Introduction
Acute rheumatic fever remains an important cause of acquired cardiovascular disease among children in endemic regions and continues to pose diagnostic challenges because of its diverse clinical manifestations. The revised Jones criteria recognise carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules as major manifestations of the disease. Reference Gewitz, Baltimore and Tani1 Among the major Jones manifestations, erythema marginatum and subcutaneous nodules are uncommon findings, reported in fewer than 6% and up to 10% of patients, respectively, and are frequently associated with carditis. Reference Gewitz, Baltimore and Tani1–Reference Dougherty, Nascimento, Carapetis, Dougherty, Carapetis, Zühlke and Wilson3 Recognition of erythema marginatum may be further hindered by its evanescent nature and reduced conspicuity in darkly pigmented skin, potentially contributing to under-recognition in endemic populations. Reference Gewitz, Baltimore and Tani1,Reference Sharma and Biswal4
We report a child with acute rheumatic fever fulfilling three major Jones manifestations—migratory polyarthritis, erythema marginatum, and subcutaneous nodules—in the complete absence of clinical, electrocardiographic, or echocardiographic evidence of carditis despite serial follow-up. The case highlights an uncommon clinical phenotype and provides serial photographic documentation of the evolving appearance of erythema marginatum in dark skin.
Case report
A previously healthy 13-year-old boy from eastern India, a moderate- to high-risk region for acute rheumatic fever, presented with fever and sore throat accompanied by a transient truncal maculopapular rash that appeared before initiation of amoxicillin therapy. He subsequently received a 7-day course of amoxicillin with symptomatic therapy, following which the acute illness resolved. Approximately four weeks later, he developed fever (maximum recorded temperature> 101°F) lasting approximately 2–3 days, followed by severe migratory polyarthritis, initially involving the left hip and subsequently both knees, resulting in inability to walk. During the same period, multiple non-pruritic annular and serpiginous erythematous lesions with central clearing appeared over the trunk and shoulders, consistent with erythema marginatum (Figure 1A–G). The eruption persisted for a few days before resolving. Over the following week, firm, painless, non-tender subcutaneous nodules developed over the forehead and along the spine (Figure 2A–B). The spinal nodules remained visible irrespective of posture.
Erythema marginatum. (A,B) Upper chest demonstrating the temporal evolution of erythema marginatum with annular and polycyclic erythematous lesions showing partial central clearing. (C) Right lower trunk and glutaeal region showing multiple serpiginous lesions in a characteristic truncal distribution. (D,E) Left shoulder and (F,G) right shoulder demonstrating evolving annular and serpiginous lesions, highlighting the dynamic and evanescent nature of the rash. The morphology and distribution of the lesions changed noticeably over short time intervals during clinical observation.

(A,B) Subcutaneous nodules over the spinal region. (C) Ocular inflammatory appearance with prominent conjunctival and circumcorneal congestion, more marked in the right eye. (D) Swelling of the lips, predominantly involving the lower lip. (E) Electrocardiogram showing normal sinus rhythm with a normal PR interval. (F,G) Parasternal long-axis and short-axis echocardiographic views with colour Doppler demonstrating no significant mitral or aortic regurgitation and no echocardiographic evidence of carditis.

Laboratory investigations demonstrated elevated inflammatory markers with an erythrocyte sedimentation rate of 60 mm/h and a C-reactive protein level of 30.6 mg/L. Evidence of recent streptococcal infection was confirmed by an antistreptolysin-O titre of 720 IU at presentation, rising to 1950 IU four weeks later. Antinuclear antibody, rheumatoid factor, and blood cultures were negative. Based on migratory polyarthritis, erythema marginatum, subcutaneous nodules, elevated acute-phase reactants, and evidence of preceding streptococcal infection, the patient fulfilled the 2015 revised Jones criteria for definite acute rheumatic fever.
There was no clinical evidence of carditis. Serial electrocardiograms demonstrated sinus rhythm with normal PR intervals (Figure 2E). Transthoracic echocardiography showed structurally normal cardiac valves, preserved biventricular systolic function, and no significant mitral, aortic, tricuspid valvar regurgitation (Figure 2F–G; Movie 1). No morphological or Doppler evidence of clinical or subclinical carditis was identified. Repeat electrocardiographic and echocardiographic evaluations during follow-up remained unchanged, confirming persistent absence of cardiac involvement over three months.
The patient was treated with anti-inflammatory therapy, aspirin for six weeks, antibiotic therapy, and secondary prophylaxis. Joint pain improved markedly within 24 hours of initiating aspirin therapy. During convalescence, transient swelling of the lips was observed (Figure 2D), followed by bilateral ocular redness with circumcorneal congestion, more prominent in the right eye (Figure 2C). No alternative cause was identified. Although slit-lamp examination was not performed, the ocular symptoms resolved promptly with topical fluorometholone. Inflammatory markers normalised during follow-up, and all clinical manifestations resolved completely. The child has had no recurrence of arthritis, joint swelling, or ocular inflammation and remains on active clinical follow-up. At three months, the patient remained asymptomatic without recurrence of symptoms or development of cardiac involvement.
Discussion
This case highlights an unusual presentation of acute rheumatic fever characterised by the simultaneous occurrence of three major Jones manifestations—migratory polyarthritis, erythema marginatum, and subcutaneous nodules—in the complete absence of clinical, electrocardiographic, or echocardiographic evidence of carditis. Erythema marginatum and subcutaneous nodules are uncommon manifestations of acute rheumatic fever and have long been recognised to occur in association with carditis. Reference Gewitz, Baltimore and Tani1–Reference Dougherty, Nascimento, Carapetis, Dougherty, Carapetis, Zühlke and Wilson3 The 2015 Jones criteria revision notes that subcutaneous nodules are more frequently observed in patients with carditis and that erythema marginatum and nodules rarely occur as isolated manifestations. Reference Gewitz, Baltimore and Tani1 Against this background, the coexistence of these two cutaneous major manifestations in a patient with persistently normal cardiac evaluation represents an uncommon clinical phenotype.
