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Concomitant calciphylaxis and COVID-19 associated thrombotic retiform purpura.

ABSTRACT: While initial reports regarding coronavirus disease 2019 (COVID-19) focused on its pulmonary manifestations, more recent literature describes multisystem abnormalities related to its associated microvascular angiopathy. Calciphylaxis is a rare systemic condition characterized by tissue necrosis in the setting of systemic microvascular calcifications. Both COVID-19 and calciphylaxis are procoagulant diagnoses associated with vascular-mediated cutaneous findings. To our knowledge, this is the first report to document the coexistence of COVID-19 associated retiform thrombotic purpura and calciphylaxis in a single patient, to link the pathologic etiologies of the two entities, and to describe the concomitant diagnoses' associated radiologic findings.

SUBMITTER: Rotman JA

PROVIDER: S-EPMC7447529 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Concomitant calciphylaxis and COVID-19 associated thrombotic retiform purpura.

Rotman Jessica A JA Dean Kathryn E KE Magro Cynthia C Nuovo Gerard G Bartolotta Roger J RJ

Skeletal radiology 20200826 11

While initial reports regarding coronavirus disease 2019 (COVID-19) focused on its pulmonary manifestations, more recent literature describes multisystem abnormalities related to its associated microvascular angiopathy. Calciphylaxis is a rare systemic condition characterized by tissue necrosis in the setting of systemic microvascular calcifications. Both COVID-19 and calciphylaxis are procoagulant diagnoses associated with vascular-mediated cutaneous findings. To our knowledge, this is the firs ...[more]

PMID: 32844243

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Project description:BackgroundThere are two distinctive acral manifestations of COVID-19 embodying disparate clinical phenotypes. One is perniosis occurring in mildly symptomatic patients, typically children and young adults; the second is the thrombotic retiform purpura of critically ill adults with COVID-19.ObjectivesTo compare the clinical and pathological profiles of these two different cutaneous manifestations of COVID-19.MethodsWe compared the light microscopic, phenotypic, cytokine and SARS-CoV-2 protein and RNA profiles of COVID-19-associated perniosis with that of thrombotic retiform purpura in critical patients with COVID-19.ResultsBiopsies of COVID-19-associated perniosis exhibited vasocentric and eccrinotropic T-cell- and monocyte-derived CD11c+ , CD14+ and CD123+ dendritic cell infiltrates. Both COVID-associated and idiopathic perniosis showed striking expression of the type I interferon-inducible myxovirus resistance protein A (MXA), an established marker for type I interferon signalling in tissue. SARS-CoV-2 RNA, interleukin-6 and caspase 3 were minimally expressed and confined to mononuclear inflammatory cells. The biopsies from livedo/retiform purpura showed pauci-inflammatory vascular thrombosis without any MXA decoration. Blood vessels exhibited extensive complement deposition with endothelial cell localization of SARS-CoV-2 protein, interleukin-6 and caspase 3; SARS-CoV-2 RNA was not seen.ConclusionsCOVID-19-associated perniosis represents a virally triggered exaggerated immune reaction with significant type I interferon signaling. This is important to SARS-CoV-2 eradication and has implications in regards to a more generalized highly inflammatory response. We hypothesize that in the thrombotic retiform purpura of critically ill patients with COVID-19, the vascular thrombosis in the skin and other organ systems is associated with a minimal interferon response. This allows excessive viral replication with release of viral proteins that localize to extrapulmonary endothelium and trigger extensive complement activation.

Dataset Information

Concomitant calciphylaxis and COVID-19 associated thrombotic retiform purpura.

Publications

Concomitant calciphylaxis and COVID-19 associated thrombotic retiform purpura.

Similar Datasets

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