Adolescent male presents with headache, upset stomach, rash
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It is back-to-school time, and you are covering the pediatric ED when an 18-year-old male presents with a familiar story. He recently started college on a hockey scholarship and was doing well until a couple of days ago, when he developed what he thought was a touch of influenza. A little headache and an upset stomach were no reasons for him to miss last nights big game against his conference rivals. Try as he might to pump himself up, however, he felt like he was dragging on the ice all night. To make matters worse, his entire upper back and neck have stiffened up, and he has had a headache he just cannot shake.
The patient spent the better part of the day in his dorm room, running to the bathroom every half hour or so to vomit. He reports being a little light-headed but figured he was just hung over from last nights after-party. Ultimately, he brought himself to the hospital after he noticed the lights in his dorm room were, literally, hurting his eyes.
Based on this history, you have narrowed your diagnosis, and the physical exam confirms your clinical suspicions. You immediately place the patient in quarantine and start him on medications that, eventually, save his life.
Which description of the rash did you most likely record in the medical record that best coincides with the patients given clinical signs and symptoms?
A.An eruption consisting of faint pink-red macules that develop rapidly into delicate teardrop vesicles on an erythematous base; the lesions quickly become pustular, umbilicated and then crusted.
B. Small (4 mm to 8 mm), rapidly ulcerating vesicles surrounded by a red areola on the buccal mucosa, tongue, soft palate and gingival. This enanthem is accompanied by red papules on the palms and soles that quickly become gray, 3 mm to 7 mm vesicles surrounded by a red halo on the hands and feet.
C. Non-blanching, 1 mm to 3 mm, angularly shaped, pink-red macules and papules with slightly raised, red borders and slightly depressed centers. The lesions are located primarily on the trunk and lower extremities; ecchymosis, bulla and necrosis are not observed.
D.Somewhat pruritic, symmetric, monomorphous, flat-topped, pink-brown papules or papulovesicles of 1 mm to 10 mm in diameter, involving the face, buttocks, forearms and extensor legs with relative sparing of the trunk.
Answer A is most consistent with a herpes simplex virus infection. Answer B represents the classic description of hand-foot-mouth disease, whereas D describes the rash of Gianotti-Crosti syndrome. Thus, the correct answer is C, as the patient was suffering an acute infection from Neisseria meningitidis, an aerobic gram-negative diplococcus.
The differential diagnosis of acute meningococcemia is extensive; other bacterial septicemias, Rocky Mountain spotted fever, toxic shock syndrome, purpura fulminans, gonococcemia, Henoch-Schonlein purpura, leptospirosis and enteroviral infections must be considered.
Thinking outside the textbook
Infection with N. meningitidis usually results in an asymptomatic carrier state that affords lifelong immunity to the specific subgroup carried. Upper respiratory symptoms, as well as bacteremia without sepsis, may also occur. When acute meningococcemia (characterized by fever, chills, hypotension and meningitis) occurs, up to two-thirds of patients may develop a skin eruption that consists, characteristically, of a petechial eruption involving the trunk and lower extremities. The petechiae are usually small, stellate and gun-metal gray with a raised border and slightly depressed vesicular or pustular center. Mucosal surfaces, including the palpebral and bulbar conjunctivae, may be involved, and the palms, soles and head are relatively spared.
In fulminant meningococcal infections, the rash may progress to ecchymosis, bullous hemorrhagic lesions, and ischemic necrosis with sloughing and eventual eschar formation. Autoamputation related to digital ischemic necrosis is a potential complication. Consumptive coagulopathy occurring in this setting portends a poor prognosis.
Notably, some patients may present with a transient, blanching, morbilliform eruption as the sole cutaneous finding. The diagnosis of meningococcemia should not, consequently, be excluded based simply on the lack of a textbook rash.
Likewise, because time is of the essence, treatment should not be delayed. Cultures for N. meningitidis from blood, skin and cerebrospinal fluid confirm the diagnosis, although these tests may have a low sensitivity after administration of antibiotics. Biopsy specimens from patients with acute meningococcemia show leukocytoclastic vasculitis and thrombosis, and organisms from skin lesions may be visualized with Grams stain. Where available, real-time polymerase chain reaction of skin tissue specimens may help confirm the diagnosis.
Patients at highest risk
Of particular relevance to this case is the CDC report that college freshmen, especially those living in dormitories, are at a slightly increased risk for meningococcal disease compared with other people of the same age.
Currently, there are two types of vaccines licensed in the United States to protect against meningococcal disease: the original meningococcal polysaccharide vaccine, or MPSV4 (Menomune, Sanofi-Pasteur), and the newer quadrivalent meningococcal conjugate vaccine, or MCV4 (Menactra, Sanofi-Aventis; Menveo, Novartis). Both protect against the A, C, Y and W-135 subtypes of meningococcus.
ACIP recommends that incoming and current college freshmen (and their parents) who plan to or already reside in dormitories and residence halls should be informed about the risks for meningococcal disease and the benefits of vaccination. College freshmen who want to reduce their risk for meningococcal disease should be vaccinated. Likewise, because the vaccine is safe and effective, it can also be provided to non-freshmen students who want to reduce their risk for meningococcal disease.
Thankfully, most states have adopted legislation that requires colleges to provide information on risks for meningococcal disease to incoming students and/or students residing on campus. Some states now mandate vaccination for certain students, unless a vaccination waiver is provided.
For more information, check out: www.cdc.gov/meningitis.
Andrew C. Krakowski, MD, completed a residency in pediatrics at Johns Hopkins Medical Institute and a residency in dermatology at University of California, San Diego. He is currently a fellow in pediatric dermatology at Rady Childrens Hospital, San Diego. Catch him on Outdoor Channel as the host of boonDOCS Wilderness & Travel Medicine Show (email:dr.k@boonDOCSmedicine.com). Disclosure: Dr. Krakowski reports no relevant financial disclosures.
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