Perineal Erythema in Kawasaki Disease and MIS-C

An 8-y-old boy presented with a history of fever, abdominal pain, diarrea, and a polymorphic rash. He developed vomiting, conjunctival injection, and fissured lips. Physical exam revealed adenopathy and a desquamative polymorphic eryhtematous rash over abdomen, extremities, and perineum (Fig. 1). Periungual desquamation was found in fingers of hands and feet. Laboratory tests showed anemia, leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate, C-reactive protein (CRP), pro-brain natriuretic peptide (proBNP) and fibrinogen. Reverse transcription polymerase chain reaction (RT-PCR) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was negative but immunoglobulin G (IgG) was positive. An echocardiogram showed aneurysms of left main coronary, left circumflex, left anterior descending, and right coronary arteries. The patient was treated with intravenous gammaglobulin (IVIG), methylprednisolone, enoxaparin, and aspirin. He continued to have fever and was retreated with IVIG, infliximab and cyclosporine with good response. Perineal erythema is a characteristic clinical finding in Kawasaki disease (KD) [1]. Kim et al. reported the first Korean patient with multisystemic inflammatory syndrome in children (MIS-C) who presented with perianal erythema with desquamation [2]. Mazori et al. also reported cases of MIS-C with perineal erythema [3]. The clinical similarities and response to similar therapy opens the possibility that MIS-C is a part of KD spectrum triggered by SARS-CoV2. The presence of perineal erythema gives an argument to consider MIS-C as part of the KD spectrum.

Fig. 1.

Perineal erythema with desquamation involving the diaper area

Declarations

Conflict of Interest

None.