A case of sialodochitis fibrinosa (Kussmaul's disease) bilaterally affecting the submandibular glands

Abstract

Sialodochitis fibrinosa (Kussmaul's disease) is a rare disease characterized by recurrent swelling of the salivary glands due to obstruction of the duct system by a fibrinous plug. This paper describes a 58-year-old woman with sialodochitis fibrinosa bilaterally affecting the submandibular glands. The patient had recurrent swelling of the submandibular glands. Histopathological examination revealed numerous eosinophils and epithelial cells in a fibrinous plug extruding from Wharton' s ducts. Symptoms were relieved by enlargement of the ori-

Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

“…The mucus plugs measured up to 3 cm in length (Pearson, ; Yoshioka et al , ) with variable consistency: sometimes gelatinous (Shimada et al , ; Pollak et al , ; Tsuchiya et al , ), but sometimes firm enough to form casts of the ducts (Motosugi et al , ). Charcot‐Leyden crystals were noted in the plugs in one case (Nemoto et al , ).…”

“…Table summarizes the key clinical features of 59 cases of eosinophilic sialodochitis, including the three cases from our clinics and 56 cases from the literature (46 from Japan) who met our case definition (Pearson, ; Waldbott and Shea, ; Bookman, ; Serafini, ; Swinny, ; Riley, ; Cohen, ; Okuda et al , ; Miyazaki and Kumagami, ; Murakami et al , ; Yoshida et al , ; Ogawa et al , ; Watanabe et al , ; Irifune et al , ; Ishii et al , ; Kiuchi et al , ; Tanimoto et al , ; Yoshioka et al , ; Motosugi et al , ; Fujii et al , ; Yamashita et al , , ; Darling et al , ; Kishimoto et al , ; Yamamoto et al , , ; Ikegami et al , ; Kinoshita and Sadanaga, ; Tsutsumi et al , ; Shino et al , ; Chikamatsu et al , ; Nemoto et al , ; Otsuka et al , ; Shimada et al , ; Kato et al , ; Konno et al , ; Pollak et al , ; Takahashi et al , ; Nakajima et al , ; Shinkawa et al , ; Shirasaki et al , ; Tsuchiya et al , ; Nagita, ; Uno et al , ; Usami et al , ). We excluded reports and series in which key phenotypic features of individual cases were not provided.…”

“…Similarly, it excluded cases of eosinophilic sialoadenitis marked by a different histopathology, including granulomatous inflammation of the minor salivary glands (Irie et al, 2004), Kimura's disease (Li et al, 1996), and angiolymphoid hyperplasia of the parotid gland (Hung et al, 2001). Table 2 summarizes the key clinical features of 59 cases of eosinophilic sialodochitis, including the three cases from our clinics and 56 cases from the literature (46 from Japan) who met our case definition (Pearson, 1935;Waldbott and Shea, 1947;Bookman, 1950;Serafini, 1951;Swinny, 1953;Riley, 1956;Cohen, 1965;Okuda et al, 1975;Miyazaki and Kumagami, 1981;Murakami et al, 1982;Yoshida et al, 1994;Ogawa et al, 1995;Watanabe et al, 1995;Irifune et al, 1996;Ishii et al, 1996;Kiuchi et al, 1996;Tanimoto et al, 1997;Yoshioka et al, 1997;Motosugi et al, 1998;Fujii et al, 2000;Yamashita et al, 2001Yamashita et al, , 2012Darling et al, 2002;Kishimoto et al, 2002;Yamamoto et al, 2002Yamamoto et al, , 2004Ikegami et al, 2003;Kinoshita and Sadanaga, 2003;Tsutsumi et al, 2003;Shino et al, 2005;Chikamatsu et al, 2006;Nemoto et al, 2006;Otsuka et al, 2006;…”

Case of Suspected Sialodochitis Fibrinosa (Kussmaul’s Disease)

“…Sialodochitis fibrinosa is characterized by recurrent swelling of the salivary glands, which may also be painful 4,5,[9][10][11] . Pressure on the salivary glands when swollen induces discharge of white fibrin clots, which then alleviates any pain.…”

“…It is charac-terized by episodic swelling of the salivary glands, which then discharge clots of fibrin into the oral cavity 5) . Although firm diagnostic criteria remain to be established, a number of studies have listed the following as typical findings in this disease: (1) episodic swelling of the salivary glands; (2) discharge of plugs containing numerous eosinophils from the main ducts; (3) elevated levels of eosinophils and IgE; (4) concomitant presence of other allergic disorders; (5) stenosis and irregular dilatation of the main duct evident on ptyalography; and (6) hypertrophy of the main ducts, edema, and lymphocyte infiltration of the interstitium surrounding the salivary ducts 3,4,9,11) .…”