Chilblains (Pernio): A Comprehensive Overview

Introduction

Chilblains, also known as pernio or perniosis, is a vascular disorder characterised by the development of erythematous, pruritic, and sometimes painful lesions on acral sites, most commonly the fingers, toes, ears, and nose. These lesions typically arise after exposure to cold but non-freezing temperatures and are the result of an abnormal vascular response. Although often considered a benign and self-limiting condition, chilblains can cause significant discomfort and, in some cases, lead to complications.

Historically, chilblains have been recognised for centuries, with early descriptions found in European medical texts. Despite advances in medical science, the precise mechanisms underlying chilblains remain incompletely understood, and the condition continues to be a topic of clinical and research interest.

Etiology and Pathophysiology

Underlying Mechanisms

Chilblains result from an abnormal vascular response to cold exposure. Upon exposure to cold, normal physiological vasoconstriction occurs to preserve core body temperature. However, in susceptible individuals, there is an exaggerated or prolonged vasoconstrictive response, followed by inadequate vasodilation upon rewarming. This sequence leads to localised hypoxia, endothelial damage, and subsequent inflammatory changes in the affected tissues.

Histopathological studies reveal perivascular lymphocytic infiltrates, oedema, and, in some cases, evidence of microthrombi. The pathogenesis also involves increased vascular permeability, leading to plasma leakage and the characteristic swelling and erythema.

Risk Factors

• Cold and Damp Environments: The primary trigger is exposure to cold (typically 2–15°C) and humid conditions, which accentuate the vascular response.
• Female Sex: Chilblains are more common in females, possibly due to hormonal influences on vascular reactivity.
• Low Body Mass Index (BMI): Individuals with lower subcutaneous fat are at higher risk due to reduced insulation.
• Genetic Predisposition: Familial cases suggest a genetic component, although specific genes remain unidentified.
• Underlying Medical Conditions: Connective tissue diseases (e.g., systemic lupus erythematosus, antiphospholipid syndrome), Raynaud phenomenon, and certain haematological disorders increase susceptibility.
• Smoking: Nicotine-induced vasoconstriction may exacerbate the risk.
• Poor Circulation: Conditions such as peripheral vascular disease can predispose individuals to chilblains.

Environmental Triggers

Sudden transitions from cold to warm environments, prolonged dampness, and inadequate protective clothing are common precipitating factors. In tropical countries, cases may be observed in hilly or high-altitude regions where temperatures drop significantly during winter months.

Epidemiology

Prevalence

The prevalence of chilblains varies widely, largely depending on climatic conditions. It is more frequently observed in regions with cold, damp winters and is relatively rare in consistently warm climates.

Demographics

Chilblains can affect individuals of any age but is most common in children, adolescents, and young adults. There is a notable female predominance, with reported female-to-male ratios ranging from 2:1 to 4:1. The condition is also more prevalent in people with low BMI and those with pre-existing autoimmune disorders.

Geographic Distribution

Chilblains are common in temperate and subarctic regions, including parts of Europe, North America, and the Indian subcontinent’s hill stations. In India, cases are reported from the Himalayan and north-eastern states during the winter season.

Clinical Features

Signs and Symptoms

• Lesions: Erythematous to violaceous macules, papules, or nodules, frequently on the toes, fingers, ears, or nose.
• Pruritus and Pain: The lesions are often intensely itchy, burning, or painful, especially when warming up after cold exposure.
• Swelling: Localised oedema may be present, sometimes with blistering.
• Ulceration: In severe or chronic cases, ulceration and secondary infection can occur.
• Colour Changes: The affected areas may appear blue or purple, reflecting underlying vascular changes.
• Duration: Lesions generally develop within 12–24 hours of cold exposure and resolve spontaneously within 2–3 weeks if further exposure is avoided.

Affected Populations

Chilblains can affect anyone exposed to cold and damp conditions, but certain groups are at higher risk, including young women, children, the elderly, and individuals with connective tissue diseases or poor peripheral circulation.

Differential Diagnosis

• Frostbite: Involves tissue freezing and necrosis, typically at lower temperatures.
• Raynaud Phenomenon: Characterised by episodic digital pallor and cyanosis, often without accompanying lesions.
• Erythromelalgia: Presents with redness and burning pain, usually triggered by heat rather than cold.
• Vasculitis: May present with similar lesions but is often accompanied by systemic symptoms and laboratory abnormalities.
• Acrocyanosis: Persistent cyanosis of the extremities, typically painless.
• Pernio-like Lesions in COVID-19: Also called “COVID toes,” these resemble chilblains but are associated with SARS-CoV-2 infection.

