LETTER TO THE EDITOR

Kawasaki disease without fever in a Costa Rican girl, first reportfrom Central America

Rolando Ulloa-Gutierrez • Alexandra Perez •

Rafael Gutierrez-Alvarez

Received: 11 April 2012 / Accepted: 21 October 2012

� Springer-Verlag Berlin Heidelberg 2012

Dear Editor,

We read with great interest the two recent papers on Ka-

wasaki disease (KD) without fever published recently by

Kato et al. [1] and Rodriguez-Lozano et al. [2]. We

describe a girl with all classic KD symptoms except for

fever, seen at the only pediatric tertiary referral and

teaching hospital of Costa Rica.

A 5-year-old girl was seen four times during a two-week

period at the emergency outpatient clinic of our hospital,

initially for a cervical lymphadenopathy for which oral

amoxicillin was prescribed. She had no clinical improve-

ment, and therefore, one dose of intramuscular benzathine

penicillin was added 48 h after. She developed an ery-

thematous skin rash, predominantly in the chest, trunk,

neck, extremities, and genital area, and she was switched to

oral cephalexin. However, a non-suppurative significant

conjunctival injection, oral mucositis, evident strawberry

tongue, erythematous and edematous palms and soles,

hyporexia, and an acutely ill aspect appeared. No pharyn-

gitis or tonsillitis was seen. Although her mother denied

fever at all and she was afebrile on physical examination at

all visits, KD was clinically suspected. A CRP was

102 mg/L, platelets were 708,000/mm3, and viral serolo-

gies and an ASO titer were negative. An infectious disease

consultation was made, and the possibility of KD was

agreed as the most likely diagnosis, except for the absence

of fever. An echocardiogram at this point was normal;

therefore, neither aspirin nor IVIG were given, and an

outpatient appointment was scheduled.

Two months after, evident bilateral Beau’s lines were

noted in all her fingers during an appointment in the

infectious disease clinic, and her mother mentioned that the

child developed bilateral periungual peeling. A repeat ASO

test and echocardiograms were negative, and her platelets

were normal. In retrospective, the diagnosis of KD without

fever was made.

In Costa Rica, KD is the leading cause of acquired

cardiac disease in children [3]. As we recently described,

the disease burden of KD in Central American children is

unknown, and it is an overlooked disease, especially when

atypical presentations occur [4]. As recently summarized

by Rodriguez-Lozano et al. [2], there have been few pub-

lications in Spanish [5] and English [1, 6] on children with

KD without manifesting fever. As opposed to these pre-

vious reports, our patient did not develop coronary aneu-

rysms. We reviewed the literature, these papers and did a

crossed search with the Science Citation Index (SCI). This

is the first report of KD without fever in children from

Costa Rica and Central America.

Conflict of interest The authors declare that they have no conflict

of interest.

R. Ulloa-Gutierrez (&)

Servicio de Infectologıa Pediatrica,

Hospital Nacional de Ninos de Costa Rica,

Dr. Carlos Saenz Herrera, Avenida Paseo Colon,

PO Box 1654-1000 San Jose, Costa Rica

e-mail: rolandoug@racsa.co.cr

A. Perez

Servicio de Emergencias Pediatricas,

Hospital Nacional de Ninos de Costa Rica,

Dr. Carlos Saenz Herrera, San Jose, Costa Rica

R. Gutierrez-Alvarez

Servicio de Cardiologıa Pediatrica,

Hospital Nacional de Ninos de Costa Rica,

Dr. Carlos Saenz Herrera, San Jose, Costa Rica

123

Rheumatol Int

DOI 10.1007/s00296-012-2563-5

References

1. Kato T, Numaguchi A, Ando H, Yasui M, Kishimoto Y, Yasuda K,

Fukumi D, Yasuda T (2012) Coronary arterial ectasia in a 2-year-

old boy showing two symptoms of Kawasaki disease without

manifesting fever. Rheumatol Int 32:1101–1103

2. Rodriguez-Lozano AL, Rivas-Larrauri FE, Hernandez-Bautista

VM, Yamazaki-Nakashimada MA (2011) Fever is not always

present in Kawasaki disease. Rheumatol Int (Epub ahead of print)

3. Ulloa-Gutierrez R (2007) Kawasaki syndrome in Costa Rican

children. In: The 10th International Kawasaki Disease Symposium,

Kyoto, Japan. Feb 7–10, 2012. Abstract 0–4

4. Ulloa-Gutierrez R, Alegrıa M, Estripeaut D, Gamero M, Rodrıguez

FJ, Hernandez-de Mezerville M, Saez-Llorens X, Somarriba MM,

Grazioso C, Melgar M, Luque MT, Tremoulet A (2011) Lack of

reports and publications on Kawasaki disease in children from

Central America, period 2000–2010. 7th World Congress of the

World Society for Pediatric Infectious Diseases (WSPID), Mel-

bourne, Australia. Nov 16–19, 2011

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Amador K, Yamazaki-Nakashimada MA (2008) Enfermedad de

Kawasaki. >La fiebre debe ser un criterio obligatorio? Rev Alerg

Mex 55:176–179

6. Hinze CH, Graham TB, Sutherell JS (2009) Kawasaki disease

without fever. Pediatr Infect Dis J 28:927–928

Rheumatol Int

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