Kawasaki Disease

Synonyms:

Mucocutaneous lymph node syndrome

Etiology:

Unknown

Associations:

None

Histology:

Coronary arteritis with macrophages, plasma cells, lymphocytes, and occasionally eosinophils; skin nonspecific with dermal edema and perivascular lymphocytes

Evaluation:

Throat culture, serologic testing for viral infections, urinalysis, complete blood count with differential, electrocardiogram, transthoracic echocardiography in younger patients, magnetic resonance angiography in older patients

Treatment:

Intravenous gammaglobulin and aspirin

Prognosis:

Good with treatment, 1%-2% cardiac sudden death due to coronary arteritis

Kawasaki disease, or mucocutaneous lymph node syndrome, was first described in Japan in the late 1960s as an illness characterized by persistent fever, conjunctivitis, mucous membrane changes, acral erythema with desquamation, and cervical adenopathy, associated with coronary arteritis (1,2). While earlier descriptions of the disease were limited to Asia and Hawaii, the disease is now known to occur worldwide. The disease is primarily one of young children, with 85% of cases occurring in children under five years. It is uncommon in children less than 6 months. There have been some epidemiologic investigations linking Kawasaki disease to freshly cleaned carpets, humidifier use, and living near a body of water, but these associations have not been observed consistently (3).

The etiology of Kawasaki disease is unknown. There are seasonal peaks in the winter and spring months, with occasional epidemics. There are only rare cases of the disease occurring in infants less than three months, suggesting protection via passive maternal antibodies. These findings have suggested an infectious agent, yet extensive investigations have failed to detect one. There is a recently described association between Kawasaki disease and a novel coronavirus in a small series, but further study will be required to evaluate the strength of this association (4). IgA plasma cells have been found in early and subacute lesions of Kawasaki vasculitis, suggesting the possibility of an immune response to a gastrointestinal or respiratory tract pathogen (5). Furthermore, the IgA response is oligoclonal rather than polyclonal, favoring an antigen-driven response over nonspecific B-cell activation (6). The incidence of Kawasaki disease is greater in the Asian population and in siblings and parents of those with the disease, suggesting a genetic predisposition (3).

Patients with Kawasaki disease present with persistent fever despite antibiotics, conjunctival congestion, oral dryness, redness, fissuring of the lips, strawberry tongue, and mucosal erythema (Figures 36.1 and 36.2). These findings are accompanied by acral erythema and edema, followed by desquamation in the convalescent stage (Figure 36.3). The acral erythema spreads to a truncal exanthem within three to five days (Figure 36.4). Cervical adenopathy is also a relatively constant feature. Other symptoms may include diarrhea, arthralgia or arthritis, and aseptic meningitis (2). A feature found in the majority of patients, but not emphasized in early descriptions, is perineal erythema and subsequent desquamation (Figure 36.5) (7). Small sterile pustules have also been described in some patients with Kawasaki disease, occurring symmetrically on erythematous skin on the buttocks, axillae, genitalia, and extensor surfaces (8).

Diagnostic criteria for Kawasaki disease are as follows (3):

Conjunctival Injection

Figure 36.1. Conjunctival injection. Courtesy of Naney Esterly, MD.

Cunjuctival Erythema

Figure 36.2. Lip and tongue erythema and edema. Courtesy of Nancy Esterly, MD.

Figure 36.1. Conjunctival injection. Courtesy of Naney Esterly, MD.

Figure 36.2. Lip and tongue erythema and edema. Courtesy of Nancy Esterly, MD.

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