May 2004

A 20-month-old girl is transferred from a community hospital for further evaluation of fever and a rash. The onset was a few days earlier, but she became more febrile and was admitted to her local hospital for evaluation and IV fluids. She was transferred the next day, as her condition seemed to be worsening.

Her past medical history is normal with no recent sick contacts, and her immunizations are up to date. Her family history is also normal. There has been no recent travel or animal exposure. She has not been taking any medications prior to this illness and has no allergies.

Her examination revealed an irritable, febrile child with a diffusely erythematous, painful rash with a maculopapular, somewhat scarlatiniform component. There was also much purulent drainage from her eyes and nose. Additionally, there was some fine, widespread desquamation noted, and some areas of denuded skin at sites where tape had been recently removed (Figures 1-4). Her temperature was 101° F. The rest of her vital signs and examination were normal.

What is the most likely cause?

1. Fourth disease
2. Kawasaki syndrome
3. Measles
4. Streptococcal scarlet fever

Answer

This is an example of one of the staphylococcal toxin syndromes called staph scarlet fever, which historically is most consistent with fourth disease (A), often referred to as Dukes’ disease or Filatov-Dukes disease. Nils Filatov (occasionally spelled Filatow) was a Russian pediatrician (1847-1902) and described the condition in 1885. Clement Dukes (1845-1925), an English physician, described the condition in 1894 and suggested calling it fourth disease in 1900.

Numbered diseases

When it comes to the historically numbered exanthems, the topic of fourth disease becomes one of some controversy. It is well known that first and second diseases (measles and scarlet fever, respectively) were first described in the 17th century. Rubella was added in 1881 by the International Congress of Medicine. Fifth and sixth diseases were described in 1905 and 1910, respectively. But fourth disease remained questionable, as many thought it to be a scarlet fever–like form of rubella. However, in the late 1970s, the fog began to clear, as the spectrum of staphylococcal toxin-related diseases became better understood. This was largely through the efforts of Marian E. Melish and her landmark work with this organism (J Pediatr. 1971;78:958-967). Since then, others have also published excellent reviews of the staphylococcal diseases, such as Andy Margileth (South Med J. 1975;68:447-454) and Jim Bass (Postgrad Med. 1982;5:58-74), and on fourth disease specifically (Keith Powell, Am J Dis Child. 1979;133:88-91). However, the most recent and thoroughly convincing paper on this topic is from Marty Weisse, professor of pediatrics at West Virginia University Health Science Center in Morgantown (Lancet. 2001;357:299-301). He pulls all the previous studies together, pointing out that some inconsistencies remain, but the evidence supports the cause of fourth disease to be a toxin-producing Staphylococcus aureus, most consistent with what we have come to know as staph scarlet fever.

Other choices

Regarding the other choices, measles results in a fever and a rash, but the rash is clearly different. It is more of a painless, maculopapular rash with early confluence, especially on the face and trunk (Figures 7 and 8). Also, it does not desquamate. The fever is usually high and has an associated cough with coryza and conjunctivitis. You may see Koplik spots, the pathognomonic enanthem of measles, if you see the patient early enough in the illness (Figure 9).

Kawasaki disease (syndrome) is also a febrile exanthem illness, but the conjunctivitis is virtually always “dry,” and the desquamation occurs later, typically in the second week. The other diagnostic criteria were missing (other mucous membrane involvement, extremity findings, enlarged lymph node).

Getting back to our patient, a culture of the nose grew a large amount of methicillin-sensitive S. aureus. There are enough hints in the brief description to rule out the other choices. It differs from streptococcal scarlet fever (SF) mainly by the painful rash and early desquamation (usually occurs in the second week in streptococcal SF). Additionally, the rash of streptococcal SF is a fine, papular, sandpaper-like rash (scarlatiniform [figure 5]), but without the positive Nikolsky’s sign seen in this patient with the damage to the skin upon removing tape. Also, purulent conjunctivitis is not seen with streptococcal SF. This patient also had no oral findings consistent with streptococcal SF (strawberry tongue, Figure 6). The patient was treated with IV nafcillin followed by oral cephalexin (Keflex, Lilly) and made a rapid recovery.

Columnist comments

I would like to credit Jim Bass for Figures 5, 6 and 9. Dr. Bass was passionate about several topics in pediatric infectious diseases. This was one of them, and many of my best pictures came from his collection, which he readily shared with anyone interested.

Marty Weisse also trained under Bass, and as you can see, we become a sort of clone of our program directors when he or she is truly a great mentor. I think this is an important point to remember when we are on teaching rounds. Students and residents really do model themselves after good people. In time, I expect to see a lot of Marty Weisse clones roaming the earth.

In my last military-deployment of pediatricians update, I mentioned that 69 pediatricians were deployed. That really should be 69 pediatricians have been deployed. Since they are on a rotating schedule, there are variable numbers there from time to time.

I also mentioned that one is a Brigadier General, commanding a Medical Brigade in Baghdad. His name is Jim Reynolds, from a Minnesota reserve unit deployed there. He recently noted to me that the medical soldiers are doing great things in Iraq, and that they are working around the clock, going from crisis to crisis. He also tells of some specific actions of some pediatricians being called on to perform life-saving pediatric-related duties.

General Reynolds also expressed his pride and honor in being able to command such fine soldiers. I think we as pediatricians can also be proud of General Reynolds and all the pediatricians and other physicians serving there, especially during these very difficult and uncertain times leading up to the transition of power.

Lastly, I want to pitch an advertisement for one of the best general pediatric meetings on the calendar. It’s the Uniformed Services Pediatric Seminar. This is an annual meeting, cosponsored by the AAP and the Uniformed Services Section, and has developed into one of the finest CME opportunities one can attend. The topics are relevant to all pediatricians. Plus, you get to learn in-depth some things that are not so typical at other meetings, like the effects of chemical/biological attacks on children and topics related to pediatricians and other physicians deployed to a combat environment. We are unlikely to find ourselves dealing with these issues, but you never know.

Lastly, these people have fun. Whether you have ever been a member of the uniformed services or not, you will enjoy the entertainment (especially the Bass Challenge Bowl) and comradery that goes with attending this meeting. Please write me (jhbrien@aol.com) if you want more details. I am fortunate enough to be a speaker at this meeting next year in San Antonio, toward the end of March. I hope to see you there.

For more information:

• James H. Brien, DO, Pediatric Infectious Disease, Scott and White’s Children’s Health Center and Associate Professor of Pediatrics, Texas A&M University, College of Medicine, Temple, Texas. E-mail: jhbrien@aol.com.