Sialodochitis fibrinosa (kussmaul disease) report of 3 cases and literature review

Abstract

Background:Sialodochitis fibrinosa is a rare disease which is characterized by recurring episodes of pain and swelling of the salivary glands due to the formation of mucofibrinous plugs. Analytic studies ascertain elevated levels of eosinophils and immunoglobulin E (IgE). Imaging studies such as magnetic resonance imaging (MRI) and sialography reveal dilation of the main salivary duct (duct ectasia). Treatment is initially supportive, consisting of compressive massages, and use of antihistamines and/or corticosteroids.Material and methods:In the following, 3 cases of sialodochitis fibrinosa are presented which were diagnosed in a third level hospital during the period of 2008 and 2016, as well as a literature review of all cases reported to our knowledge.Results:Of the 41 cases found, including the 3 of this article, 66% were women with an average age of 45 years old. However, 75% of reported cases were of Japanese heritage. Involvement of the parotid glands was more frequent than the submandibular glands. In more than half of all cases treatment with compressive massages, antihistamines and/or corticosteroids was effective.Conclusion:Clinicians should consider sialodochitis fibrinosa as a diagnostic possibility when presented with cases of recurring parotid and submandibular gland tumescence.

Sialodochitis fibrinosa: Salivary duct obstruction by eosinophil extracellular traps?

“…Histopathology of Wharton's duct and submandibular gland tissue from the patient revealed epithelial dislodgment and the presence of a dense periductal lymphoplasmacytic infiltrate, consistent with findings in previous reports (Baer et al., 2017; Flores et al., 2016). Immunohistochemical staining revealed the presence of lymphocytes, primarily CD4 + helper T cells (Figure ).…”

“…Sialodochitis fibrinosa (also known as Kussmaul disease) was originally described by Kussmaul in 1879. This condition involves recurrent parotid grand swelling and mucus plugs containing leucocytes and Charcot–Leyden crystals (CLCs; Baer, Okuhama, Eisele, Tversky, & Gniadek, 2017; Flores et al., 2016; Ray, Burgin, & Spector, 2015). Sialodochitis fibrinosa also includes recurrent salivary gland swelling with fibrinous eosinophil‐rich mucus plugs.…”

Eosinophilic sialodochitis: a case report of a rare disease

“…However, sialodochitis is impossible to separate from sialadenitis clinically and the disease appears initially duct associated. The literature reported that the secretion of a fibrinous material containing numerous eosinophils from the glandular duct orifices was a distinctive finding of ES [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ]. Few histopathological investigations into ES have been published.…”

“…The etiology of ES is not well understood, although the most accepted hypothesis is that it is an allergic process [ 7 , 8 , 9 , 10 , 11 , 12 , 13 ]. Support for an allergic etiology is based on significant medical history of asthma and chronic rhino-conjunctivitis in some patients and a raised peripheral eosinophil count in affected patients [ 9 ]. In our case, we were unable to correlate symptoms with the consumption of food allergens.…”

Recurrent Submandibular Gland Swelling Relieved by Antihistamine Medication: Is It Eosinophilic Sialodochitis?

“…Although diagnostic criteria for eosinophilic sialodochitis have not been well established, specific findings commonly observed in this disease can aid its diagnosis. 3) In addition, antihistamine medication usually improves obstructive symptoms in this disease. 4) Herein, we report two cases of recurrent submandibular gland (SMG) swelling relieved by antihistamine medication, suspicious of organ-specific eosinophilic disease, with a literature review.…”