Management of Chilblains (Pernio)
For idiopathic chilblains, begin with cold avoidance and protective measures; when pharmacologic therapy is needed, oral nifedipine is the first-line treatment with moderate evidence supporting its efficacy in severe or refractory cases. 1
Initial Assessment and Diagnosis
- Confirm the diagnosis clinically by identifying erythematous, purplish, edematous lesions on acral sites (fingers, toes) that develop 12-24 hours after cold exposure, accompanied by pain, itching, or burning 2
- Screen for secondary causes including systemic lupus erythematosus, antiphospholipid antibodies, cryoproteins, and anorexia nervosa, particularly in thin patients (BMI <25th percentile) 2
- Obtain antinuclear antibody profile to exclude underlying autoimmune disease 2
- Consider skin biopsy only in dubious cases to confirm dermal edema and inflammatory infiltrate, particularly around eccrine glands 3
Non-Pharmacologic Management (First-Line)
- Advise strict cold avoidance and keeping extremities warm, as prevention is the best therapy 3, 2
- Recommend smoking cessation if applicable 3
- Ensure proper rewarming of affected limbs by cleaning, drying, and gradual warming (not rapid reheating) 2
- Counsel on appropriate clothing including avoiding sandals in winter and wearing protective footwear 2
- Reassure patients that acute pernio is usually self-limited and resolves spontaneously with warmer weather, typically by late spring 4
Pharmacologic Treatment
First-Line: Nifedipine
- Prescribe oral nifedipine (calcium channel blocker) for severe or refractory cases, as it has moderate evidence for reducing pain, facilitating healing, and preventing new lesions through vasodilation 2, 1
- Note that effectiveness is not confirmed by all studies, but it remains the most evaluated treatment 3
Second-Line: Pentoxifylline
- Consider pentoxifylline as an alternative with moderate evidence for severe or refractory cases 1
Topical Therapies
- Topical betamethasone is commonly used but lacks confirmation from randomized clinical trials 3
- Topical glyceryl trinitrate (GTN) has shown positive effects in limited patient numbers 3
- Topical minoxidil has been studied but with inadequate evidence 1
Other Options with Limited Evidence
- Hydrochloroquine has shown benefit only in a reduced number of patients 3
- Diltiazem, corticosteroids, and vitamin D have inadequate evidence or nonsignificant results compared to placebo 1
- Tadalafil has been studied but lacks strong supporting evidence 1
Management of Complications
- Treat secondary bacterial infection with appropriate antibiotics if ulceration occurs; hospitalization may be required for severe infections 4
- Manage thickening of small joints where lesions involve these areas with symptomatic treatment 4
- Use NSAIDs or prednisolone for symptomatic relief in acute exacerbations 4
Important Clinical Pitfalls
- Do not confuse with Raynaud's phenomenon, which presents with sharply demarcated pallor and cyanosis of shorter duration (hours rather than days) 2
- Distinguish from frostbite, which involves actual tissue freezing and necrosis 2
- Avoid excessive investigation in systemically well patients with typical presentation, as chilblains are almost always benign 4
- Recognize that elderly, females, outdoor workers, and those with chronic/recurrent episodes are less tolerant to cold and develop disease under lesser ambient cold conditions 5
Weather-Related Risk Factors
- Susceptibility increases when ambient temperature is <10°C and relative humidity is >60% 5
- Cold, damp conditions (not just dry cold) predispose to chilblain development 3, 5
- Clusters may occur during unusually cold winters, suggesting chilblains may be more common than previously recognized 4