Afebrile Kawasaki disease is not a benign form of the disease.

Abstract

We read with great interest the recent article by Yoshino et al., who reported on two Japanese patients with afebrile Kawasaki disease (KD).1 In 2008 Saltigeral-Simental et al. reported on a Mexican patient with KD without fever.2 Since that report, several cases have been reported from different countries.1, 2 We recently treated two new patients with KD who also did not present with fever. The first patient was a 1-year-old boy, who presented with a 1 week history of generalized rash and bilateral conjunctival hyperemia, erythema and desquamation in the bacille Calmette–Guérin (BCG) scar, lip fissures, and desquamation of the perineal region, edema and desquamation of the hands and feet, rhinorrhea, dry cough, diarrhea and vomiting (Fig. 1a). The following data were obtained from standard screening: hemoglobin (Hb), 12.4 g/dL; white blood cells (WBC), 10 800/μL; platelets, 433 000/μL; erythrocyte sedimentation rate (ESR), 22 mm/h; C-reactive protein (CRP), 0.3 mg/dL; aspartate aminotransferase (AST), 45 IU/L; alanine aminotransferase (ALT), 78 IU/L; and albumin, 3.9 mg/dL. Echocardiogram did not show any abnormalities. I.v. immunoglobulins (IVIG) and acetylsalicylic acid were given and the symptoms resolved. The second patient was a 1-year-old girl with erythema on her cheeks and BCG scar without fever 6 days prior to admission (Fig. 1b). On the next day, the patient had generalized polymorphic erythema, hand and feet edema and erythema in the perineal area. On the fourth day cheilitis and strawberry tongue were present. Laboratory data were as follows: albumin, 4.3 mg/dL; AST, 44 IU/L; ALT, 18 IU/L; WBC, 6.73/μL; platelets, 484 000/μL; and CRP, 0.71 mg/dL. Physical examination indicated cheilitis, BCG scar erythema, edema and erythema of the feet and hands, and perineal erythema. IVIG and aspirin were given. Echocardiogram was normal. Since the description of the disease, fever has been considered a constant finding. Similar to the Yoshida et al. patients, the present patients had erythema in the BCG inoculation site and also perineal erythema, which has been considered an important manifestation of the disease. In a previous review by Gamez-Gonzalez et al., only one patient presented BCG erythema.3 Incomplete clinical manifestations in infants are related to the development of KD-associated coronary artery abnormalities. The present patients did not have cardiac complications. Afebrile KD, however, is not a mild form of the disease given that it has been reported as a cause of sudden death in infants.4 Recently, Pinches et al. reported the case of a completely asymptomatic 3-month-old infant with KD but without fever.5 Because cardiovascular complications are modifiable with early intervention, it is necessary to consider the existence of a broad clinical spectrum in which fever is not essential, facilitating the early identification of the disease and avoiding treatment delay. Fukuda et al. emphasized the importance of non-fever symptoms as a reflection of late inflammation, which may increase coronary artery abnormalities.6 The recent American Heart Association guidelines consider fever as an indispensable sign in KD, but the Japanese guidelines give equal weight to fever and the other five symptoms.7, 8 These recent cases may help to show that KD can be present in afebrile patients. The authors declare no conflict of interest. E.V.M., M.E.N., S.S.M. contributed to data acquisition and analysis; M.G.G. contributed to data acquisition and critically revised the manuscript; and M.Y.N. contributed to conception, design, analysis and interpretation. E.V.M., M.E.N. and M.Y.N. wrote the manuscript. All authors approved the final manuscript.