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November 29, 2022
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Toddler presents with fever, malaise

What’s your diagnosis?

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James Brien

A 3-year-old boy presented to his family provider’s office for evaluation of acute onset of fever, with a temperature of 102°F (38.9°C), which began 2 days ago.

He also complained of malaise, along with and decreased appetite and activity. He had no other complaints at that time.

The mother denied any sick contacts, unfamiliar animal contact or insect bites, and no recent travel. His immunizations were up to date for his age, and his past medical history was that of a healthy toddler. The family history was also unremarkable. The patient has a healthy 8-year-old sister, and the family has a healthy dog.

The family provider diagnosed viral upper respiratory infection (URI) and prescribed antipyretics. However, the child returned 2 days later (day 4 of fever) with no significant improvement, and now with a generalized rash and red, dry eyes. A repeat history at that time revealed that the parents forgot to mention before that the 8-year-old sister was treated for strep throat with 10 days of amoxicillin, which was completed 2 weeks ago.

Examination revealed a fever, with a temperature of 102.4°F (39.1°C), with mild tachycardia and a normal respiratory rate. His general appearance was that of a 3-year-old who is somewhat tired and irritable, with some clear rhinorrhea. His eyes were diffusely pink with no discharge seen (Figure 1), and his lips and tongue were slightly erythematous. His skin had a polymorphous rash, with mixed maculopapular and morbilliform components and some areas of confluence (Figure 2). Toward the end of the exam, the mother pointed out that she thought that his hands and feet appeared a bit swollen.

IDC1122WYD_Figure1_1200X630
Figure 1. Conjunctival erythema without discharge. Source: James H. Brien, DO.

He is sent for admission. Admitting lab results show the following:

  1. white blood cell count = 16,000 with 350,000 platelets;
  2. hemoglobin and hematocrit = 9 g/L and 29%;
  3. C-reactive protein = 48 mg/dL;
  4. albumin = 120 U/L; and
  5. urinalysis = 15 WBCs/ hpf

IDC1122WYD_Figure2_1200x630
Figure 2. Generalized polymorphous rash. Source: James H. Brien, DO.

What’s your diagnosis?

A. Kawasaki disease

B. Multisystem inflammatory syndrome in children

C. Rubeola

D. Streptococcal scarlet fever

Answer and discussion:

With 1 more day of fever, this child would fit the diagnostic criteria for Kawasaki disease, or KD (choice A), which consists of 5 days of fever, bulbar conjunctivitis with limbic sparing, a polymorphous rash, extremity changes (swelling and/or erythema), nonsuppurative cervical lymphadenitis and mucous membrane inflammation of the mouth and lips. Some will also count inflammation of the urethra or vagina or the perianal mucocutaneous junction. Many experts suggest making the diagnosis before the 5th day of fever to avoid a delay in therapy if the criteria are there without another explanation. Additionally, even if there is evidence of a viral URI, as in this patient, that should not exclude the diagnosis KD because this can occur simultaneously. However, if the conjunctivitis is purulent, and/or the erythema of the throat is suppurative, the diagnosis of KD should not be made using those criteria because they would not be consistent with KD but more likely indicate adenovirus. It would be extremely unlikely for KD and adenovirus infection to occur in the same patient at the same time. To avoid missing a case of KD, criteria have been established for those who lack enough criteria. The AAP’s Red Book has a detailed chart to help in the evaluation of suspected incomplete KD (page 459), which can also be found here.

Treatment of KD is with IV immune globulin (IVIg) of 2 g/kg. More than one dose may be needed. High-dose aspirin (80 to 100 mg/kg per day in four divided doses) is used until afebrile for 48 to 72 hours, followed by low-dose aspirin (3 to 5 mg/kg per day). This is usually stopped by the cardiologist in follow-up. Repeat dosing of IVIg or other anti-inflammatory agents, such as infliximab or cyclosporin, should be on the advice of a KD expert. As the patient moves through the acute stage and into the subacute-convalescent stage, they will typically have significant thrombocytosis and desquamation of the terminal digits and areas where the rash was most intense.

The similarities between KD and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 are discussed in detail by Lee and colleagues in Frontiers in Pediatrics.

Rubeola — also known as measles or first disease — presents as an acute febrile illness caused by the measles virus, characterized by progressive fever, with cough, coryza and conjunctivitis (the “three Cs”), along with Koplik spots within the first few days, and a morbilliform rash appearing on day 3 or 4. The rash typically is first seen on the head and face, then rapidly spreads down to the upper extremities and trunk (Figure 3), with the confluence of lesions creating blotches of raised erythema. Diagnosis is usually confirmed by serologic testing or by viral detection by PCR of oral or respiratory secretions or blood. Physicians must beware of false positives of both techniques if the patient has received an MMR immunization recently. Treatment is supportive and includes vitamin A supplementation (see the Red Book for more details).

IDC1122WYD_Figure3_1200x630
Figure 3. Morbilliform rash of measles. Source: James H. Brien, DO.

Scarlet fever is a classic group A streptococcal (GAS) infection with a diffuse, erythematous rash with a fine, papular rash, resembling sandpaper, along with a “strawberry tongue,” without eye inflammation. The focus of the GAS infection can be anywhere but usually in the posterior pharynx and tonsils.

References:

American Academy of Pediatrics. Committee on Infectious Diseases. Red Book. Report of the Committee on Infectious Diseases. Academy of Pediatrics; 2021. https://redbook.solutions.aap.org/redbook.aspx. Accessed Nov. 21, 2022.

Lee MS, et al. Front Pediatr. 2021;doi:10.3389/fped.2021.640118.

For more information:

Brien is a member of the Healio Pediatrics Peer Perspective Board 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.