This article is more than 5 years old. Information may no longer be current.
A 5-year-old female admitted with respiratory distress, bilateral pneumonia and rash
Click Here to Manage Email Alerts
A 5-year-old Hispanic female presented to the urgent care clinic with respiratory distress, fever and a rash. The onset of her symptoms began 4 days earlier with the onset of a pruritic rash. Fever was not noticed until 2 days later, followed the next day by increased coughing and shortness of breath. She was subsequently admitted to the hospital.
An Pham
James H. Brien
Her medical history is positive for having been diagnosed with precursor B-cell acute lymphoblastic leukemia in May 2009. She was seen in the oncology clinic 6 days before admission for a scheduled visit during the maintenance phase of her chemotherapy treatment. Her physical examination at that time was normal with the exception of mild angular cheilitis. Laboratory tests obtained during that visit were remarkable for mild leukopenia and neutropenia with a WBC count of 1,800 cells/mcL and an absolute neutrophil count (ANC) of 972 cells/mcL, respectively; liver enzymes and kidney function were within normal limits.
Figure 1. The patient was noted to have several scattered lesions consisting of macular, papular and vesicular lesions similar to those shown in the figure above.
Source: Cater M
She then received her scheduled vincristine and intrathecal methotrexate and was started on a 5-day pulse of oral dexamethasone, besides continuation of daily oral 6-mercaptopurine, as per protocol. Her only other medication was Pneumocystis prophylaxis with trimethoprim-sulfamethoxazole. She had been doing well until the onset of her chief complaint. The rest of her history and family history was unremarkable. She had no known sick contacts.
It is notable that her immunization status was incomplete, with no immunizations since her 6-month well baby series.
Figure 2. Her chest radiograph is shown in the figure above.
Source: Brien JH
On examination in the urgent care clinic, she was found to be febrile at 101.7·F and had numerous lesions in different stages of development on her scalp, face, trunk and extremities, gingiva and oropharynx. In the hospital, her vital signs revealed a temperature of 104·F, blood pressure of 106/63 mm Hg, heart rate of 138, and a respiratory rate of 36. She was noted to have several scattered lesions consisting of macular, papular and vesicular lesions similar to those shown in Figure 1. Some lesions were crusted, but none appeared to be secondarily infected. Her lung exam revealed bilateral rales and rhonchi with some retractions and mild hypoxia. The rest of her exam was unremarkable.
Laboratory results upon admission compared with those obtained in clinic a few days earlier were significant for a lower ANC of 740 cells/mcL and thrombocytopenia with a platelet count of 42,000 cells/mcL, compared with 212,000 cells/mcL previously. Liver enzymes were mildly elevated with AST/ALT levels of 108 IU/L and 199 IU/L, respectively. Her chest radiograph is shown in Figure 2.
What’s your diagnosis?
A.Staphylococcus aureus pneumonia with pyoderma
B.Varicella pneumonia
C.Pneumocystis jirovecii pneumonia
D.Mycoplasma pneumoniae pneumonia
Case Discussion
The radiological findings of diffuse bilateral reticulonodular infiltrates demonstrated in Figure 2, along with the characteristic vesicular rash occurring in crops shown in Figure 1 (courtesy of Michael Cater, MD), are consistent with varicella pneumonia (B).
Primary varicella infection is usually a mild, self-limited disease in immunocompetent children but can be severe in adolescents, adults (especially pregnant women) and immunosuppressed individuals at any age. Patients with an underlying malignancy, chronic or even episodic steroid use, HIV infection or history of solid organ transplantation are more susceptible for disseminated varicella disease due to impaired cellular immunity and experience more frequent severe morbidity and higher mortality rates compared with normal hosts.
Complications of varicella include bacterial superinfection of skin lesions, which can be severe with necrotizing fasciitis as shown in Figure 3. Other complications include central nervous system involvement with encephalitis or acute cerebellar ataxia, pneumonia and hepatitis. Treatment in immunocompromised patients must be initiated early in the course of the illness, ideally within 24 hours of rash onset, to maximize efficacy. Although oral acyclovir should be considered for patients who are at increased risk for moderate to severe varicella disease, it is inadequate for immunocompromised patients. For those who are immunosuppressed, it is recommended that they receive acyclovir given intravenously at 10 mg/kg every 8 hours for the duration of 7 days, or until all lesions are crusting with no new lesions seen.
