Neurovascular Instability Syndrome: A Unifying Term to Describe the Coexistence of Temperature-Related Vascular Disorders in Affected Patients

George, Rosalyn; Fulchiero, Gregory J.; Marks, James G.; Clarke, Jennie T.

doi:10.1001/archderm.143.2.274

Cited by 8 publications

(3 citation statements)

References 7 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

186

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

186

328

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“…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]. The occurrence of chilblain in situations such as anorexia or conditions causing weight loss such as bariatric surgery, suggests that thermoregulation plays a central role in this disorder.…”

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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“…[15] Nevertheless, a detailed history of cold exposure and cutaneous response can aid in distinguishing between acral vascular syndromes. Pathologic responses can be either idiopathic (primary) or secondary.…”

Section: Cold-induced and Cold-associated Conditionsmentioning

confidence: 99%

The Purple Digit

Brown

Zirwas

English

2010

American Journal of Clinical Dermatology

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The acute onset of purple digits is a concerning manifestation and may represent underlying, potentially life-threatening disease. Correctly identifying the etiology of purple digits is essential to proper management, and can aid in the diagnosis of systemic disease. Multiple causes of purple digits and significant overlap in clinical presentation can make diagnosis difficult. Despite the various causes of acute purple digits in the published literature, an algorithmic approach to the evaluation and management of the most common and alarming etiologies has yet to be established. The initial step in evaluating a patient with purple digits is to determine if the cause is associated with hypoxemia or trauma. If the patient is in a stable condition, the dermatologist needs to determine if the process could be related to cold exposure such as Raynaud phenomenon, acrocyanosis, pernio, cryoglobulinemia or frostbite. If the disease occurs independent of temperature, physical examination and histological evaluation of the skin is recommended. The lack of peripheral pulses are concerning for acute arterial thrombosis from peripheral vascular disease or arterial embolism. Non-blanching skin changes on the digit that lack inflammation and microthrombosis most likely represent a bleeding or platelet abnormality; however, if microthrombi are identified a more life-threatening processes such as purpura fulminans or embolic phenomenon may be occurring. Evidence of blood vessel inflammation suggests a leukocytoclastic vasculitis. The patient with a purple blanching digit and normal pulses requires an extensive historical review to help determine the cause. This review presents an algorithmic approach to assist in the evaluation and management of the purple digit.

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Neurovascular Instability Syndrome: A Unifying Term to Describe the Coexistence of Temperature-Related Vascular Disorders in Affected Patients

Cited by 8 publications

References 7 publications

Chilblains in children in the setting of COVID‐19 pandemic

Chilblains in children in the setting of COVID‐19 pandemic

Chilblains

The Purple Digit

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