Diagnosis

Clinical Criteria

Diagnosis of chilblains is primarily clinical, based on a history of cold exposure, characteristic lesions, and exclusion of other causes. Key features include:

• Recent exposure to cold and damp conditions
• Development of erythematous or violaceous papules/nodules on acral sites
• Resolution upon avoidance of further cold exposure

Laboratory Tests

Laboratory investigations are usually not required for typical cases but may be indicated to rule out secondary causes or associated systemic diseases. Relevant tests include:

• Complete blood count (CBC)
• Antinuclear antibody (ANA) panel for autoimmune disorders
• Serum cryoglobulins
• Inflammatory markers (ESR, CRP)
• Skin biopsy (in atypical, persistent, or ulcerative cases)

Imaging

Imaging is rarely necessary but may be used to assess for underlying vascular pathology in atypical presentations or suspected systemic disease.

Differential Diagnosis (Revisited)

Careful assessment is needed to distinguish chilblains from other cold-induced or vascular conditions, as misdiagnosis can delay appropriate management.

Treatment

Pharmacological Options

• Topical Corticosteroids: May reduce inflammation and pruritus in mild to moderate cases.
• Calcium Channel Blockers (e.g., Nifedipine): Oral nifedipine has been shown to improve symptoms by promoting vasodilation. Doses typically range from 10 to 20 mg thrice daily, tailored to the patient’s tolerance.
• Vasodilators: Other vasodilatory agents, such as pentoxifylline, have been used in refractory cases.
• Topical Nitroglycerin: Occasionally used to enhance local blood flow.
• Analgesics: Nonsteroidal anti-inflammatory drugs (NSAIDs) for pain management if required.
• Antibiotics: Indicated only if secondary infection is suspected or confirmed.

Non-Pharmacological Approaches

• Warming Measures: Gradual rewarming of the affected areas, using dry heat (e.g., warm water immersion, heating pads). Sudden exposure to high heat should be avoided to prevent burns.
• Avoidance of Further Cold Exposure: Essential to prevent recurrence and promote healing.
• Protective Clothing: Use of warm gloves, socks, and insulated footwear in cold weather.
• Skin Care: Gentle skin care to prevent breakdown and secondary infection.
• Elevation and Rest: Useful for reducing swelling and discomfort.

Emerging Therapies

Recent research has explored the use of alternative vasodilators, immunomodulatory agents, and novel topical treatments. However, robust clinical trial data are limited, and these interventions remain investigational.

Prevention

Lifestyle Modifications

• Adequate Clothing: Wearing multiple layers of warm, moisture-wicking clothing, particularly for the extremities.
• Environmental Control: Avoiding prolonged exposure to cold and damp environments, and ensuring living spaces are adequately heated.
• Gradual Acclimatisation: Gradually increasing exposure to cold can help some individuals develop tolerance.
• Smoking Cessation: Quitting smoking reduces vasoconstrictive risk.
• Nutrition: Maintaining a healthy BMI through balanced nutrition may offer additional protection.

Protective Measures

• Use of insulated gloves, socks, and appropriate footwear
• Frequent movement and exercise to promote circulation
• Immediate drying and warming of wet or cold skin

Patient Education

Educating patients, especially those with recurrent chilblains or underlying medical conditions, is vital. Information should focus on recognising early symptoms, the importance of protective measures, and when to seek medical attention.

Complications

• Chronicity: Recurrent or persistent chilblains may lead to chronic skin changes, including hyperpigmentation, atrophy, and scarring.
• Secondary Infection: Ulcerated or fissured lesions are susceptible to bacterial infection, requiring prompt intervention.
• Functional Impairment: Severe pain or ulceration may limit daily activities, particularly in manual labourers or those reliant on fine motor skills.
• Quality of Life: Chronic or severe cases can negatively impact psychological well-being and social functioning.

Nursing Care of Patients with Chilblains (Pernio)

Effective nursing care is vital for symptom relief, prevention of complications, and patient education to minimize recurrence.

Nursing Assessment

Nursing assessment is the foundation of care for patients with chilblains. A systematic approach ensures recognition, differentiation from other conditions (like frostbite, Raynaud’s phenomenon, or vasculitis), and identification of risk factors. Key assessment steps include:

• Detailed subjective history: onset of symptoms, duration, exposure to cold, previous episodes, and associated medical conditions
• Physical examination: inspection for color, swelling, presence of blisters, erosions, or ulcers
• Assessment of functional impact: pain scale, interference with daily activities
• Evaluation of patient’s environment: housing, access to heating, occupation, and clothing habits
• Review of medication and comorbidities, particularly circulatory or autoimmune disorders

Planning of Nursing Care

The goals of nursing care in chilblains are:

• Alleviation of discomfort and promotion of skin healing
• Prevention of complications such as infection or ulceration
• Education and support to prevent recurrence
• Promotion of psychosocial well-being

Implementation: Nursing Interventions

1. Symptom Relief and Skin Care

• Rewarming: Advise gentle, gradual warming of the affected areas. Sudden or direct heat (such as hot water, heating pads) should be avoided, as it may exacerbate tissue damage.
• Skin Protection: Keep the area clean, dry, and covered with soft, non-constrictive dressings if necessary. Protect against further cold exposure.
• Topical Treatments: Apply soothing lotions (e.g., calamine) or topical corticosteroids for severe inflammation, as prescribed.
• Antihistamines: May be used for severe itching, under medical supervision.
• Pain Management: Use of analgesics for significant discomfort, as appropriate.

