Author
Angelika Rampal, MD
Pediatrician
Reston Town Center Pediatrics
Reston, Virginia
Disclosure: Angelika Rampal, MD, has disclosed no relevant financial relationships.
Editor
Lars Grimm, MD, MHS
House Staff
Department of Internal Medicine
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Reviewer
Robert A. Schwartz, MD
Professor and Head, Dermatology
Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health
UMDNJ-New Jersey Medical School
Newark, New Jersey
Disclosure: Robert A. Schwartz, MD, has disclosed no relevant financial relationships.
Pediatric patients are susceptible to a unique set of serious dermatologic conditions. Without prompt detection and treatment, patients may suffer significant long-term morbidity or mortality. A series of case-based examples of can't-miss pediatric dermatologic conditions is presented to help physicians identify the key distinguishing factors for each disease process. The image shown here is a 4-month-old with gangrene of her feet from meningococcemia. Image courtesy of the US Centers for Disease Control and Prevention.
A young child presents to the local ED with a fever, rash, and lymphadenopathy. An examination of his mouth demonstrates a strawberry tongue (shown here). You suspect Kawasaki disease and tell the parents that he is most likely to suffer long-term complications in which organ system?
A. Cardiovascular
B. Neurologic
C. Gastrointestinal
D. Musculoskeletal
E. Pulmonary
The correct answer is A. Kawasaki disease is an acute febrile vasculitis of unknown etiology. Epidemics in young children occur in the late winter and spring, with the highest incidence among individuals of Japanese descent. There are 3 distinct stages. In the acute stage (1-11 days), patients develop high fevers (> 100° F), ocular changes (conjunctivitis, anterior uveitis), perianal erythema, acral edema and erythema, oropharyngeal changes (strawberry tongue, hyperemia, fissuring), and lymphadenopathy. In the subacute stage (11-30 days), there may be persistent irritability, anorexia, conjunctival injection, thrombocytosis, acral desquamation (shown), and aneurysmal formation.
In the late stage (beyond 30 days), cardiac abnormalities predominate with coronary aneurysms, pericardial effusions, congestive heart failure, and myocardial infarction. The chest x-ray demonstrates coronary aneurysms in a patient with Kawasaki disease. Kawasaki disease is now the leading cause of acquired heart disease in the United States, with 25% of patients who are untreated developing cardiac complications. The main goals of treatment are to prevent coronary artery disease with aspirin and intravenous immunoglobulin, which reduces the risk for cardiac involvement to 5%. Patients are typically followed with serial echocardiograms.
A 3-year-old girl presents with a blistering rash on her face and body. Her mouth is pictured above. All of the following are true statements regarding Stevens-Johnson syndrome (SJS) EXCEPT:
A. Medications, including nonsteroidal anti-inflammatory medications (NSAIDs), sulfonamides, antiepileptics, and allopurinol, are commonly accepted triggers
B. Viruses, such as herpes simplex virus, Epstein-Barr virus, enteroviruses, and influenza, are accepted triggers
C. Malignancy can be associated with SJS
D. Bacterial etiologies include mycoplasma and group A beta-hemolytic streptococcus, among others
E. Idiopathic causes are unlikely
Image courtesy of Allen W. Mathies, MD, and the US Centers for Disease Control and Prevention.
The correct answer is E. SJS, often referred to as a more severe version of erythema multiforme, is a hypersensitivity reaction that occurs in response to various triggers, including infections, medications, and malignancy. Infectious agents include viruses, bacteria, fungi, and protozoa. Many medications, such as NSAIDs, antiepileptics, penicillins, sulfa drugs, TNF alpha antagonists, and some antidepressants, have also been implicated. More than a quarter of cases may be idiopathic. Image courtesy of Allen W. Mathies, MD, and the US Centers for Disease Control and Prevention.
SJS may involve the mucous membranes, including the eyes and gastrointestinal tract. When more than 30% of the body surface area is involved, cases are generally referred to as toxic epidermal necrolysis. Treatment is symptomatic, including treatment of superinfection and pain control. Patients with severe cases should immediately be fluid-resuscitated and treated as burn victims. Offending agents should be removed or treated. Use of steroids is controversial. Involvement of specialists, including ophthalmologists, immunologists, and burn specialists, may be indicated. Morbidity and mortality are correlated with the percentage of body surface area involved. Image courtesy of John Noble, Jr., MD, and the US Centers for Disease Control and Prevention.