The well-recognised association between subcutaneous nodules and carditis makes the present case particularly noteworthy. Major reference texts consistently describe subcutaneous nodules as typically accompanying significant cardiac involvement. Reference Lennon, Webb, Cherry, Kaplan, Demmler-Harrison, Steinbach, Hotez and Williams2,Reference Dougherty, Nascimento, Carapetis, Dougherty, Carapetis, Zühlke and Wilson3 In a previously reported child with acute rheumatic fever and extensive subcutaneous nodules, the nodules occurred in association with severe rheumatic carditis, significant mitral regurgitation, and PR interval prolongation. Reference Singhi, Bobhate and Kappanayil5 Similarly, in the Brazilian series reported by Robazzi et al., the only patient exhibiting the combination of arthritis, erythema marginatum, and subcutaneous nodules also had carditis. Reference Robazzi, de Araújo, Costa Sde, de Oliveira Júnior, Nunes and Guimarães6 In contrast, our patient demonstrated persistently normal electrocardiograms and serial echocardiographic examinations over three months of follow-up, with no evidence of either clinical or subclinical cardiac involvement.
Findings from larger cohorts are consistent with this observation. In a South China cohort, carditis was a predominant manifestation, whereas erythema marginatum and subcutaneous nodules were infrequent findings. Reference Chen, Xie, Gu, Xu, Yang and Yu7 In contrast, our patient fulfilled three major Jones manifestations, including both cutaneous major criteria, yet remained free of clinical, electrocardiographic, and echocardiographic evidence of cardiac involvement throughout follow-up. Collectively, these observations suggest that the coexistence of arthritis, erythema marginatum, and subcutaneous nodules without accompanying carditis is distinctly uncommon.
An additional educational aspect of this report is the serial photographic documentation of erythema marginatum. The rash evolved over several days, displaying changing annular, polycyclic, and serpiginous morphologies with central clearing. Recognition of erythema marginatum may be challenging because of its evanescent nature and because lesions may be less conspicuous in darkly pigmented skin. Previous reports and guideline documents have highlighted the potential for under-recognition of this manifestation in darker skin tones. Reference Gewitz, Baltimore and Tani1,Reference Sharma and Biswal4,Reference Animasahun, Lawani and Lamina8 The serial images presented in this report may therefore assist clinicians practising in endemic regions where acute rheumatic fever remains prevalent but erythema marginatum is infrequently documented.
The persistent absence of carditis also deserves emphasis. Previous reports have demonstrated that cardiac involvement may develop after an initially normal cardiac assessment. Khriesat and Najada described four patients with acute rheumatic fever in whom carditis was absent at presentation but subsequently became apparent during follow-up, underscoring the importance of serial echocardiographic surveillance. Reference Khriesat and Najada9 In contrast, our patient underwent repeated clinical evaluation, electrocardiographic and echocardiographic assessments, including serial follow-up examinations over three months, all of which remained normal. These findings strengthen the conclusion that cardiac involvement was genuinely absent rather than simply delayed in onset.
A further observation was the occurrence of transient inflammatory manifestations during convalescence, including swelling of the lips and subsequent bilateral ocular redness with circumcorneal congestion. Although slit-lamp examination was not performed and a specific ophthalmological diagnosis could not be established, the temporal association with the acute rheumatic episode is noteworthy. Ocular inflammatory manifestations have only rarely been described in association with rheumatic fever and post-streptococcal immune syndromes. Ortiz and colleagues reported severe bilateral scleritis, uveitis, and secondary glaucoma in a patient with rheumatic fever, suggesting that ocular inflammation may occur within the broader spectrum of post-streptococcal immune-mediated disease. Reference Ortiz, Kamerling, Fischer and Baxter10 However, the significance of the lip swelling and ocular findings observed in our patient remains uncertain. In the absence of definitive ophthalmological evaluation and given the self-limited nature of these manifestations, only a temporal association with the acute rheumatic episode can be inferred.
Conclusion
This case illustrates an uncommon phenotype of acute rheumatic fever in which three major Jones manifestations—migratory polyarthritis, erythema marginatum, and subcutaneous nodules—occurred despite persistent absence of clinical, electrocardiographic, and echocardiographic evidence of carditis. The case highlights the continuing clinical value of careful recognition of extracardiac manifestations of acute rheumatic fever and emphasises that definite rheumatic fever may occur even when cardiac involvement is not demonstrable. Ongoing clinical and echocardiographic follow-up remains important to confirm the absence of delayed cardiac manifestations.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S1047951126124743.
Acknowledgements
The authors gratefully acknowledge all physicians, nurses, allied health professionals, and other healthcare personnel involved in the care of this patient. The authors also thank the hospital administration for their support. The authors further acknowledge the Dr Anil Singhi Foundation for its generous academic support of our scientific research endeavours.
Financial support
None.
Competing interests
The authors declare no conflict of interest.
Ethical standard
This study was conducted in accordance with the approved protocol of the Institutional Clinical Ethics and Research Committee (CERC/2021/Jun/iv). Patient confidentiality was maintained through complete anonymization of all clinical data and images. Written informed consent for publication was obtained from the patient’s legal guardian.
Clinical trial grant/support
Not applicable.