Chilblains (Pernio): A mild form of cold injury caused by a localized inflammatory reaction involving small blood vessels, nerves, and skin after exposure to cold and damp conditions.
Pathophysiology
Vascular Response: Exposure to cold causes constriction of small arteries and arterioles followed by a delayed and exaggerated re-warming (reactive hyperemia), which causes leakage from capillaries and subsequent inflammation.
Tissue Damage: This inflammatory response can lead to the formation of red to purple bumps, which are painful and/or itchy.
Epidemiology
Demographics: More prevalent among young to middle-aged adults, predominantly females. Less common in children and the elderly.
Geographical Influence: Common in humid, temperate climates during colder seasons (late winter and early spring). Less frequent in extremely cold, dry climates due to better personal protection and adaptation.
Risk Factors
Individual Susceptibility: Includes poor peripheral circulation, a history of frostbite, and hormonal changes.
Environmental: Living or working in damp, cold environments without adequate heating or clothing.
Medical Conditions:
Connective Tissue Diseases: Especially systemic lupus erythematosus, which can manifest as chilblain lupus.
Hematologic Disorders: Like chronic myelomonocytic leukemia.
Vascular Disorders: Such as Raynaud’s phenomenon or acrocyanosis.
Lifestyle: Smoking, low body mass index, and poor nutritional status can predispose individuals to chilblains.
Occupational Hazards: Outdoor workers like fishermen and farmers are at increased risk.
Clinical Manifestations
Common Sites: Fingers, toes, ears, nose, and cheeks.
Symptoms: Initial redness and itching, progressing to painful inflammation and swelling. Lesions can become painful and may burn or itch severely.
Appearance: Lesions typically appear as small, red, swollen patches and can develop into blisters or ulcers in severe cases.
Association with COVID-19
COVID-Toes: Reports during the pandemic indicated a surge in chilblain-like lesions in patients with mild or asymptomatic COVID-19, possibly due to immune reactions affecting the blood vessels.
Diagnosis
Clinical Evaluation: Diagnosis is primarily based on the appearance of the skin and a history of exposure to cold.
Dermoscopy: Limited use but can help in excluding other differential diagnoses.
Biopsy: Not routinely performed but can confirm the diagnosis showing features of superficial and deep perivascular lymphocytic infiltration and edema.
Mayo Clinic Criteria: Includes persistent local redness and swelling, seasonal worsening, and improvement with warmth.
Management
Prevention: Key to management, involving protection against cold, wearing warm clothes, and improving home heating.
Topical Treatments: Application of topical corticosteroids like betamethasone valerate to reduce inflammation and creams like nitroglycerin to improve circulation.
Systemic Treatments: Nifedipine to reduce the frequency and severity of episodes by dilating small blood vessels and improving blood flow; pentoxifylline for its rheologic benefits.
Lifestyle Modifications: Cessation of smoking and addressing nutritional deficiencies.
Complications and Prognosis
Complications: Severe chilblains can lead to skin breakdown, ulceration, and secondary infections.
Prognosis: Generally good as chilblains often resolve spontaneously within 1-3 weeks, though they can recur in colder seasons. Chronic or recurring chilblains may require ongoing management.
Differential Diagnoses
Frostbite: More severe cold injury that requires different management.
Chilblain Lupus Erythematosus: Part of the lupus spectrum and needs autoimmune management.
Vasculitis and Other Perniosis: Need consideration of systemic involvement.