A 39-month-old boy is brought to the office due to fever. He has had fever spikes the past 5 days that have not improved with oral acetaminophen. He has been very cranky and his appetite has decreased. The patient has had a slightly stuffy nose but no cough, emesis, or diarrhea. He has also developed a bad diaper rash that has not improved with topical barrier ointments. The patient does not attend day care, and there are no sick contacts at home. Medical history is significant for an episode of otitis media last year and occasional upper respiratory infections, but he has had no chronic medical illnesses. Immunizations are up to date, and the patient takes no medications. Temperature is 40 C (104 F), blood pressure is 100/70 mm Hg, pulse is 120/min, and respirations are 18/min. On physical examination, the child is awake and alert but very cranky. The head is normocephalic and atraumatic. The conjunctivae are injected without discharge bilaterally, and the pupils are equal and reactive to light and accommodation. There is scant nasal congestion. The lips, tongue, and oral mucosa are erythematous, but there is no pharyngeal erythema or exudate. Neck is supple without lymphadenopathy. S1 and S2 are normal without murmurs; lungs are clear to auscultation bilaterally. Abdomen is soft, nontender, and nondistended, without organomegaly. There is nontender erythema and peeling of the skin in the perineum and inguinal folds and several erythematous, blanching macules on the trunk; there are no other skin lesions. The patient is sent home but returns to the office a day later due to worsening symptoms. His fever has persisted and he remains extremely cranky. His mother states that his rash has worsened and he now looks "swollen." Temperature is 39.4 C (103 F), blood pressure is 102/74 mm Hg, pulse is 118/min, and respirations are 18/min. On physical examination, he remains awake and alert but irritable. The lips now appear cracked. The neck is supple without lymphadenopathy. S1 and S2 are normal without murmurs, lungs are clear to auscultation bilaterally, and there is no abdominal tenderness or organomegaly. There is a diffuse erythematous, blanching, maculopapular rash on the patient's trunk, abdomen, and extremities, with increased peeling in the perianal and inguinal areas. Edema is present in the hands and feet. Peripheral pulses are present and symmetrical bilaterally; capillary refill is normal. What is the most important next step in the management of this patient?

Question

A 39-month-old boy is brought to the office due to fever.  He has had fever spikes the past 5 days that have not improved with oral acetaminophen.  He has been very cranky and his appetite has decreased.  The patient has had a slightly stuffy nose but no cough, emesis, or diarrhea.  He has also developed a bad diaper rash that has not improved with topical barrier ointments.  The patient does not attend day care, and there are no sick contacts at home.  Medical history is significant for an episode of otitis media last year and occasional upper respiratory infections, but he has had no chronic medical illnesses.  Immunizations are up to date, and the patient takes no medications.  Temperature is 40 C (104 F), blood pressure is 100/70 mm Hg, pulse is 120/min, and respirations are 18/min.  On physical examination, the child is awake and alert but very cranky.  The head is normocephalic and atraumatic.  The conjunctivae are injected without discharge bilaterally, and the pupils are equal and reactive to light and accommodation.  There is scant nasal congestion.  The lips, tongue, and oral mucosa are erythematous, but there is no pharyngeal erythema or exudate.  Neck is supple without lymphadenopathy.  S1 and S2 are normal without murmurs; lungs are clear to auscultation bilaterally.  Abdomen is soft, nontender, and nondistended, without organomegaly.  There is nontender erythema and peeling of the skin in the perineum and inguinal folds and several erythematous, blanching macules on the trunk; there are no other skin lesions. The patient is sent home but returns to the office a day later due to worsening symptoms.  His fever has persisted and he remains extremely cranky.  His mother states that his rash has worsened and he now looks "swollen."  Temperature is 39.4 C (103 F), blood pressure is 102/74 mm Hg, pulse is 118/min, and respirations are 18/min.  On physical examination, he remains awake and alert but irritable.  The lips now appear cracked.  The neck is supple without lymphadenopathy.  S1 and S2 are normal without murmurs, lungs are clear to auscultation bilaterally, and there is no abdominal tenderness or organomegaly.  There is a diffuse erythematous, blanching, maculopapular rash on the patient's trunk, abdomen, and extremities, with increased peeling in the perianal and inguinal areas.  Edema is present in the hands and feet.  Peripheral pulses are present and symmetrical bilaterally; capillary refill is normal.  What is the most important next step in the management of this patient?

Quizplus · Accepted Answer

Explanation: 11ec2b69_c1cf_6e8e_8f63_574da27f973b_MD0005_00 Besides fever, the patient now fulfills 4 diagnostic criteria for Kawasaki disease (KD): rash, conjunctivitis, mucositis, and extremity edema/erythema. Therefore, a definitive diagnosis can be made. (The fifth diagnostic criterion, lymphadenopathy, is the least frequently observed.) The first step in treatment is administration of intravenous immunoglobulin (IVIg). It is administered in conjunction with aspirin, which reduces systemic inflammation. Although its exact mechanism of action in KD is unknown, the use of IVIg greatly improves morbidity and mortality and significantly decreases the risk of cardiac sequelae. (Choice A) Intravenous acyclovir is used for herpes encephalitis, which can cause fever, irritability, and cutaneous lesions. However, this condition often presents with focal neurologic symptoms (eg, seizures), is primarily associated with a vesicular (rather than a desquamative maculopapular) rash, and does not cause conjunctivitis and extremity edema. Although a viral etiology for KD has been suggested, none has been determined, and antivirals do not provide any benefit. (Choices C and E) Corticosteroids can treat both severe juvenile idiopathic arthritis, which can cause spiking fevers and rash but would cause arthritis, and systemic lupus erythematosus, which can cause neurologic symptoms but would be associated with other findings (eg, malar rash, oral ulcers). Neither is associated with conjunctivitis or edema. In children with KD, corticosteroids and plasmapheresis are sometimes used to reduce systemic inflammation in those who fail to respond to IVIg, but these treatments have limited clinical benefit. (Choice D) Broad-spectrum antibiotics are appropriate in the setting of serious bacterial infections or sepsis. Despite having fevers for several days, this patient has normal blood pressure and heart rate, no lethargy, and no delayed capillary refill, making bacterial infection unlikely. Educational objective: First-line treatment for Kawasaki disease includes intravenous immunoglobulin (which reduces the risk of cardiac sequelae by an unknown mechanism of action) and aspirin. References: Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance. (http://www.ncbi.nlm.nih.gov/pubmed/21168857)

A 39-Month-Old Boy Is Brought to the Office Due to Fever