologic findings. Clinical examination revealed enlarged cervical lymph nodes, bilateral mild swelling posterior to angle of mandible, perioral rash, dry tongue covered with white coating, erythema around the orifices of parotid ducts, and labial minor salivary glands enlargement. There was no secretion upon milking of major salivary glands. After stimulation with citric acid, foamy saliva secretion was noted, and on milking a mucous plug was released followed by clear saliva secretion. Sialography demonstrated dilated parotid gland ducts. No focal lymphocytic infiltration was noticed on minor salivary gland biopsy. Cytology of the mucous plugs demonstrated the presence of eosinophils. In the light of salivary glands swelling, dilated parotid ducts, the presence of eosinophils within the mucous plugs, and atopic background, the diagnosis of sialodochitis fibrinosa of Kussmaul was made. <h3>Conclusions</h3> Sialodochitis fibrinosa should be included in the differential diagnosis of patients presenting with sialadenitis, recurrent swelling of major salivary glands, and atopic background.