A Rare, Recurrent, Red, Ringed Rash in a Young Girl
Patient also has fever, fatigue, and nonpainful nodules with the rash, which is worse after exertion. What is your diagnosis?
Rounds for a Rare Recurrent, Red, Ringed Rash: Follow the case of Scarlet Tina Lancefield, from our collection of Dr Jonathan Schneider's classic case studies that focus on the nuts and bolts of clinical assessment and differential diagnosis. What is wrong with Scarlet? Click through the slides to follow the case.
An 8-year-old girl recovered from an apparent cold with sore throat and subsequently developed painful bumps on limbs, fatigue, rash, and neurologic symptoms.
Nonpainful bumps appeared on surface of skin on elbows/upper arms & legs. Round reddish rash on arms, chest, abdomen comes and goes; reappears after exertion, bathing. No travel, exposure to Lyme.
The eruption is erythematous and appears as multiple ringed lesions varying from a few mm to several cm in size. There is no evidence that they are pruritic (no scratch marks).
Physical exam:Slightly elevated heart rate, body temperature. Nodules are noted over areas of tendon insertions behind elbows, knees. A mid-diastolic murmur is heard at at the mitral area.
ECG: Revealed a second degree AV block (of Mobitz type 1).
The distinctive rash: No facial involvement. Lesions are evanescent; fade over several hours to days. Some are macular, some papular. They vary in size from a few mm to several cm.
Recap of exam: Carditis/pericarditis with mitral valve insufficiency; arthritis; subcu nodules; erythema marginatum.
A short poem is recited as a reminder of symptoms that add up to Jones Criteria.
Symptoms appear to suggest acute rheumatic fever: Carditis, Arthritis, Nodules (subcutaneous), Chorea, ERythema marginatum = C. A. N. C. E. R.
Differential diagnosis:Acute rheumatic fever (ARF)*; Lyme disease with erythema migrans chronicum; bacterial endocarditis; Still disease (juvenile rheumatoid arthritis); post-streptococcal arthritis; Kawasaki disease; SLE; erythema annulare centrifugum
Work-up:Rapid strep test, possible throat culture if test is negative; antistreptococcal antibodies, specifically ASO, anti-DNPase
Work-up: Acute phase reactants (CRP, ESR, CBC, UA); chest x-ray; serial echocardiography/Doppler studies.
Test results:Rapid strep and throat culture negative; Antistreptococcal antibodies-- ASO, elevated; anti-DNPase, elevated.
Test results:Acute phase reactants CRP, ESR, WBC elevated; UA, no signs of renal failure; CXR – bilateral diffuse infiltrates, cardiomegaly
Cardiology work-up results: Mitral valvulitis; mitral regurgitation, per AHA criteria.
Diagnosis: Acute Rheumatic Fever: In brief: Latency, 2-4 wks after GAS infection; most common ages 5 to 15 years; acute febrile illness with migratory arthritis, carditis/valvulitis, skin lesions, neurologic disturbances
Scarlet followed up by cardiology. Jones Criteria should be familiar to primary care physicians.
Thomas Duckett Jones, MD is recognized for his pioneering work on group A streptococci disease and its association with rheumatic disease.
Modified Jones Criteria
Jones Criteria includes minor criteria: C.A.F.E P.A.L.S. for CRP increased, Arthralgias, Fever, ESR elevated, Prolonged PR interval, Anamnesis of rheumatism, Leukocytosis
Diagnosis of Rheumatic Fever using 2015 Jones Criteria: Requires 2 major criteria or 1 major and 2 minor criteria plus evidence of recent group A streptococcal infection (ie, positive throat culture or elevated ASO titers)
Resources
Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. A Scientific Statement from the American Heart Association. Circulation. 2015; Originally published April 23, 2015 CIR.0000000000000205.
Shulman ST, Bisno AL, Clegg HW, et al. Clinical practice guideline for the diagnosis and management of group A streptococcal pharyngitis: 2012 update by the Infectious Diseases Society of America. Clin Infect Dis. 2012;55:e86-102.
Webb RH, Grant C, Harnden A. Acute rheumatic fever [clinical review]. BMJ 2015;351:h3443
Tracing the development of the Jones Criteria (interesting historic perspective for the curious) https://www.kaleidahealth.org/childrens/grandrounds/pdfs/Grand_Rounds_111414.pdf
A typically active 8-year-old girl is seen after the holiday season for symptoms that intially don't seem connected to the scratchy throat and cold she experienced, along with many of her classmates, around Thanksgiving time.While that ilness is long-resolved, there are troublesome new symptoms, including bumps near joint insertions, severe fatigue, and an evanescent rash, that have her mother extremely concerned.