A 4-month-old girl presents to the ED in extremis with rapidly developing gangrene of the extremities. All of the following are true of meningococcemia EXCEPT:
A. The mortality rate is 5%-10%
B. Meningitis is present in all cases of septicemia
C. Empiric antibiotic treatment should be initiated immediately
D. Transmission is person-to-person by direct contact via respiratory droplets
E. Gangrene is caused by arterial occlusion
Image courtesy of the US Centers for Disease Control and Prevention.
The correct answer is B. Meningococcemia is caused by the gram-negative diplococcus Neisseria meningitides. Transmission is person-to-person via respiratory droplets, often from an asymptomatic carrier. Up to 30% of teenagers and 10% of adults carry meningococci in the upper respiratory tract. The clinical presentation is variable, with 50% of patients developing meningitis only, 10% developing septicemia only, and 40% developing both. Children with meningitis are usually febrile and ill-appearing, with symptoms of lethargy, vomiting, or nuchal rigidity. Septicemia leads to capillary leak, coagulopathy, profound acidosis, and myocardial failure. Septic emboli cause arterial occlusion in the distal extremities, as shown in this infant. Image courtesy of the US Centers for Disease Control and Prevention.
Patients must be closely monitored for hypotension, shock, pericarditis, organ failure, and coagulopathy, usually in an intensive care unit setting. In patients with both septicemia and meningitis, a depressed level of consciousness may be from elevated intracranial pressure or hypotension. The mortality rate, even with prompt treatment, is 5%-10%. If meningococcemia is suspected, antibiotic treatment should be initiated without waiting for confirmatory culture testing. Patients with coagulopathy and gangrene may be candidates for anticoagulation therapy.
A child is brought into his pediatrician's office after developing a diffuse rash. His parents do not believe in vaccinations. You suspect that the child has developed measles. All of the following are true about measles EXCEPT:
A. Measles is a leading cause of death in young children worldwide
B. The classic triad is cough, coryza, and conjunctivitis
C. Koplik spots are pathognomonic white spots that appear on the buccal cheeks
D. A major early complication is acute sclerosing panencephalitis
E. Since the introduction of the measles vaccine, the annual incidence in the United States has decreased by 99%
The correct answer is D. Measles is one of the most contagious infectious diseases, with a secondary infection rate of 90% in susceptible individuals. A vaccine was introduced in 1963 which has resulted in a reduction in annual incidence in the United States of greater than 99%. However, it remains one of the leading causes of death in young children worldwide, with an estimated 197,000 deaths yearly. After exposure, the incubation period lasts for 7-14 days. Patients then develop a prodrome of high fevers, often > 104° F, with the classic triad of cough, coryza, and conjunctivitis. A couple of days later, Koplik spots develop on the buccal mucosa, appearing as white spots on an erythematous base, as shown.
Within a couple of days after the Koplik spots appear, an exanthem develops which consists of blanching, erythematous macules and papules, as shown. It begins on the face at the hairline and coalesces into patches and plaques that spread cephalocaudally to the trunk and extremities. This lasts for a week before fading to hyperpigmented patches which desquamate. Vitamin A deficiency has been associated with a worse prognosis, including blindness, so all children diagnosed with measles receive supplementation. Care is otherwise supportive with adequate hydration. Subacute sclerosing panencephalitis is a late, long-term complication caused by persistent infection of immune resistant measles.
A 6-year-old boy presents with swelling on both sides of his face and complains of pain while chewing. He has a fever of 101.1° F. He has not been vaccinated for mumps. All of the following are true statements about mumps EXCEPT:
A. It is generally a benign condition, with the majority of those infected being asymptomatic
B. Viral encephalitis is a rare but serious complication
C. Orchitis and infertility are rare complications and are less likely to occur in children
D. Mumps is not associated with hearing loss
E. Complications of mumps include meningitis, pancreatitis, oophoritis, and miscarriage
The correct answer is D. Mumps is caused by a virus that can cause inflammation of the parotid glands. It is generally a benign condition, with the majority of those infected being asymptomatic. However, certain rare but serious complications can occur. Prior to immunization against mumps, it was a major cause of hearing loss in children. Rarely it causes viral meningitis, encephalitis, and pancreatitis. Orchitis does not usually occur in children but can occur in teens and young adults. Infertility can occur in those with orchitis. Miscarriage can occur in women with mumps in the first trimester. Two recent outbreaks occurred, in 2006 and 2009, highlighting the importance of vaccination. Image courtesy of Heinz F. Eichenwald, MD, and the US Centers for Disease Control and Prevention.
