Pediatrics

A 9-month-old previously healthy male presented with a fever and a rash. The history of his illness began 2 days earlier with the onset of fever, irritability and decreased oral intake without vomiting; however, he had some mild diarrhea. He had been receiving topical benzocaine (Orajel, Church & Dwight Co. Inc.) and acetaminophen for presumed teething pain without relief. His family history and medical history were unremarkable, and immunizations were up to date for his age.

Examination at that time revealed general fussiness and a temperature of 104.3·F with a maculopapular rash that started on his face (Figure 1) that quickly progressed to the rest of his body (Figure 2), including his feet, which may have had some mild swelling (Figure 3). There were no blistering or target lesions seen. The rest of his exam was unremarkable; specifically, no cough, congestion, mucous membrane inflammation or adenopathy. He was diagnosed with a viral infection with an associated exanthem and treated symptomatically.

Lana Soylu

James H. Brien

He followed up the next day and was noted to have a temperature of 99.9·F with worsening irritability. The rash was now a faint, generalized maculopapular rash. However, he had developed erythema of his lips (Figure 4) and oral mucosa. Additionally, his eyes were erythematous but without any exudate seen (Figure 5). He was then admitted for further evaluation.              

Figure 1. Examination revealed general fussiness and a temperature of 104.3°F with a maculopapular rash that started on his face. Images: Brien JH

Figure 2. The rash quickly progressed to the rest of his body.

Figure 3.including his feet, which may have had some mild swelling.

His admission lab tests included a CBC with mild anemia, but otherwise unremarkable; a C-reactive protein of 13.6 mg/L; an erythrocyte sedimentation rate of 89 mm/hour; and a urinalysis with 75 WBCs per high-power field. A polymerase chain reaction for enterovirus and herpes simplex from the eye were negative. The rest was unremarkable. Because of the persistent irritability and fever to higher than 101·F, a lumbar tap was performed the next day revealing normal cerebrospinal fluid. On hospital day 4, all cultures (blood, urine and CSF) were negative, but the fever continued.

Figure 4. He had developed erythema of his lips and oral mucosa.

Figure 5. His eyes were erythematous but without any exudate seen.

What’s Your Diagnosis?

A. Measles (rubeola)

B. Kawasaki disease

C. Roseola

D. Stevens-Johnson syndrome

This is a case of Kawasaki disease (B). On day 5 of fever (hospital day 3), he had an echocardiogram that revealed ectasia of the left main coronary artery. He then received 2 g/kg of IV immune globulin and began treatment with high-dose aspirin. His fever and irritability quickly resolved, and his follow-up echocardiogram 8 weeks later showed resolution of the ectasia. Of the five diagnostic criteria for Kawasaki disease (KD), the baby was noted to have: 1) a compatible rash; 2) inflamed oral mucous membrane; 3) eye inflammation without discharge; 4) possible extremity changes; and only lacking 5) adenopathy. He also had supporting laboratory findings of elevated C-reactive protein and erythrocyte sedimentation rate and sterile pyuria. This is certainly enough to make the diagnosis of KD. However, with coronary artery ectasia, the diagnosis can be confirmed with only two of the listed criteria.

Infants with KD are more likely to develop coronary aneurysms than older children, and more likely to have fewer diagnostic criteria (incomplete KD). In fact, a febrile infant who is 6 months of age or younger can be diagnosed with coronary ectasia alone with none of the five diagnostic criteria.

The baby in Figure 6 is 7 weeks old with a persistent fever and rash. He was found to have a giant aneurysm on echocardiogram when his platelet count sharply rose to more than 1 million during the second week of his illness. He had no other criteria of KD. The AAP Red Book has an excellent section on KD, along with an algorithm for diagnosing and managing incomplete KD. If a child does not respond to one dose of IVIG, it would be advisable to consult with a KD expert for further management, but guidance for this can certainly be found in the Red Book.

Figure 6. The baby is 7 weeks old with a persistent fever.

Measles is a febrile disease with an exanthem that begins on the face (Figure 7) and spreads down. It is typically accompanied by an exudative conjunctivitis, coryza and cough. The enanthem of measles are Koplik’s spots, which are small pearly white lesions, usually found on the buccal mucosa and often not seen due to their fleeting nature. The rash may be similar to that of KD, but the other findings make it fairly easy to distinguish the difference.

Figure 7. Measles is a febrile disease with an exanthem that begins on the face.

Roseola, or sixth disease, due to human herpesvirus 6, causes a high fever with some irritability, but the rash appears at the end of the febrile phase, making it easy not to confuse these conditions. Also, there is no enanthem or other mucous membrane inflammation with roseola.

Lastly, Stevens-Johnson syndrome (SJS) is an acute, inflammatory condition resulting in necrosis of localized areas of the skin and mucous membranes, usually triggered by medications, but also infectious agents such as herpes simplex. Figure 8 reveals some of the typical lesions seen in the acute stage; inflamed skin with various sized blisters and vesicles and necrotic mucous membrane lesions with cracking and bleeding of the lips, and the same patient in the healing stage several days later (Figure 9).

Figure 8 reveals some of the typical lesions seen in the acute stage including necrotic mucous membrane lesions with cracking and bleeding of the lips.

Figure 9. The same patient in the healing stage several days later.

Severe cases involving large areas of the skin are usually referred to as severe SJS or toxic epidermal necrolysis (TEN); however, I still find the nomenclature of these conditions in some modern references confusing, with too many different names with overlapping features; TEN, SJS, severe SJS, erythema multiforme (EM) major and EM minor. These conditions may very well represent a spectrum of the same condition, but there are enough differences that it would seem reasonable to add some clarity by uniformly defining and re-naming these conditions so that a simple-minded person (like me) can understand it.

Columnist comments

I normally don’t promote meetings in this column unless I have personal knowledge of the quality and content. This is the case for the Primary Care Pediatric Conference on July 15 – 18 at the Hyatt Regency Lost Pines resort, just East of Austin, Texas. It is an excellent meeting at a fun, family-friendly venue. For more information go to BaylorCME.org/CME/1455. Hope to see you there.

• Lana Soylu, MD, is an assistant professor in General Pediatrics at the University of South Florida. Dr. Soylu received her bachelor’s degree in biology from the University of Colorado at Boulder and her medical degree from the University of Colorado in Denver. She completed her pediatric residency at the University of South Florida in Tampa. James H. Brien, DO, is a member of the Infectious Diseases in Children Editorial Board, as well as Vice Chair for Education at The Children’s Hospital at Scott and White, and is the Associate Professor of Pediatrics at Texas A&M University, College of Medicine, Temple, Texas. email: jhbrien@aol.com.

• Disclosure: Drs. Brien and Soylu report no relevant financial disclosures..