Vasospasm Is a Consistent Finding in Pernio (Chilblains) and a Possible Clue to Pathogenesis

Shahi, Varun; Wetter, David A.; Cappel, Jonathan A.; Davis, Mark D. P.; Spittell, Peter C.

doi:10.1159/000437224

Cited by 23 publications

(20 citation statements)

References 9 publications

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“…Persistent or prolonged cold-induced vasospasm has been proposed to lead to hypoxemia and a subsequent secondary inflammatory response. 9,25 Also, neurovascular instability with inappropriate neural responses to temperature has been suggested for chilblains, 26 and neuropathy has been reported in patients with COVID-19. 27 The pathophysiology of acute chilblain in COVID-19 is unknown, and cold is not a trigger in our cases.…”

Section: Discussionmentioning

confidence: 99%

Chilblains in children in the setting of COVID‐19 pandemic

Andina

Noguera‐Morel

Bascuas-Arribas

et al. 2020

Pediatric Dermatology

191

328

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BackgroundDifferent skin manifestations of COVID‐19 are being reported. Acral lesions on the hands and feet, closely resembling chilblains, have been recognized during the peak incidence of the COVID‐19 pandemic.Material and methodsA retrospective review of 22 children and adolescents with chilblain‐like lesions seen over a short period of time in the Emergency Department of a children's hospital during the peak incidence of COVID‐19 in Madrid, Spain.ResultsAll patients had lesions clinically consistent with chilblains of the toes or feet, with three also having lesions of the fingers. Pruritus and mild pain were the only skin symptoms elicited, and only 10 had mild respiratory and/or GI symptoms. None had fever. Coagulation tests, hemogram, serum chemistry, and lupus anticoagulant were normal in all patients tested. One out of 16 tested cases had elevated D‐dimer results, but without systemic symptoms or other laboratory anomalies. SARS‐CoV‐2 PCR tested in 19 cases was positive in just one case. Skin biopsies obtained in six patients were consistent with chilblains. On follow‐up, all cases showed spontaneous marked improvement or complete healing.ConclusionAcute chilblains were observed during COVID‐19 pandemic in children and teenagers. It is a mildly symptomatic condition with an excellent prognosis, usually requiring no therapy. Etiopathogenesis remains unknown.

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Section: Discussionmentioning

confidence: 99%

Chilblains in children in the setting of COVID‐19 pandemic

Andina

Noguera‐Morel

Bascuas-Arribas

et al. 2020

Pediatric Dermatology

191

328

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“…The hypothesis is that patients with chilblains have persistent or prolonged cold induced vasospasm leading to hypoxemia, with a subsequent secondary inflammatory response. A recent study suggests that vasospasm plays a role in the physiopathology of pernio [34]. A neurovascular instability with inappropriate neural responses to temperature has been suggested [35].…”

Section: Physiopathologymentioning

confidence: 99%

Chilblains

Nyssen

Benhadou

Magnée

et al. 2020

Vasa

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Summary: Idiopathic chilblain is a relatively common yet poorly recognized acrosyndrome. This literature review aims to better understand and draw attention to this disorder. Chilblain is a localized inflammation of the skin that occurs on exposure to cold but non-freezing wet weather. It usually resolves spontaneously. The etiology is uncertain, but vasospasm seems to play a role in this abnormal reaction to cold. Diagnosis is most often based on clinical presentation, but a skin biopsy can be useful in dubious cases. In histology, dermal edema and an inflammatory infiltrate are usually present. A distribution of the infiltrate particularly around the eccrine gland is typical. Systemic symptoms and underlying autoimmune disease should be screened. Avoiding cold and keeping extremities warm is the first recommendation for management, as well as smoking cessation. Calcium channel blockers (in particular nifedipine) seems to be the treatment that has been most evaluated in chilblains. However, their effectiveness is not confirmed by all studies. Topical betamethasone is often used but its effect has not been confirmed by randomized clinical trials. Other treatments, such as pentoxifylline, hydrochloroquine and topical nitroglycerin have shown positive effects only in a reduced number of patients. Acupuncture seems to bring a benefit.

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“…Primary perniosis is typically a seasonal condition that presents initially with erythema and swelling of the digits upon exposure to cool, damp conditions followed by red-purple purpuric patches and papules on the dorsal digits. The changes are thought to result from vasoconstriction and vasospasm leading to anoxia and vascular damage 6. Peripheral skin changes including acrocyanosis and livedo reticularis are commonly observed.…”

mentioning

confidence: 99%

Clustered cases of acral perniosis: Clinical features, histopathology, and relationship to COVID‐19

Cordoro

Reynolds

Wattier

et al. 2020

Pediatric Dermatology

158

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Background/Objectives: There has been a recent marked increase in pediatric and adult patients presenting with purpuric acral lesions concerning for ischemia, thrombosis and necrosis in COVID-19 prevalent regions worldwide. The clinical and histopathological features and relationship to COVID-19 have not been well described. The objective of this case series is to describe the clinical features and determine the histopathologic findings and clinical implications of the clusters of acral perniosis cases identified in pediatric patients.Methods: We describe 6 otherwise healthy adolescents -3 siblings per family from 2 unrelated families -presented within a 48-hour period in April, 2020, with acral perniosis-like lesions in the context of over 30 similar patients who were evaluated within the same week. Accepted ArticleThis article is protected by copyright. All rights reserved Results: Affected patients had mild symptoms of viral upper respiratory infection (URI) or contact with symptomatic persons 1-2 weeks preceding the rash. They all presented with red to violaceous macules and dusky, purpuric plaques scattered on the mid and distal aspects of the toes. Skin biopsies performed on each of the 6 patients demonstrated near identical histopathologic findings to those of idiopathic perniosis, with a lymphocytic inflammatory infiltrate without evidence of thromboembolism or immune complex vasculitis. While SARS-CoV-2 polymerase chain reaction was negative, testing was performed 1-2 weeks after URI symptoms or sick contact exposure.Conclusion: We offer a clinical approach to evaluation of patients with this presentation and discuss the possibility that these skin findings represent a convalescent-phase cutaneous reaction to SARS-CoV-2 infection.

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Vasospasm Is a Consistent Finding in Pernio (Chilblains) and a Possible Clue to Pathogenesis

Cited by 23 publications

References 9 publications

Chilblains in children in the setting of COVID‐19 pandemic

Chilblains in children in the setting of COVID‐19 pandemic

Chilblains

Clustered cases of acral perniosis: Clinical features, histopathology, and relationship to COVID‐19

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