An 11-year-old girl presents with raised violaceous plaques on her legs and arms that developed over the last few days. She has had a temperature of 100-101° F at home but no other complaints. Her current temperature is 100.2° F. The rest of her exam is normal. You conclude that she has Henoch-Schonlein purpura (HSP). Which of the following tests are most appropriate to perform at this point?
A. CBC, lumbar puncture
B. CBC, electrolytes, stool for occult blood, urinalysis
C. CBC, electrolytes, stool for occult blood, renal ultrasound
D. CBC, head CT, lumbar puncture, abdominal ultrasound
E. CBC, abdominal ultrasound
The correct answer is B. The images shown are purpuric lesions. In this case of a healthy-appearing child with this history and physical examination, HSP is a reasonable diagnosis. HSP is a vasculitis that can affect the skin, joints, gastrointestinal tract, and kidneys. In a well-appearing child, HSP can be managed on an outpatient basis. It is helpful to check blood pressure, urine, and electrolytes to look for a glomerulonephritis. Urinalysis and blood pressure may be followed for several months to monitor kidney function. A fecal occult blood test can help rule out significant gut involvement, especially in children with pain. Intussusception is the most serious complication of HSP; if it is suspected, the child should be admitted and monitored.
Platelet count is generally normal in HSP, helping to differentiate it from idiopathic thrombocytopenic purpura (ITP), which has low platelets. ITP presents with petechiae, bruising (as shown), or bleeding, often in areas of trauma. If counts are sufficiently low, there is a risk for intracranial bleed. A consult with hematology can help determine appropriate management in case of uncertainty.
A child is brought to the clinic to see his pediatrician after he began to develop a worrisome rash. He just got a new dog and had been playing in the woods behind his home. You suspect Rocky Mountain spotted fever (RMSF). All of the following are true regarding RMSF EXCEPT:
A. It is the most common fatal tick-borne disease in the United States
B. There is a bimodal incidence with young children and the elderly
C. Low-grade fevers are the most common symptom
D. Clinical symptoms begin 1 week after infection on average
E. Multiple tick species may serve as vectors
Image courtesy of the US Centers for Disease Control and Prevention.
The correct answer is C. RMSF is the most common fatal tick-borne disease in the United States. It is caused by Rickettsia rickettsii. Multiple ticks may serve as vectors, including the American dog tick, the common brown dog tick, and the Rocky Mountain wood tick. The incidence is highest in individuals aged 5-9 and 60-69 years. The majority of cases occur between April and September in the Southeast and Midwest United States. A tick must feed for 6 hours before infection transmission occurs, and Rickettsia replicate for an average of 1 week before clinical symptoms begin. The most common symptoms are high fever (> 102° F), headache, rash, and myalgias. Image courtesy of the US Centers for Disease Control and Prevention.
The petechial rash typically begins on the wrists and ankles but may be found anywhere, including the oral mucosa, as in this child. Spotless RMSF occurs in 10%-15% of cases. A high index of suspicion must be maintained, as the mortality rate is 20%-30% for untreated patients and 1%-5% for treated ones. Empiric antibiotic therapy is usually initiated based on the history and physical examination, without waiting for confirmatory serologic testing. There is typically a delay between presentation and diagnosis of RMSF. Up to three quarters of patients may require hospitalization. Outpatients must be closely monitored, as rapid deterioration is not uncommon and requires subsequent admission. Image courtesy of the US Centers for Disease Control and Prevention.
Author
Angelika Rampal, MD
Pediatrician
Reston Town Center Pediatrics
Reston, Virginia
Disclosure: Angelika Rampal, MD, has disclosed no relevant financial relationships.
Editor
Lars Grimm, MD, MHS
House Staff
Department of Internal Medicine
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Reviewer
Robert A. Schwartz, MD
Professor and Head, Dermatology
Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health
UMDNJ-New Jersey Medical School
Newark, New Jersey
Disclosure: Robert A. Schwartz, MD, has disclosed no relevant financial relationships.
Allergic Reactions, From Simple to Severe
Can't-Miss Findings on Noncontrast Head CT
Can't Miss Intestinal Complications on Abdominal Radiographs
Distinguishing Rheumatologic Diseases on Plain Radiography
Venomous Spiders, Keys to Identification and Essentials of Management