Figure 3. Complications of varicella include bacterial superinfection of skin lesions, which can be severe with necrotizing fasciitis.
Since implementation of the single-dose childhood varicella vaccination program in 1995, there has been a dramatic decline in the incidence of the disease, as well as the morbidity and mortality rates from complications of the disease, including those with suppressed immune systems. However, even with a two-dose recommendation, cases such as this still occur, which stresses the importance of ensuring that all children be frequently screened for having all of the recommended routine childhood immunizations.
For patients who are immunocompromised, including those receiving immunosuppressive medications and those with hematologic malignancies, varicella vaccine is generally contraindicated because it is a live-virus preparation. However, close contacts can be screened for a history of disease or immunization to better protect the susceptible family member from exposure. If an immunocompromised patient without previous varicella infection and unknown or negative serology becomes exposed to varicella, as was the case for the patient described in this column, passive immunoprophylaxis with administration of varicella-zoster immune globulin (VariZIG) or immune globulin intravenous (IGIV) is recommended as soon as possible. However, it can be given even as long as 10 days after exposure.
This, again, emphasizes the importance of knowing the immunization status of all patients, especially those with high-risk conditions, and providing appropriate anticipatory guidance regarding actions to take once exposure to disease has occurred. Of course, knowing one has been exposed is not always apparent, as in the case presented. Although data are lacking, most experts will also use post-exposure chemoprophylaxis for these compromised patients with acyclovir (see 2012 AAP Red Book).
Pneumocystis jirovecii pneumonia (C), previously known as P. carinii, commonly presents with fever and a nonproductive cough associated with dyspnea, tachypnea and hypoxia. The chest radiograph often shows diffuse bilateral infiltrates. The treatment of choice is TMP-SMX given intravenously for 21 days.
Patients who are at risk, including those with HIV with a CD4+ cell count less than 200/mcL, those with primary immune deficiencies, those who are receiving long-term immunosuppressive regiments, and those with hematologic and non-hematologic malignancies, are routinely placed on P. jirovecii pneumonia prophylaxis with oral TMP-SMX, usually 3 days a week. Of course, the varicella rash is the difference in this case, making this choice unlikely.
Figure 4 and 5. Staphylococcus aureus pneumonia with pyoderma would likely produce a more localized infection (Figure 4) with possible abscess formation (Figure 5, removed abscessed segment shown in Figure 4).
Mycoplasma pneumoniae (D) is a common cause of community-acquired pneumonia and usually has a gradual and insidious onset over several days to a week. Patients will most often have nonspecific complaints, including fever, malaise and headache, besides having a persistent, non-productive cough. Chest radiograph findings vary, but usually show bilateral, diffuse infiltrates, with focal findings such as consolidation or pleural effusions less commonly seen. The preferred antimicrobial agents for treatment of pneumonia caused by M. pneumoniae are macrolides because mycoplasma organisms lack a cell wall and are inherently resistant to agents that act on cell wall synthesis, such as beta-lactams.
Lastly, Staphylococcus aureus pneumonia with pyoderma would likely produce a more localized infection (Figure 4) with possible abscess formation (Figure 5, removed abscessed segment shown in Figure 4). Staphylococcal pyoderma would bear no resemblance to common varicella rash.
Figure 6. The patient presented was treated with IV acyclovir with a good outcome.
The patient presented was treated with IV acyclovir with a good outcome, as shown in Figure 6.
Columnist Comments
I would like to thank Dr. Pham for researching and writing this case and Mr. Lewis Tsai, a pre-med student at The University of Texas in Austin, for his help with the literature review and critical analysis of the column.
Lewis Tsai
Last month I mentioned Dr. Itzhak Brook and his inspirational message of overcoming adversity and teaching empathy. On this 11th anniversary of Sept. 11, 2001, I might suggest that you take a look at a website by Itzhak’s son, Yoni, at www.ybpix.com/Yoni_Brook/Yoni_Brook.html. He is an award-winning photographer who was there that day and recorded many outstanding images.