Kawasaki disease in a US army soldier highlights surveillance

Abstract

A 26-year-old otherwise healthy black US army soldier was admitted to an army community hospital for the assessment of a fever and widespread eruption unresponsive to antibiotics and oral steroids. Important laboratory values included a C-reactive protein of 14.4 mg/dL (normal reference range, < 3), a white blood cell (WBC) count of 14,000 cells/mL (normal reference range, 5000–10,000 cells/mL), and a platelet count of 469,000/mL (normal reference range, 150,000–350,000/mL). A skin biopsy showed bland dermatitis, and a touch preparation grew Group B streptococcus. Blood cultures were negative and hepatitis, human immunodeficiency virus (HIV), syphilis, and malaria were ruled out. Empiric ceftriaxone and azithromycin were administered, but the signs worsened. On transfer to our center, the patient had a temperature of 101°F, widespread cutaneous desquamation, bilateral conjunctival suffusion, a beefy red tongue, and bilateral inguinal lymphadenopathy. There were no mucosal lesions, organomegaly, cervical lymphadenopathy, perineal erythema, or penile ulcers. Chest, abdominal, and pelvic computerized tomography (CT) scans were normal. A differential diagnosis included occult abscess or viral infection, scalded skin syndrome, exfoliative erythroderma, Stevens–Johnson syndrome, Sezary syndrome, and Kawasaki disease (KD). He received clindamycin, but there was no improvement. On dermatology consultation about 10 days after presentation, the patient had bilateral conjunctivitis, a beefy red tongue, generalized desquamation, especially prominent on the hands (Fig. 1), and inguinal lymphadenopathy. The differential diagnosis was narrowed to KD and exfoliative erythroderma. Fever persisted, and a blood test showed 499,000 platelets/mL and leukocytosis. KD was diagnosed and he immediately received high-dose aspirin (650 mg orally four times a day for 3 days, then 325 mg/day orally) and intravenous immunoglobulin (IVIG; 1 g/kg daily for 2 days). A repeat chest CT scan and echocardiogram showed no abnormalities. Within 1 day of treatment, the patient became afebrile and the leukocytosis started to normalize rapidly. Three days after completing IVIG, the patient was discharged with aspirin therapy until follow-up with his primary care provider to include an echocardiogram. Patient with Kawasaki disease showing bilateral conjunctivitis, a beefy red tongue, and hand desquamation KD, first described in 1967 by Tomisaku Kawasaki, is a febrile illness of unclear etiology, most commonly diagnosed in young children.1 Vasculitis is a cardinal feature of KD, accounting for potential cardiac-related sequelae such as coronary aneurysms. It is estimated that up to 25% of children who go untreated develop serious cardiovascular sequelae.1 Other cardinal features include a desquamating eruption, mucosal involvement, and thrombocytopenia,2 as noted here. Although KD is rare in adults,3 this report illustrates the importance of considering KD in adults presenting with a febrile illness and eruption of unclear etiology. KD was not initially on the original differential diagnosis list because of its low incidence in adults, but the lack of any infectious, malignant, or medication etiology, fever despite antibiotics, a bland skin biopsy, and characteristic cutaneous signs widened the differential diagnosis. On diagnosis, the rapid response to IVIG and aspirin was especially encouraging,4 with nearly complete resolution of all signs and symptoms within 2–3 days and no cardiovascular sequelae at the time of hospital discharge. The etiology of KD remains unclear.2 KD occurs seasonally and in epidemics, suggesting an infectious etiology.2 KD may also represent an immune reaction against a staphyloccocal or streptococcal superantigen.5 Our patient had a negative antistreptolysin O antibody titer, and the relevance of Group B streptococcus cultured from the skin was unclear. A KD-like syndrome has been reported in HIV-positive adults,6, 7 but a negative HIV serology about 2 months before presentation argued against HIV-related disease. This case underscores the importance of including KD in the differential diagnosis of adults presenting with fever and eruption, and demonstrates the effectiveness of aspirin and IVIG in resolving adult KD.