This article is more than 5 years old. Information may no longer be current.
A 7-year-old boy with strep pharyngitis
Click Here to Manage Email Alerts
Because of concern for acute rheumatic fever, a 7-year-old boy was sent for admission to the hospital with a recent history of streptococcal pharyngitis, followed by arthritis and rash.
The history of this illness began about 10 days earlier with a sore throat and upper respiratory congestion. His rapid strep test was positive, so he was treated with amoxicillin. However, his fever persisted with the development of a rash on his lower extremities the next day, followed by pain and swelling of his ankles, knees and wrists the day after. The antibiotic was empirically changed to a first-generation cephalosporin without benefit.
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
His past medical history was normal. His vaccinations were up to date, and there had been no travel, camping, tick bites or other insect bites. He does live in a wooded area with deer, but his only direct animal exposure is to the family dog.
Examination revealed normal vital signs and a rash consisting of petechiae and nonblanching red bumps on his lower extremities and buttocks, as shown in Figures 1 through 4. He also had painful swelling of the joints of his right hand, knees and elbows, with decreased range of motion testing but no erythema over the joints (Figures 5 and 6). The rest of his examination, including his throat, was normal.
Lab tests included a complete blood count that was normal, including the platelet count, but he had mild elevation of the C-reactive protein.
What is the most likely cause of this diagnosis?
- Henoch-Schönlein purpura
- Juvenile idiopathic arthritis
- Rheumatic fever
- Streptococcal scarlet fever
Answer
The answer is A. Henoch-Schönlein purpura.
Although first recognized as a distinct syndrome by the British physician William Heberden (1710-1801) in the 18th century, it was the German physician Johann Schönlein (1793-1864) who first described the characteristic rash. Later, the German pediatrician Edouard Heinrich Henoch (1820-1910) described the gastrointestinal and renal manifestations of the disease during the 19th century. Henoch-Schönlein purpura is an immunoglobulin A-mediated autoimmune small-vessel vasculitis that is most common in children aged between 2 and 8 years, with boys being affected about twice as often as girls. Some infectious triggers have been identified, such as group A streptococcus (as in the patient above), mycoplasma and viruses of the respiratory tract. However, many cases have no identifiable trigger.
All Henoch-Schönlein purpura patients have the rash, which can be a mix of petechiae, purpuric and maculopapular lesions. The distribution of the rash tends to be in gravity-dependent areas, such as the legs and buttocks. Over time, the color of the lesions becomes darker, changing to a rusty, brownish color, as shown in Figure 7. Arthritis is found in about 75% of patients and gastrointestinal pain in about 50%. Renal involvement is seen in about 30% to 40% but can be a serious complication with the possibility of progression to renal failure. A rare but potentially life-threatening complication is involvement of the central nervous system.
Diagnosis is made by recognition of the rash with one or more of the associated findings, which may not appear at the same time. There is no specific treatment, but antiinflammatory agents may help, or possibly steroids for those with severe CNS involvement.
The most important diagnostic decision is to differentiate Henoch-Schönlein purpura from meningococcemia (Figures 8 and 9), which is why most patients are initially admitted to the hospital. There can be enough overlap that some patients are treated empirically with antibiotics until the diagnosis is clear, and that may take a day under observation. However, most patients with meningococcemia are clinically much sicker than these Henoch-Schönlein purpura patients, and it does not take long to sort them out.
We know the patient had streptococcal pharyngitis; however, there was no other evidence of scarlet fever. The usual scarlatiniform rash is a fine, papular rash on a diffuse erythematous base, as shown in Figure 10. which depicts a patient with multiple infected insect bites with group A strep and a scarletiniform rash. The erythema is accentuated by the “thumb print” on his back, like a capillary refill test. This is easy to distinguish from a petechial or purpuric rash. Other associated findings of scarlet fever include circumoral pallor, Pastia’s lines and a “strawberry” tongue, which this patient did not have.
