Four patients with varicella develop complications
What’s your diagnosis?
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Patient No. 1, an unvaccinated 12-year-old girl, acquired chickenpox, also known as varicella, 6 days ago and was recovering well, but she awoke during the night with high fever and significant pain in her upper left abdominal area.
In the ED, her vital signs reveal a temperature of 103.5°F (39.7°C). She is alert and cooperative, with an obvious area of dark erythema on her abdomen, mixed with some patches of painless black skin (Figure 1). The pain extended well beyond the black and erythematous areas. The darkest areas were numb.
Patient No. 2, a 2-year-old girl, contracted varicella before the vaccine was available in the 1980s. She was doing well but had a sudden onset of high fever and an erythematous and extremely painful, rapidly spreading rash in her lower left abdomen and inguinal and perineal areas (Figure 2).
Patient No. 3, a 6-year-old male, is brought to the ED for evaluation of a rapidly worsening sore on his left medial thigh (Figure 3). He was diagnosed with varicella 5 days earlier. A review of his immunization record reveals that he had been given most but not all recommended immunizations, including varicella vaccine.
Patient No. 4, a toddler with varicella, presents on day 6 of the rash, with sudden onset of high fever and lethargy, and a rapidly worsening area of painful erythema on the left side of the abdomen and thorax, with some skin breakdown (Figure 4).
Old, healing varicella lesions are seen in all four patients.
What’s your diagnosis (the most likely cause)?
A. Escherichia coli
B. Pseudomonas aeruginosa
C. Staphylococcus aureus
D. Streptococcus pyogenes
Answer and discussion:
The answer is D, Streptococcus pyogenes, or group A strep (GAS) — one of the most common bacteria with which we live, and also one of the most dangerous. Studies have confirmed time and again that necrotizing fasciitis (NF) is an uncommon, potentially fatal complication of varicella, and GAS is recovered in approximately 80% of cases, with Staphylococcus aureus accounting for most of the rest. The organism enters through a pox lesion and gets into deeper layers of tissue fairly quickly, allowing the rapid movement along deep, often fascial planes. One of the clinical features of this infection is the extreme pain associated with it but also relative numbness in the central areas due to dead tissue there. Also, the pain will typically extend beyond the edges of the erythema. This is always a medical and surgical emergency, as deep debridement of dead and infected tissue is critical to interrupt the process. Initial antimicrobial therapy in this scenario should be directed against GAS and S. aureus, pending culture results. Most recommend a combination of a penicillin and clindamycin (for its anti-toxin effect and anti-staph activity). If there is a question of S. aureus sensitivity in your area, vancomycin can be temporarily added, pending culture results. Again, aggressive surgical debridement is critical (Figure 5).
Aggressive debridement may be the difference between life and death but is obviously disfiguring. Patient No. 1 underwent grafting after the infection was cleared (Figure 6 shows the infection just prior to grafting).
The patient in Figure 7 is not the same as the patient shown in Figure 2, but the case had the same varicella-associated NF scenario involving the lower abdomen/inguinal area after debridement of the infection.
Figure 8 shows the post-debridement appearance of the infection shown in patient No. 3.
Patient Nos. 1, 2 and 3 survived their infections. Patient No. 4 did not survive, even after surgical debridement (Figure 9). Also, the cause of this patient’s post-varicella NF and sepsis was due to S. aureus.
There are numerous other organisms associated with deep, necrotizing infections, but they are much less common in the immunocompetent host. Up to 20% of these patients will die of sepsis. Consequently, some experts recommend using IV immunoglobulin as additional, immune-modulating therapy. Lastly, while unproven, many recommend that nonsteroidal anti-inflammatory agents be avoided or used with caution in cases of acute varicella, due to their theoretical suppressive impact on the immune response. To complicate this recommendation, it is also known that varicella infection itself may have an adverse effect on the immune system of some patients.
Columnist comments
I want to thank my old friend, Michael Cater, MD, of Orange County, California, for contributing case No. 4 (and many other cases to this column over the years). Varicella immunization has made varicella uncommon, and many younger physicians have yet to see a case. The vaccine was developed in Japan and in use there by 1988 but was not added to the U.S. immunization schedule until 1995, mostly due to concerns for the possibility of causing shingles. For those who need a refresher on the pertinent findings of varicella, the best review of can be found in the varicella chapter of the 2021-2024 Red Book, or any edition of Sarah Long’s Principles and Practice of Pediatric Infectious Diseases.
References:
American Academy of Pediatrics. Committee on Infectious Diseases. Red Book. Report of the Committee on Infectious Diseases. American Academy of Pediatrics; 2021. https://redbook.solutions.aap.org/redbook.aspx. Accessed June 29, 2023.
Long S, et al, eds. Principles and Practice of Pediatric Infectious Diseases. 6th ed. Elsevier; 2022.
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.