Dermatologic Manifestations of Kawasaki Disease
- Author: Elizabeth Kline Satter, MD, MPH; Chief Editor: Dirk M Elston, MD more...
Overview
Kawasaki disease is a dynamic illness with various features that are most pronounced at different times. (See the chart below.) The most characteristic features of Kawasaki disease are usually present at the time of presentation or appear shortly thereafter.[1, 2, 3]
Clinical manifestations and time course of Kawasaki disease. Courtesy of Paul R. Ogershok, MD. The 5 major clinical findings in Kawasaki disease are as follows:
- Change in the extremities, typically painful erythema and edema
- Polymorphous exanthem
- Changes in the lips and oropharyngeal mucosa
- Bilateral, nonexudative, bulbar conjunctival injection
- Unilateral, nonsuppurative cervical lymphadenopathy
Go to Kawasaki Disease and Ophthalmologic Manifestations of Kawasaki Disease for complete information on these topics.
Dermatologic Presentation
More than 90% of patients present with a polymorphic exanthema within 3-5 days of the onset of fever. Initially, the patient may present with nonspecific erythema of the palms, soles, and perineal regions, which gradually and diffusely involves the trunk and extremities. The eruption is usually pruritic and can be macular, papular, morbilliform (as in the first image below), scarlatiniform, urticarial, erythrodermatous, targetoid, or composed of fine micropustules, but it is never vesicular or bullous. Within a few days after the onset of the eruption, a fine desquamation of the perineal region occurs. (See the second image below.)
Morbilliform eruption in Kawasaki disease. Courtesy of Noah S. Scheinfeld, MD, JD.
Desquamation in a patient with mucocutaneous lymph node syndrome (Kawasaki disease). Courtesy of Chemene Y. Quinn, MD. The extremities show distinctive changes in 94% of patients. After the initial acral erythema, the palms and soles gradually become indurated and painful, which may limit mobility. Then, during the subacute phase of the illness, approximately 14 days after the onset of fever, desquamation occurs in a glovelike fashion, first involving the periungual region of the fingers and followed a week later by a similar desquamation of the toes. (See the image below.)
Acral desquamation during the subacute phase of the illness. Courtesy of Noah S. Scheinfeld, MD, JD. Other Manifestations
More than 95% of Kawasaki disease patients present with protracted fever, which has a remittent pattern of several temperature spikes each day. The patient is ill and often extremely irritable.
Mucous membrane and oropharyngeal alterations begin within the first few days after the onset of fever. Changes in the lips are seen in 75-90% of patients and include erythema, dryness, cracking, and bleeding. From 50-77% of patients have an extremely erythematous tongue, with protuberance of the fungiform papillae, which has been referred to as strawberry tongue. This feature, while clinically impressive, is indistinguishable from that seen in patients with scarlet fever, a streptococcal infection. (See the image below.)
Oral manifestations of Kawasaki disease: red lips and strawberry tongue. Courtesy of Paul R. Ogershok, MD. Bilateral, nonexudative, bulbar conjunctival injection with relative sparing around the limbus occurs within a few days after the onset of fever and lasts 1-3 weeks in approximately 88% of patients. (See the image below.) Mild iridocyclitis or anterior uveitis may be seen during a slit-lamp examination but is rarely associated with eye pain or photophobia.
Bulbar conjunctiva congestion in a patient with mucocutaneous lymph node syndrome (Kawasaki disease). Courtesy of Chemene Y. Quinn, MD. Go to Ophthalmologic Manifestations of Kawasaki Disease for complete information on this topic.
The least common of the 5 major diagnostic criteria for Kawasaki disease is a unilateral, nontender, nonsuppurative, anterior cervical lymphadenopathy. This typically involves a single node larger than 1.5 cm in diameter. This finding is only seen in 70% of cases in Japan and in 50-86% of cases in the United States. Although uncommon, Kawasaki disease patients who present with only fever and cervical lymphadenopathy at admission typically have a greater risk of developing coronary artery abnormalities and are at risk of being nonresponders to intravenous immunoglobulin treatment.[4]
The last feature seen during the acute febrile stage is cardiovascular alterations. Cardiac auscultation may reveal various findings, such as a hyperdynamic precordium, tachycardia, a gallop rhythm, or an innocent flow murmur. These findings are nonspecific and may be initially attributed to mild anemia and/or fever.
Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. Mar 1967;16(3):178-222. [Medline].
Rowley AH, Shulman ST. Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther. Feb 2010;8(2):197-203. [Medline].
Han RK, Sinclair B, Newman A, et al. Recognition and management of Kawasaki disease. CMAJ. Mar 21 2000;162(6):807-12. [Medline].
Nomura Y, Arata M, Koriyama C, et al. A Severe Form of Kawasaki Disease Presenting with Only Fever and Cervical Lymphadenopathy at Admission. J Pediatr. Jan 22 2010;[Medline].

