Management of Chilblains (Perniosis)
For patients presenting with chilblains, the primary treatment approach is prevention through cold avoidance and warming measures, with nifedipine as the most effective pharmacologic intervention for reducing pain, facilitating healing, and preventing new lesions. 1
Initial Assessment and Diagnosis
When evaluating a patient with suspected chilblains, focus on:
- Clinical presentation: Single or multiple erythematous, purplish, edematous lesions on acral sites (toes, fingers) accompanied by intense pain, itching, or burning that develop 12-24 hours after cold exposure 1
- Timing: Lesions typically begin in fall/winter and resolve in spring/early summer 1
- Physical findings: Papular lesions with signs of peripheral vasoconstriction causing acrocyanosis, possible thickening of small joints where lesions are present, and occasional ulceration 2
- Risk factors: Thin body habitus (BMI <25th percentile), cold exposure, outdoor activities in cold weather 1, 3
Distinguish from mimics: Raynaud's phenomenon presents with sharply demarcated pallor and cyanosis of shorter duration (hours vs days), while frostbite involves actual tissue freezing and necrosis 1
Laboratory Evaluation
For most patients with typical presentation, minimal investigation is needed 2:
- Antinuclear antibody (ANA) profile to exclude secondary causes (systemic lupus erythematosus, connective tissue disease) 1, 4
- Consider additional testing only if clinical features suggest underlying disease: cryoproteins, cryoglobulinemia, antiphospholipid antibodies, or monoclonal gammopathy 1, 4
Most cases are idiopathic and benign, requiring no extensive workup 2
First-Line Treatment: Non-Pharmacologic Measures
Prevention is the most important therapeutic strategy 1, 5:
- Immediate management: Clean and dry the affected limb, allow gradual rewarming 1, 5
- Cold protection: Minimize cold exposure after initial insult, wear appropriate protective clothing including adequate footwear (avoid sandals in winter) 1, 2
- Environmental modifications: Avoid exposure to temperatures <10°C and relative humidity >60%, which significantly increase susceptibility 3
- Symptomatic relief: Most patients improve spontaneously with warmer weather or respond to cold protection advice alone 2
Pharmacologic Treatment
When symptoms are significant or lesions persist despite conservative measures:
Nifedipine (First-Line Pharmacologic Agent)
- Mechanism: Produces vasodilation 1
- Efficacy: Demonstrated effectiveness in reducing pain, facilitating healing, and preventing new lesions 1
- Indication: Consider for patients with persistent symptoms, recurrent disease, or significant functional impairment 1
Alternative Pharmacologic Options
- NSAIDs: For symptomatic pain relief 2
- Prednisolone: May be used for severe inflammatory cases 2
- Topical corticosteroids: High-potency preparations (clobetasol propionate 0.05%) can be considered for localized inflammatory lesions, though not specifically studied for chilblains 6
Special Populations and Considerations
High-risk groups requiring closer monitoring 3:
- Elderly patients
- Females
- Outdoor workers
- Patients with chronic or recurrent episodes
- Thin individuals with low BMI 1
These populations are less tolerant to cold weather and develop disease under lesser ambient cold exposure 3
Management of Complications
- Secondary bacterial infection: Requires hospitalization and systemic antibiotics if severe 2
- Ulceration: Maintain wound care principles, ensure adequate protection from further cold exposure 2
- Chronic/recurrent disease: Occurs with repeated cold exposure; requires aggressive prevention strategies and may benefit from prophylactic nifedipine 1
Expected Course and Follow-Up
- Acute pernio: Usually self-limited, resolving within days to weeks with appropriate management 1
- Seasonal resolution: Most cases completely resolve by late spring (November in Southern Hemisphere) 2
- Prognosis: Excellent for properly treated pernio 1
- Recurrence risk: Patients remain susceptible to future episodes with cold re-exposure 1
Common Pitfalls to Avoid
- Over-investigation: Extensive laboratory workup is unnecessary for typical presentations in systemically well patients 2
- Misdiagnosis: Failing to distinguish from vasculitis, cutaneous leukemia, or connective tissue disease in atypical cases 4
- Inadequate prevention counseling: Not emphasizing cold avoidance as primary prevention leads to recurrent episodes 1
- Delayed recognition: Unfamiliarity with presentation in warmer climates may lead to unnecessary anxiety and testing 4