Girl presents with swelling on left side of her face, occasional fever
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A 10-year-old girl is brought to your clinic by her mother, complaining of left-sided facial swelling with mild-to-moderate pain.
The onset of the complaint was about 1 week earlier, with slow progression that included occasional low-grade fever.
She has no other complaints and is otherwise well, with no other past medical problems identified. However, during the review of systems, her mother recalled two other similar episodes of facial swelling over the last 2 years. The girl was seen in an urgent care clinic for those episodes, and each time she was treated with an antibiotic, but the mother could not recall the name. When various choices were listed, she thought it might have been clindamycin. However, they recalled that the girl seemed to get better within a few days of beginning the medication. She has had no known sick contacts recently, and her immunizations are up to date, including two doses of MMR given at the recommended times.
Examination revealed a healthy-appearing 10-year-old female, with a temperature of 100.9°F (38.3°C). The only positive finding was some diffuse, preauricular swelling on the left side of her face that extended around the subauricular area, with a blush of erythema, as shown in Figures 1 and 2. There was some minimal pain on palpation, and the subcutaneous tissue was smooth, with no nodules or lumps felt. With gentle application of pressure over the swelling, there was no material seen exiting the Stensen duct on oral examination.
No imaging or lab tests were obtained.
Summary
- A previously healthy 10-year-old female presents with acute, mildly painful swelling of the right side of her face and low-grade fever.
- She experienced two previous episodes over the last couple of years.
- Her immunizations, including the MMR, are up to date.
What’s your diagnosis?
A. Acute cervical lymphadenitis
B. Juvenile recurrent parotitis
C. Mumps parotitis
D. Sjögren’s syndrome
Answer and discussion
This is juvenile recurrent parotitis (choice B), a fairly uncommon condition that seems to affect children and young adults from 2 to 20 years, with the peak being around puberty to mid-adolescence. It is thought to be the result of an ill-defined autoimmune inflammatory condition vs. dysfunctional salivary ducts vs. recurrent bacterial parotitis due to retrograde movement of mouth organisms (mostly Staphylococcus aureus), which may explain why some cases seem to respond to antimicrobial agents. If the patient or the parents can clearly recall past episodes, a detailed history may be all that is needed to make this diagnosis. However, if further evaluation is needed, CT imaging may help discover a stone within the parotid duct system or, if purulent material can be expressed from the Stensen duct, a Gram’s stain and culture may reveal the diagnosis. I personally have never found blood testing in these cases to be of benefit where recurrence has been established. In such cases, it is reasonable to prescribe an appropriate anti-staph/anti-strep antimicrobial, especially if past episodes seemed to benefit from similar treatment. In cases that recur every 1 to 2 months, a referral to an otolaryngologist would be in order for more in-depth evaluation of the anatomy and function of the parotid gland. However, because of the risk for facial nerve damage, surgical removal should almost never be done for this self-limiting condition.
Acute cervical lymphadenitis may also have similar symptoms, but the location typically is easy to differentiate. As shown in Figure 1, the left side of the patient’s face is swollen, making the ear barely visible on frontal viewing, whereas the ear is clearly seen on the patient with lymphadenitis in Figure 3. Also, the distorting effect of the mass on the ear, seen in Figure 4, would not occur with parotitis.
Mumps parotitis may present in a similar fashion but is usually bilateral. Also, in the face of recurrence of similar episodes and two doses of MMR immunization, as well as a lack of sick contacts, mumps becomes very unlikely but not impossible. There are numerous examples of mumps vaccine failure, often in patients attending college.
Sjögren’s syndrome is an autoimmune disorder of the exocrine glands that most prominently results in the clinical findings of a dry mouth and dry eyes. Such was not the case with this patient. However, just to “muddy things up” a bit more, even though it is very uncommon in children, Sjögren’s syndrome in a child may present as recurrent parotitis. But again, these patients eventually develop the eye and mouth findings as well.
Columnist comments
I presented a similar case of this unusual condition in an 11-year-old patient in the May 2010 issue of IDC . A fairly recent review paper can be found here .
In memoriam
Two giants of infectious diseases were lost earlier this year.
I first heard of Jerome Klein, MD, when I started my pediatric infectious diseases fellowship in 1982. Dr. Klein was the editor of the 1982 Red Book, which all fellows completely consume during the years of fellowship (and beyond). Additionally, he was on the Red Book Committee from 1973 to 1980. Jerry died at the age of 90 years on Feb. 16, 2021. A beautiful tribute written by his friends and colleagues at Boston University School of Medicine can be found at the Pediatric Infectious Diseases Society (PIDS) website.
In addition to the Red Book Committee, Dr. Klein served in many other leadership roles, including president of PIDS. As noted below, he was also co-editor of Remington and Klein’s Infectious Diseases of the Fetus and Newborn Infant, now in its 8th edition. As a young member of the pediatric ID staff in the Army Medical Corps, I referred to books and other publications with Dr. Klein’s name on them on a weekly basis. When I was fortunate enough to begin speaking at national CME meetings, Dr. Klein was often on the same program, which made me feel very small, but he always made me feel like I belonged there. To a young physician with a chronic inferiority complex, this was a tremendous confidence builder. Then, when Jerry and Stephen Pelton, MD, invited me to present at the annual “Pediatric Infectious Disease in the News Update” in 2009, I felt especially honored. It seemed like a short journey to have gone from a new fellow reading Jerry’s Red Book to presenting in Boston with him on the front row 27 years later.
Less than 2 months later, on April 8, 2021, Jack Remington, MD, professor emeritus of infectious diseases at Stanford, died — also at 90 years of age. Dr. Remington earned his MD from the University of Illinois in 1956. During his military service, he became a research associate at the NIH’s National Institute of Allergy and Infectious Diseases. His interest in toxoplasmosis grew out of that experience and followed him throughout his career, and he became the world’s leading expert on the subject. As noted earlier, Drs. Remington and Klein joined forces to publish the definitive textbook on infectious diseases of the fetus and newborn infant.
I met Dr. Remington on several occasions over the years at the winter infectious diseases course that now bears his name. I always found him to be a very pleasant and agreeable gentleman. A detailed memorial about his professional and personal life can be found here. I would encourage anyone in the field of infectious diseases to read the tributes at the links above. We owe it to ourselves and, most of all, to our students to know our history and upon whose shoulders we stand.
References:
American Academy of Pediatrics. Committee on Infectious Diseases. Red Book. Report of the Committee on Infectious Diseases. Academy of Pediatrics; 2021. Accessed June 15, 2021. https://redbook.solutions.aap.org/redbook.aspx.
Wilson CB, Nizet V, Maldonado YA, Remington JS, Klein JO, eds. Remington and Klein’s Infectious Diseases of the Fetus and Newborn Infant. 8th ed. Elsevier/Sanders, 2015.
For more information
Brien is a member of the Infectious Diseases in Children and Infectious Disease News Editorial Boards, and an adjunct professor of pediatric infectious diseases at McLane Children's Hospital, Baylor Scott & White Health, in Temple, Texas. He can be reached at jhbrien@aol.com.