SARS-CoV-2–Induced Kawasaki-Like Hyperinflammatory Syndrome: A Novel COVID Phenotype in Children

Abstract

We describe 2 children with persistent fever and profuse diarrhea who developed signs of mucocutaneous involvement (conjunctivitis, fissured lips, skin rash, erythema, and edema of the hands and feet). Blood tests revealed elevated markers of inflammation, lymphopenia, thrombocytopenia, and complement consumption. Afterward, diffuse edema with hypoalbuminemia appeared in the context of a capillary leak syndrome. In both patients, repeated nasal swabs were negative for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), but each patient had high titers of immunoglobulin G and immunoglobulin M against the SARS-CoV-2 virus. The negative PCR results in the presence of immunoglobulin M and immunoglobulin G suggested that the inflammatory response developed in the late phase of viral infection, when SARS-CoV-2 was not detectable in the upper airway. In this report, we describe patients with what we propose to name as SARS-CoV-2–induced Kawasaki-like hyperinflammatory syndrome. SARS-CoV-2–induced Kawasaki-like hyperinflammatory syndrome seems to be caused by a delayed response to SARS-CoV-2. It resembles Kawasaki disease complicated by macrophage activation syndrome, although it has peculiar features, such as prodromal diarrhea, capillary leak syndrome, and myocardial dysfunction. Intravenous corticosteroid treatment appears to be helpful.

Dermatologic manifestations of COVID-19-associated multisystem inflammatory syndrome in children

“…With regard to distribution, some rashes were generalized while others were localized to the face, trunk, extremities, or acral regions. Palm and sole involvement including edema or erythema were present in some patients 30 , 32 , 41 , 46 , 47 while others had desquamation of the extremities and/or digits. 24 , 30 , 42 , 51 Conjunctivitis and cheilitis were described in many patients.…”

“…We identified a total of 34 case series 3 , 6 , 7 , 8 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 and case reports 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 published between May and July 2020 that mention dermatologic findings in 736 unique children with MIS-C. Cutaneous manifestations were present in 417 of 736 patients (57%). Fifteen (44%) of these articles state “rash” as the sole descriptor of skin findings.…”

“…Some smaller case series and case reports provide more detailed characterizations: polymorphic, maculopapular, morbilliform, and diffuse erythroderma were the most common morphologies noted. 3 , 25 , 34 , 38 , 39 , 40 , 45 , 46 , 48 , 51 Skin lesions in single case reports were described as urticarial 34 , reticular 47 , petechial 32 , and purpuric. 46 …”

“… 52 This is supported by the fact that many children do not display typical preceding symptoms of COVID-19 infection prior to developing MIS-C. 53 Further, many patients test positive for anti-SARS-CoV-2 antibodies at the time of MIS-C diagnosis, but lack polymerase chain reaction (PCR) positivity for the virus. 32 , 53 Others speculate MIS-C is a result of the known ability of SARS-CoV-2 to block type I and type III interferon responses, resulting in unrestrained viral proliferation and high viral load. 54 …”

Covid-19 : aspects cliniques et principaux éléments de prise en charge

“…Des cas de myocardite avec défaillance circulatoire ainsi qu’une recrudescence de cas de maladie de Kawasaki atypiques sans défaillance cardiaque ont fait l’objet d’alertes à partir de fin avril [ 19 ]. Ces enfants présentent un tableau fébrile avec des éléments cliniques de la maladie de Kawasaki.…”