January 2006

An 11-year-old boy is admitted to the hospital with respiratory distress and a rash. The onset of his rash began two days earlier and has been progressive in severity.

On the day of admission, he was noted to be having some respiratory distress, not relieved by using his albuterol metered-dose inhaler that he received about a year earlier when he was diagnosed with asthma.

In the clinic, he received a couple of treatments with nebulized albuterol, and one treatment with ipratropium bromide without improvement. He was given oxygen at 3 L/minute through nasal prongs, which raised his oxygen saturation from 91% on room air to 97%. He also received a 500 mL bolus of normal saline. Along with shortness of breath, his only other complaints were sore throat, dizziness and chest pain.

In addition to asthma, his past medical history is positive for being born premature at 31 weeks and spent about a month in the neonatal ICU, but apparently had no significant sequelae. His immunization history is unknown. His two siblings also have a febrile illness with the same rash, but no other sick contacts. There’s been no recent travel or exposure to animals or insects. He takes no medication on a regular basis.

Examination revealed a fever of 103.5° F, respiratory rate of 40/minute, heart rate of 150/minute and blood pressure of 129/63. He was noted to be coughing and in moderate respiratory distress, with mild retractions, decreased air movement with wheezing and rales. He also had a generalized rash with mixed lesions including macules, papules, pustules, vesicles and scabs, with a generalized distribution, including mouth, ears and eyes, as shown in figures 1-6. The rest of his exam was unremarkable.

His admitting lab tests included a normal complete blood count, electrolytes, and metabolic profile. His chest radiograph is shown in figures 7 and 8. The next morning, his respiratory distress had worsened (figure 9), and he had some mental status changes and was transferred to the ICU for a terbutaline drip.

Of the following, what else would you give?

1. Clindamycin + ceftriaxone + albuterol
2. Vancomycin + acyclovir + steroids
3. Ceftriaxone + vancomycin + acyclovir
4. Vancomycin + ceftriaxone + steroids

Answer

Sorry for the “board-like” question, but there are several teaching points that can be made here. My choice is C, ceftriaxone, vancomycin and acyclovir. This is a complex patient who has (1) varicella, (2) some component of reactive airways disease, as well as clinical and radiological evidence of (3) varicella pneumonia, and it’s rapidly getting worse.

So he should receive IV acyclovir at high doses (60 mg/kg/day ÷ Q 8 hours) for 7 days (provided no new lesions are being seen). Some experts recommend a dose of 1,500 mg/M²/day, which works out to be about the same as 60 mg/kg/day.

There is a good chance that this patient may also be developing a secondary bacterial pneumonia, and until that question is resolved, most of us recommend a combination of a third-generation cephalosporin and vancomycin, just as we would for any patient with unknown severe pneumonia, to cover resistant pneumococcus, Staphylococcus aureus and possible gram-negatives. Some may also use a macrolide to cover atypicals.

Although they are routinely used in status asthmaticus, the use of steroids in a case like this would seem contraindicated because of the active varicella. Also, albuterol is probably not needed as it is being replaced by the terbutaline drip. Lastly, we are using more clindamycin for a variety of problems, like aspiration pneumonia, but it would not be the best antibiotic in this case because of its lack of gram-negative coverage and the problems of possible resistance related to methicillin–resistant Staphylococcus aureus (MRSA) cases. In reality, many patients in the ICU with respiratory failure end up being treated with combinations of broad-spectrum antibiotics, sometimes making the above combination look trivial.

This patient worsened over a couple of days, and then rapidly improved, being discharged about a week after admission (figure 10). His chest radiographs revealed the typical findings consistent with varicella pneumonia of reticular, nodular opacities throughout both lung fields, being more pronounced in the perihilar areas. And while he clinically seemed to have some degree of reactive airways disease, the radiographs showed no hyperinflation of the lungs. His distress was probably all due to the pneumonia.

Varicella pneumonia in normal hosts is most common in adults, but obviously does occasionally occur in children. They all should probably receive an immune screen, especially of their cellular immunity. No immunodeficiency was found in this patient, as is most often the case. The patient in figure 11, courtesy of my old friend, Ed Fajardo, MD, from his Navy days, was a similar patient who was treated in the ICU, and whose immune system was also found to be “normal.”

Nowadays, pediatric and family medicine residents are seeing fewer and fewer cases of varicella. We have had some residents go through their entire three years without seeing more than a couple of cases. Don’t misunderstand, I’m not complaining. This is a good thing. Thankfully, as with many other vaccine-preventable diseases, varicella is now so uncommon, many young physicians of the future may never see a case.

Of course the problem with that is, unlike smallpox, varicella will not likely be eradicated from the world in the foreseeable future. Therefore it will still occasionally occur, and when physicians unfamiliar with varicella see a case, it may go unrecognized for a while until they can get a consult from some old goat like me to help out. I have been on the consultant end of sorting out insect bites from varicella a few times already. What seems obvious to us old-timers is a mystery rash to some of our younger providers. This may be especially problematic for young hematologists when an immunosuppressed cancer patient or one of their close contacts comes in for evaluation with varicella. Not recognizing this in a timely fashion can be a fatal mistake for those patients. The case presented reveals the classic varicella rash, with lesions at various stages of development, from macules to papules to vesicles and pustules to crusted lesions.

Additionally, when the rash is complicated by secondary infection, especially with an epidermolytic toxin-producing S. aureus, the lesions can be distorted beyond the usual appearance, making the diagnosis even more difficult, as shown in figures 12–14 (a child with bullous varicella). It can also be complicated by secondary group A strep infection, often progressing to necrotizing fasciitis, as shown in figure 15 from Jim Bass’ collection. But whether its rash is distorted or associated with cellulitis, it is varicella nonetheless, with all the potential dangers that it poses to the immunosuppressed. So it must never be misdiagnosed.

So, keep using the vaccine, and when you see a case, don’t miss the opportunity to use it to teach others about this classic, old, disappearing disease and take a picture. It may be the last one you see.

Comment

Remember, the Uniformed Services Pediatric Seminar (USPS) is moving from Biloxi, Miss., to Portsmouth, Va., and will be held March 13-16, 2006. It’s a great meeting for general pediatric continuing medical education and an opportunity to associate with some really great people. I hope to see you there.

Also, the 26th Annual (AND LAST) National Pediatric Infectious Diseases Seminar (NPIDS) will be in San Francisco, April 19-22, 2006. Organized by John D. Nelson and George H. McCracken, Jr., of The University of Texas Southwestern Medical Center at Dallas, the NPIDS has been a leading pediatric infectious diseases seminar since it began in Las Vegas 25 years ago. Many loyal attendees (like me) bemoan the fact that it is all coming to an end this year. Don’t miss the opportunity to be a part of this historic event. You won’t regret it and I hope to see you there also.