key: cord-0793910-f4097ehc
authors: Fritz, Mike; Wessel, Alex W.; Tinker, Daniel; Morris, Gabriela M.; Goldenberg, Linda; Fesler, Mark; Rukmangadachar, Lokesh; Hurley, M. Yadira
title: SARS-CoV-2: A Potential Trigger of Dermato-Neuro Syndrome in a Patient with Scleromyxedema
date: 2021-11-06
journal: JAAD Case Rep
DOI: 10.1016/j.jdcr.2021.10.027
sha: eb2156ce223c70c1fcb56262d166221c72970c44
doc_id: 793910
cord_uid: f4097ehc

nan

Scleromyxedema (SMX) is characterized by excessive mucin deposition in connective tissues 26 and skin. 1 It shares clinical features with other mucinoses including generalized myxedema, pre-27 tibial myxedema, reticular erythematous mucinosis, and scleredema. 1 However, diagnosis of SMX 28 is distinguished by these criteria: absence of thyroid disease, presence of monoclonal gammopathy 29 (classically IgG λ), and cutaneous eruption of waxy papules and plaques, characterized 30 histopathologically by increased dermal mucin, fibroblast proliferation, and fibrosis. 2 SMX also 31 causes multisystem disease with approximately 20% fatality. 3 32

Dermato-neuro syndrome (DNS), a potentially fatal complication of SMX, occurs in 33 approximately 18% of patients. 4 It presents with fever and neurologic disturbances including 34 confusion, dysarthria, seizures, and coma. Pathogenesis of DNS remains uncertain; however, 35 reports speculate that elevated levels of vasoactive cytokines (e.g., interleukin-6) during viral 36 infection may contribute by compromising the blood-brain-barrier (BBB). 5,6 Enhanced BBB 37 permeability in the setting of monoclonal gammopathy may elevate IgG levels in the brain 38 microvasculature, causing hyperviscosity, sludging, and neurologic abnormalities. 7-9 The available 39 literature indicates that reported cases of DNS are frequently preceded by a flu-like prodrome 3,4,9-40 18 or other suspected upper respiratory tract infection. 8, 19 Notably, two case studies identified 41 influenza A infection in association with DNS. 6,10 Here, we describe one patient with SMX 42 complicated by 2 episodes of DNS, each associated with different RNA virus infections: influenza 43 A or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). 44

A 48-year-old man with a history of hypertension initially presented with 3 months of skin 46 tightening, difficulty clenching his fists or opening his mouth, and mild shortness of breath. Three years after the initial episode of DNS, the patient presented with loss of smell and 76 malaise 2 days prior to scheduled IVIG treatment. PE showed no progression of cutaneous disease 77 (Fig 2, 3) . PCR testing of a nasopharyngeal swab, however, confirmed SARS-CoV-2 infection, 78 precluding treatment at his regular outpatient infusion center. Consequently, he was admitted to 79 the hospital to receive his scheduled IVIG. During IVIG infusion, he developed altered mental 80 status, tonic-clonic movements, and fever to 39.4°C. EEG revealed status epilepticus. He was 81 transferred to the ICU, intubated, and treated with IV antiepileptics and broad-spectrum antibiotics. 82 CSF analysis revealed mild pleocytosis (7 WBC/L), mildly elevated protein (63 mg/dL), and an 83 opening pressure of 24 cm H20. CSF testing was negative for gram stain, cryptococcus antigen, 84 and HSV 1 and 2. CBC, CMP, UA, CXR, blood cultures, and brain CT/MRI were unremarkable. 85

Neurology was consulted and could not identify underlying cause of the precipitous neurologic 86 decline; thus, recurrent DNS was considered as the potential diagnosis. The patient was treated 87 with a 3-day course of IV methylprednisolone and IVIG (3 g/kg of IBW). forehead. Induration of the glabella, an early finding in progression to leonine facies, can also be 198 appreciated.

Dermato-neuro syndrome in a patient treated with autologous stem 177 cell transplant for scleromyxedema

Scleromyxedema: response to high-dose intravenous immunoglobulin 180 (hdIVIg)

Infection as an Environmental Trigger of Multiple Sclerosis Disease 182

Clinical evaluation of the BioFire® Respiratory Panel 2.1 and 185 detection of SARS-CoV-2