The other streptococcal-related disease listed among the choices was rheumatic fever, which is the real reason the primary provider wanted the patient admitted. However, when the Jones criteria are considered, the joint complaints and elevated C-reactive protein were the only minor criteria; there were no major criteria. This, coupled with the rarity of rheumatic fever, makes this diagnosis very unlikely. However, an electrocardiogram was done and was normal. The rash of rheumatic fever is referred to as erythema marginatum and is virtually pathognomonic of rheumatic fever. I do not have any pictures of erythema marginatum, but you can probably find them on the internet.
Lastly, although juvenile idiopathic arthritis can have unusual rashes, it cannot be diagnosed this early in the course of a febrile illness. Normally, the diagnosis is not made until there have been symptoms present for four to six weeks and no other explanation for the illness.
Gerald Merenstein, MD
I would like to dedicate this column to the memory of Gerald (Jerry) Merenstein, MD (Feb. 14, 1941-Dec. 14, 2007). Periodically, I feel a sad responsibility to inform you of the death of someone who we know, or perhaps should have known. Unless you are a neonatologist, you may not know who Jerry was, unless you happen to be a pediatrician who trained in Denver during the last 40 years.
If not for Jerry, I probably would have ended up an orthopedic surgeon (my ambition as a second-year student at the Texas College of Osteopathic Medicine in Fort Worth) and would be writing this column in Sawbones Monthly instead of Infectious Diseases in Children. As a student participant in the U.S. Army Health Professions Scholarship Program, I went to Fitzsimmons Army Medical Center in Denver to spend my summer active duty. I had arranged to do a rotation with the orthopedic department there. I drove all day one Saturday in early June 1975 to get there. It was still light after checking into the visiting officer quarters, so I took a walk to the hospital to get the big picture. I wandered into the emergency room and after introducing myself to the charge nurse, I met the intern on duty, who happened to be a first-year orthopedic resident: Tom Hawks, MD. I was excited that things were coming together.
Then I bumped into Jerry Merenstein, who was there on this Saturday night seeing children. So, what is unusual about that? At that time, Jerry happened to be a lieutenant colonel, acting chief of the department of pediatrics, the residency program director, chief of the Newborn Services and director of the Neonatology Fellowship Program. Wearing all those hats, Jerry still took his turn (on a Saturday night) at duty in the ER seeing children. He invited me to “hang out” with him as he saw patients. He got off duty at about 11 p.m., and we both left for the night. To shorten the story, I ended up reporting for duty to the pediatric department the following Monday instead of orthopedics.
Jerry did not try to talk me into anything but rather showed me by his actions what I thought a physician should be, and I simply wanted to pursue his style a bit further. The rest is history. After finishing a very difficult and demanding pediatric residency at the same place, I left with confidence that I could deal with just about any neonatal problem a general pediatrician would be expected to handle and that I knew how to transport the rest. To say the least, Jerry Merenstein had a profound and lasting effect on my professional and personal life. In fact, when I received notice that I had passed the pediatric boards in 1981 and was a general pediatrician at Fort Hood, Texas, the first person I called was Jerry. He said, “Of course you passed. I knew you would,” in a proud, fatherly voice. That acknowledgement from Jerry meant a great deal to me, as I felt I had not let him down. Few people, including parents, will have that kind of effect on a person.
Jerry’s achievements cover a long and distinguished academic as well as clinical career. After 20 years in the Army Medical Corps, Jerry retired from active duty as chief of the department of pediatrics at Fitzsimmons Army Medical Center and moved on to The University of Colorado, where he had a second career of distinguished excellence in teaching and patient care. He published many papers on neonatology as well as several editions of his “Handbook of Neonatal Intensive Care.” In fact, I was first introduced to clinical research as an intern when Jerry recruited some of us to enter patients into one of his neonatal group B streptococcus prevention studies, some of which helped form the basis of the protocol we follow today.
After a long illness, Jerry died on Dec. 14, 2007 at his home in Denver with Bonnie, his wife of more than 47 years, at his side. Realizing that I will never achieve it, I am nonetheless still pursuing his style and high standard, which I hope will honor his memory. Shalom, my friend. Shalom.
What’s Your Diagnosis? is a monthly case study featured in Infectious Diseases in Children, with treatment information and discussion to follow.