2. Prevention of Complications

• Infection Control: Monitor for signs of infection (increased redness, warmth, pus, or odor). Teach the patient to report such symptoms promptly. Administer antibiotics if prescribed for secondary infection.
• Ulcer Management: For ulcers or blisters, provide appropriate wound care, including sterile dressings, monitoring for delayed healing, and referral to wound care specialists if needed.

3. Patient Education

Education is central to long-term prevention of chilblains, empowering patients with the knowledge and skills to minimize recurrence. Key educational points include:

• Risk Avoidance: Advise on minimizing exposure to cold and damp environments. Emphasize the importance of adequate insulation, including warm socks, gloves, and layered clothing.
• Proper Rewarming: Teach safe methods for rewarming, stressing gradual warming and avoidance of direct heat sources.
• Foot and Hand Care: Encourage daily inspection for new lesions, especially in patients with diabetes or circulatory disorders; prompt reporting of changes is crucial.
• Environmental Adjustments: Encourage improvements at home or work, such as using heaters, dehumidifiers, and weather-appropriate attire.
• Hydration and Nutrition: Support a balanced diet and adequate hydration, as these contribute to skin integrity and overall health.

4. Addressing Psychosocial Needs

• Anxiety and Distress: Provide reassurance and emotional support. Address concerns related to appearance or limitations in activity.
• Social Support: Assist with connecting to community resources for heating, clothing, or housing needs if relevant.

5. Collaborative Care

• Work with physicians, dermatologists, and wound care nurses as needed for complex or non-healing cases.
• Refer to social workers or occupational therapists for additional support in vulnerable populations.

Evaluation of Nursing Care

Ongoing evaluation is necessary to determine the effectiveness of interventions and to adjust the care plan as needed. Evaluation parameters include:

• Resolution or improvement of skin lesions
• Reduction in pain, itching, and swelling
• Absence of infection or complications
• Patient’s ability to implement preventive strategies independently
• Improvement in quality of life and return to normal activities

Special Considerations

Vulnerable Populations

• Elderly: May have reduced mobility and impaired thermoregulation, requiring additional education and monitoring.
• Children: Require careful assessment and family education.
• Chronic Illness: Patients with diabetes, peripheral vascular disease, or autoimmune conditions require more intensive skin monitoring and education.

Pharmacological Support

• Vasodilators (e.g., nifedipine) may be prescribed in recurrent or severe cases to improve blood flow, under medical supervision.
• Topical steroids for inflammatory lesions, used judiciously and as prescribed.
• Antibiotics for secondary infection only if necessary.

Alternative and Adjunct Therapies

• Some patients may seek natural remedies like aloe vera or vitamin E for skin healing. Nurses should advise on evidence-based practices and caution against unproven treatments.

Documentation

Accurate and timely documentation is essential, including:

• Description of lesions and progression
• Interventions provided and patient response
• Educational topics covered and patient understanding
• Referrals and collaborative care notes

REFERENCES

1. DermNet (NZ). Chilblains. https://dermnetnz.org/topics/chilblains/. Last updated 2/2021.
2. Nyssen A, Benhadou F, Magnee M, Andre J, Koopmansch C, Wautrech JC. Chilblains.Vasa. 2020 Mar;49(2):133-140. doi:10.1024/0301-1526/a000838
3. Cappel JA, et al. Clinical characteristics, etiologic associations, laboratory findings, treatment, and proposal of diagnostic criteria of pernio (chilblains) in a series of 104 patients at Mayo Clinic, 2000 to 2011. Mayo Clinic Proceedings; 2014.
4. Dubey S, Joshi N, Stevenson O, Gordon C, Reynolds JA. Chilblains in immune-mediated inflammatory diseases: a review. Rheumatology (Oxford). 2022 Apr 12:keac231. doi:10.1093/rheumatology/keac231
5. Danzl DF. Nonfreezing tissue injuries. Merck Manual Professional Version. https://www.merckmanuals.com/professional/injuries-poisoning/cold-injury/nonfreezing-tissue-injuries#
6. National Health Service (UK). Chilblains. https://www.nhs.uk/conditions/chilblains/. Last reviewed 7/29/2022
7. Whitman PA, Crane JS. Perni.. https://www.ncbi.nlm.nih.gov/books/NBK549842/. 2023 Aug 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan.
8. Goldman L, et al., eds. Other peripheral arterial diseases. In: Goldman-Cecil Medicine. 26th ed. Elsevier; 2020. https://www.clinicalkey.com.

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