An Atypical Case of Parotid Swelling

A previously well 2-year 11-month-old boy presents to the emergency department with acute-onset left facial swelling of fewer than 24 hours’ duration and the complaint of left cheek pain. His parents report a low-grade fever but no rhinorrhea, cough, nasal congestion, or other respiratory tract infection symptoms. His oral intake is decreased, attributed to his cheek discomfort. His medical history is notable for mild eczema and IgE-mediated food allergy to eggs, for which he is followed by the allergy service at the institution of presentation. He has undergone no previous surgery or required any previous hospitalizations. He takes no daily medications other than topical moisturization for his eczema. He has no known medication allergies. He is an only child who lives with his parents; there are no pets in the home. He attends child care but has had no known sick contacts. His family history is noncontributory, with no known atopy. His immunization status, inclusive of measles, mumps, and rubella, is up to date.On physical examination he is afebrile with evident swelling of the left lateral face anterior to the tragus at the angle of the mandible. There is no erythema of the overlying skin and minimal tenderness to palpation. The left auricle, external ear canal, and tympanic membrane are normal. His oral cavity, oropharyngeal, and neck examinations are likewise normal, with no palpable lymph nodes. Mild eczema is noted on skin examination. His systemic examination findings are otherwise normal.Ultrasonography evaluation reveals bilateral, left greater than right, parotid gland enlargement with loss of the normal echotexture, multiple hypoechogenic nodules, and mildly increased vascularity on color Doppler examination. Enlarged left intraparotid lymph nodes and bilateral enlarged cervical lymph nodes are also noted. The submandibular glands are normal in appearance.A diagnosis of bilateral, predominantly left-sided, viral parotitis is made. Symptomatic treatment with analgesics is recommended.The child improves in the interim with diminution but not complete resolution of his left lateral facial fullness. He returns, however, to the emergency department 3 weeks later due to a 3-day history of recurrent, progressively worsening, left facial swelling. He has again been otherwise well with no fever or respiratory tract infection manifestations. His systemic examination remains normal. His pertinent head and neck findings are as follows.Otolaryngology consultation is requested and confirms an approximate 1.5-cm, firm, nontender, preauricular mass overlying the left mandibular angle. Bilateral cervical lymphadenopathy and a very subtle asymmetry of the left oral commissure and lower eyelid are also noted. Whereas clear saliva is expressed from the right parotid duct, discolored secretions are expressed from the left parotid duct and sent for culture.Repeated ultrasonography reveals progressive enlargement and abnormal echotexture of both parotid glands, left worse than right, as well as persistent bilateral cervical lymphadenopathy. New-onset enlargement and abnormal echotexture of the bilateral submandibular glands is additionally noted (Fig 1). Notably, no calculi are identified.A differential diagnosis inclusive of juvenile recurrent parotitis of possible autoimmune disorder etiology, recurrent parotitis secondary to salivary duct obstruction, or bacterial superimposed on viral parotitis with secondary lymphadenopathy is discussed.Juvenile recurrent parotitis is characterized by recurrent episodes of parotid gland swelling—typically over years—often (but not always) accompanied by fever and discomfort. The condition most commonly occurs in children 3 to 6 years of age. The etiology is not fully understood, with both local (congenital ductal malformation) and systemic (autoimmune disorder manifestation) causes considered. This child’s bilateral glandular enlargement noted on ultrasonography raises concern of a systemic process, but the time course is premature for definitively making a diagnosis of juvenile recurrent parotitis.Recurrent parotitis can additionally occur secondary to ductal obstruction, either due to congenital stricture or stone (sialolith) formation. The time frame is again limited with respect to making this diagnosis, and no calculi are identified on ultrasonography.Given the previous presumptive diagnosis of viral parotitis, and the thicker secretions expressed from the left Stenson duct on parotid palpation, a superimposed bacterial parotitis was considered most likely, and a therapeutic course of amoxicillin-clavulanate was prescribed.The child is seen for an outpatient otolaryngology examination 1 week later. He has been asymptomatic and afebrile with residual left, nontender, parotid gland swelling. His bilateral parotid and submandibular ducts appear normal, with clear saliva. Left anterior and posterior cervical lymphadenopathy of