What is the treatment for perniosis?

Treatment of Perniosis (Chilblains)

Nifedipine is the first-line pharmacologic treatment for perniosis, as it has been demonstrated to be effective in reducing pain, facilitating healing, and preventing new lesions through vasodilation. 1, 2

Initial Management Approach

Prevention and Non-Pharmacologic Measures

• Avoidance of cold, damp environments is the cornerstone of management, as perniosis develops among susceptible individuals exposed to nonfreezing cold temperatures 1, 2
• Protective measures including warm socks and gloves should be used consistently to prevent cold exposure after an initial insult 1, 2
• The involved limb should be cleaned, dried, and gradually rewarmed when acute lesions develop 1
• Fashion choices that expose acral skin (such as wearing sandals in winter) must be addressed, as inappropriate cold exposure is a modifiable risk factor 1

Patient Assessment Priorities

• Evaluate for underlying predisposing conditions including systemic lupus erythematosus, antiphospholipid antibodies (in adults), anorexia nervosa, cryoproteins, and thin body habitus (BMI <25th percentile), as these increase susceptibility 1
• Laboratory evaluation should include antinuclear antibody profile to exclude lupus-associated chilblain lupus erythematosus, though most primary perniosis cases will have negative results 1
• Distinguish perniosis from Raynaud's phenomenon (which has sharply demarcated pallor/cyanosis lasting hours rather than days) and frostbite (which involves tissue freezing and necrosis) 1

Pharmacologic Treatment Options

First-Line: Nifedipine

• Nifedipine produces vasodilation and has demonstrated effectiveness in reducing pain, facilitating healing, and preventing new lesions 1, 2
• This calcium channel blocker should be considered the standard of care for perniosis treatment 2
• Complete relief of symptoms can occur within 3 weeks of nifedipine therapy 2

Alternative Vasodilator: Topical Nitroglycerin

• Topical 0.2% nitroglycerin ointment is a promising alternative for patients who cannot tolerate or prefer topical therapy 3
• Regression of lesions typically occurs within the first week, with complete regression in 2 weeks in most patients (18 of 22 in one series) 3
• Response may be delayed in patients with longer disease duration, requiring up to 3 weeks for complete resolution 3
• Relapse can be successfully treated with another course of topical nitroglycerin 3

Second-Line: Pentoxifylline

• Oral pentoxifylline 400 mg three times daily for 3 weeks is an effective and safe alternative treatment option 4
• This agent demonstrated significantly better therapeutic response compared to placebo in a randomized controlled trial (p<0.0001) 4
• No side effects were encountered in the treatment group, making it a well-tolerated option 4

Refractory Cases

• Botulinum toxin injections may be considered for severe, ulcerative perniosis or chilblain lupus erythematosus that is refractory to standard treatments 5
• This represents a therapeutic option when conventional vasodilators fail 5

Clinical Pearls and Pitfalls

Disease Characteristics to Recognize

• Acute perniosis develops 12-24 hours after cold exposure with single or multiple erythematous, purplish, edematous lesions accompanied by intense pain, itching, or burning 1
• Chronic perniosis occurs with repeated cold exposure and persistence of lesions 1
• Lesions typically begin in fall/winter and disappear in spring/early summer among susceptible individuals 1
• Thin body habitus may be associated with increased cutaneous vasoreactivity, making these patients particularly vulnerable 1

Prognosis

• The prognosis for properly treated perniosis is excellent when prevention strategies are combined with appropriate pharmacotherapy 1
• Acute lesions are usually self-limited but may lead to recurrent disease without adequate preventive measures 1