Abstract
Kawasaki disease rarely affects adults, and recurrence is extremely uncommon. This report describes a 21-year-old man with Down syndrome who developed a second episode of KD four years after the first. The patient presented with fever, rash, extremity oedema, mucosal changes, and lymphadenopathy. Laboratory tests showed inflammation and temporary renal impairment, while cardiac examinations remained normal. Treatment with IVIG and aspirin led to full recovery, with earlier IVIG administration during the recurrence. The case underscores the importance of considering KD in adults with febrile mucocutaneous symptoms and highlights the need for early treatment and long-term cardiac follow-up.
Keywords
kawasaki disease, adult, recurrence, systemic vasculitis, intravenous immunoglobulin
Introduction
Kawasaki disease (KD) is an acute systemic vasculitis of medium-sized vessels that mainly affects children under 5 years of age. Adult-onset KD is rare, with fewer than 100 cases reported in the literature [1-3]. Recurrence of KD in adults is exceptional and represents a diagnostic and therapeutic challenge [2].
Case presentation
We report the case of a 21-year-old man with Down syndrome and psychomotor delay, previously diagnosed with KD at the age of 16. The patient presented again, four years later, with fever (39–40 °C), scarlatiniform rash, oedema of the hands and feet (Figure 1), strawberry tongue, erythematous and dry oral mucosa, and cervical lymphadenopathy (Figure 2). Cardiac assessment, including ECG and echocardiography, was normal during both admissions [4]. Laboratory findings at the first hospitalization showed hepatic cytolysis, cholestasis, normocytic hypochromic anaemia, renal impairment (MDRD clearance 46 ml/min), elevated CRP (98 mg/L), and negative HIV, CMV, EBV and Covid 19 serologies. Similar results were observed at the second hospitalization, except for normal liver function tests. The patient was treated with aspirin and intravenous immunoglobulin (IVIG), administered late (day 25) during the first episode and earlier (day 7) during the recurrence. The clinical and biological course was favourable in both episodes.
Figure 1. Cutaneous rash involving trunk, hands, and feet with oedema
Figure 2. Cervical lymphadenopathy
Discussion
Adult-onset KD is rare and often under-recognized, with clinical features that may overlap with other systemic vasculitis’s, infectious syndromes, or drug reactions [3]. While paediatric KD has been extensively described, adult presentations remain less standardized, frequently leading to diagnostic delays. In our patient, the constellation of fever, mucocutaneous changes, extremity involvement (Figure 1), and lymphadenopathy (Figure 2) fulfilled the classical diagnostic criteria. The absence of cardiac involvement is noteworthy, as coronary aneurysms remain the most feared complication, particularly in untreated or late-treated cases.
Recurrence of KD in adults is exceptionally uncommon. The few published cases suggest that recurrence may occur years after the initial episode and present with similar clinical features. Factors predisposing to recurrence remain unclear but may involve host genetic susceptibility and immune dysregulation. Our case highlights the importance of early intravenous immunoglobulin (IVIG) therapy, which was administered late during the first episode and earlier during the recurrence, with consistently favourable outcomes. This emphasizes that prompt treatment remains effective in preventing severe complications, even in adult and recurrent forms [4]. Long-term follow-up, including cardiovascular evaluation and risk factor management, is therefore essential.
Conclusion
Recurrent KD in adults is an extremely rare entity. Although less likely to result in coronary aneurysms compared to children, adult KD requires systematic cardiovascular evaluation and long-term follow-up. Physicians should maintain a high index of suspicion when faced with febrile mucocutaneous syndromes in adults.
Conflicts of interest
The authors declare that there are no competing interests.
References
- Gomard-Mennesson E, Landron C, Dauphin C, Epaulard O, Petit C, et al. (2010) Kawasaki disease in adults: Report of 10 cases. Medicine (Baltimore) 89: 149-158. [Crossref]
- Jones TP, Petrie C, Sykes C (2021) Adult-onset recurrent Kawasaki disease: An unusual cause of fever of unknown origin. BMJ Case Rep 14: e243704. [Crossref]
- Sève P, Stankovic K, Smail A, Durand DV, Marchand G, et al. (2005) Adult Kawasaki disease: Report of two cases and literature review. Semin Arthritis Rheum 34: 785-792. [Crossref]
- Fraison JB, Sève P, Dauphin C, Mahr A, Gomard-Mennesson E, et al. (2016) Kawasaki disease in adults: Observations in France and literature review. Autoimmun Rev 15: 242-249. [Crossref]