insignificant size dimensions is evident on neck palpation. He demonstrates symmetrical bilateral facial nerve function. The previously obtained left parotid duct secretion culture yielded normal oral flora. Completion of the amoxicillin-clavulanate course is recommended.The following day his mother calls to report that he has developed mild lip swelling and a nonpruritic rash over his face, chest, and abdomen within 12 to 24 hours of completing his 7-day amoxicillin-clavulanate treatment course. The family denies any exposure to eggs, his only known allergy. Close observational monitoring is recommended, and follow-up with his pediatrician and allergist is advised.His parents page the on-call allergy service the same night, prompting another emergency department evaluation. On examination, he is noted to have a morbilliform rash with mild lip angioedema. His family denies stridor, wheezing, dysphagia, vomiting, or any additional respiratory or gastrointestinal symptoms. He is afebrile and well-appearing, with no swelling of his tongue or soft palate. An adverse drug reaction to the amoxicillin-clavulanate is diagnosed. Given the patient’s relatively mild rash and stable condition, he is discharged on as-needed diphenhydramine. Initial follow-up with his primary care provider is advised to ensure rash resolution, and subsequent follow-up with his allergist is recommended.The allergy service is paged again the following evening due to a worsening rash involving his face, trunk, and extremities. Given confirmation of a progressive rash documented in pictures electronically forwarded by his family, a repeated emergency department evaluation is arranged, and a dermatology consultation is requested. He appears mildly itchy but in no acute distress. He remains afebrile. A diffuse erythematous, maculopapular, nonblanching rash of the face, torso, back, and extremities (Fig 2), with sparing of his palms and soles, is confirmed. He has redness and swelling of the lips but no additional mucosal involvement. A drug reaction without evidence of Stevens-Johnson syndrome or DRESS (drug reaction with eosinophilia and systemic symptoms) is suspected. A diagnostic laboratory evaluation is performed, with additional studies obtained approximately 10 hours later, before admission for observation (Table).Complete blood cell count reveals a mild leukocytosis without eosinophilia but with an elevated atypical lymphocyte percentage. Erythrocyte sedimentation rate and C-reactive protein level are normal, as are complement levels and urinalysis. Liver function tests reveal mildly elevated alanine aminotransferase and aspartate aminotransferase levels. A rapid mononucleosis screen proves positive, and both IgG and IgM antibodies to Epstein-Barr virus (EBV) capsid antigens are elevated.In the context of these laboratory studies and the morbilliform rash in association with recent amoxicillin-clavulanate exposure, his final diagnosis is retrospectively determined to be sialadenitis and lymphadenitis of EBV etiology. Systemic cetirizine as well as topical triamcinolone ointment are recommended as supportive measures for symptomatic relief of itching.EBV, or human herpesvirus 4, is associated most with infectious mononucleosis. Fever, pharyngitis, tonsillitis, and cervical lymphadenitis are the classic clinical manifestations. Parotitis is rarely a presenting feature of infectious mononucleosis, with a few case reports in the literature. (1)(2).Viral parotitis is common and typically of paramyxovirus etiology in unvaccinated individuals. Alternative viral etiologies in vaccinated children include coxsackieviruses, influenza A, and parainfluenza viruses in addition to EBV. (3) EBV has been implicated as having a potential pathogenic role in recurrent parotitis. (4)The role of antibiotic treatment in acute parotitis is debatable. (5) Antibiotics are contraindicated, unless there is a concurrent alternative indication, in the setting of suspected or established infectious mononucleosis given the risk of rash development. (6) The biological reason for this well-documented clinical observation is unknown. (7) Ironically, the use of amoxicillin-clavulanate and the patient’s adverse reaction to this treatment led to the proper diagnosis.Subsequent otolaryngology assessment 18 days thereafter revealed both his left parotid region swelling and his rash to have completely resolved. On physical examination there was a barely detectable subcutaneous nodule overlying the left parotid gland and no palpable cervical lymphadenopathy.Although uncommon, Epstein-Barr virus can be a cause of acute parotitis. The multiglandular involvement and cervical lymphadenopathy noted particularly on ultrasonography should have raised concern for this etiology.Had it not been for his adverse reaction to the amoxicillin-clavulanate prompting evaluation for Epstein-Barr virus infection as one of the potential triggers, this diagnosis would likely have been missed.