What is pernio (chillblains) and what is the recommended management?

Pernio (Chilblains): Definition and Management

Definition and Pathophysiology

Pernio (chilblains) is a localized inflammatory skin condition caused by an abnormal vascular response to non-freezing cold and damp exposure, distinct from frostbite which involves actual tissue freezing. 1, 2, 3

• Pernio develops in susceptible individuals exposed to cold, damp conditions without tissue freezing, typically appearing 12-24 hours after exposure 2, 4
• The pathophysiology involves vasospasm and complex vascular reactivity related to both patient susceptibility and environmental factors 4
• This condition must be distinguished from frostbite, which requires tissue freezing at temperatures causing actual necrosis and demands immediate rewarming to 37-40°C 1

Clinical Presentation

• Single or multiple erythematous-to-purplish, edematous lesions appear on acral sites (fingers, toes) accompanied by intense pain, itching, or burning 2, 3
• Acute pernio develops 12-24 hours post-exposure; chronic pernio occurs with repeated cold exposure and persistent lesions 2, 4
• Lesions typically begin in fall/winter and resolve in spring/early summer 2
• The condition predominantly affects young women and thin individuals (BMI <25th percentile) 2, 5

Differential Diagnosis

Key conditions to exclude:

• Raynaud's phenomenon: presents with sharply demarcated pallor and cyanosis lasting hours (not days), versus the persistent purplish lesions of pernio 2
• Frostbite: involves actual tissue freezing with resultant necrosis, requiring different management 1, 2
• Secondary causes: systemic lupus erythematosus, antiphospholipid antibodies, cryoproteins, connective tissue disease, monoclonal gammopathy, cryoglobulinemia, or chronic myelomonocytic leukemia 2, 3

Diagnostic Evaluation

• Clinical diagnosis based on typical lesion appearance during cold/damp season 3
• Laboratory workup to exclude secondary causes: antinuclear antibody profile, cryoglobulins, serum protein electrophoresis 2, 3
• Histopathology cannot distinguish primary from secondary forms but may exclude vasculitis or other mimics 3
• All patients require thorough history and physical examination to identify underlying connective tissue disease or other systemic conditions 3

Management Algorithm

First-Line Treatment (All Patients)

Prevention is the most effective therapy:

• Minimize cold exposure after initial insult 2, 6
• Protect acral parts with warm socks and gloves 6, 5
• Clean and dry the involved limb 2
• Gradual rewarming (NOT sudden warming, which worsens vasospasm) 5
• Avoid cold, damp environments 6

Pharmacologic Treatment

For symptomatic relief and prevention of recurrence, nifedipine should be considered the standard of care:

• Nifedipine (dihydropyridine calcium channel blocker) is effective in reducing pain, facilitating healing, and preventing new lesions 2, 6, 4
• Nifedipine promotes faster healing and prevents recurrence through vasodilation 4
• Complete symptom relief typically occurs within 3 weeks of nifedipine therapy 6

Adjunctive Topical Therapy

• Topical corticosteroid ointment may be used based on severity 5
• Antihistamines when indicated for pruritus 5

Prognosis and Follow-Up

• Acute pernio lesions are usually self-limited but may lead to recurrent disease 2
• Prognosis for properly treated pernio is excellent 2
• Potential sequelae include superinfection, depigmentation, and scarring 4
• Symptoms typically resolve within 2-3 weeks with appropriate treatment 3, 6

Clinical Pearls

• Thin body habitus may be associated with increased cutaneous vasoreactivity and higher risk 2
• Fashion choices (e.g., wearing sandals in winter) contribute to cold exposure risk 2
• The condition can occur even in warmer climates during winter months, so maintain clinical awareness year-round 3
• Peak presentation occurs during December-February (coldest months) 5