What are the management options for a patient with perniosis?

Management of Perniosis

For patients with perniosis, initiate oral nifedipine as first-line pharmacologic therapy combined with cold avoidance and protective measures, as this calcium channel blocker has demonstrated effectiveness in reducing pain, facilitating healing, and preventing new lesions. 1, 2

Initial Assessment and Diagnosis

• Confirm the diagnosis clinically by identifying characteristic erythematous, purplish, edematous lesions on acral sites (fingers, toes) that develop 12-24 hours after cold exposure and persist for days rather than hours 2
• Exclude underlying systemic conditions including systemic lupus erythematosus, antiphospholipid antibodies, cryoprotein disorders, and anorexia nervosa through appropriate laboratory evaluation (antinuclear antibody profile, complete metabolic panel) 2, 3
• Assess body habitus as thin patients (BMI <25th percentile) demonstrate increased susceptibility and cutaneous vasoreactivity 2

First-Line Management

Non-Pharmacologic Interventions

• Implement strict cold avoidance as prevention is the most effective therapy, with patients instructed to minimize cold exposure after initial insult 2
• Ensure proper protective clothing including warm gloves and socks, avoiding fashion choices that expose acral areas to cold (such as sandals in winter) 1, 2
• Clean and dry affected limbs with gradual rewarming to prevent tissue damage 2

Pharmacologic Treatment

• Prescribe oral nifedipine as the standard of care, which produces vasodilation and has been demonstrated effective in multiple case series 1, 2
• Consider topical 0.2% nitroglycerin ointment as an alternative vasodilator option, with studies showing regression of lesions within 1 week in 82% of patients (18/22) and complete resolution within 2 weeks 4

Second-Line Options for Refractory Cases

• Trial oral pentoxifylline 400 mg three times daily for 3 weeks based on randomized controlled trial evidence showing significantly better therapeutic response compared to placebo (p<0.0001) with no reported side effects 5
• Consider botulinum toxin injections for severe, ulcerative cases, particularly in chilblain lupus erythematosus that fails conventional therapy 3

Clinical Pearls and Pitfalls

Distinguish perniosis from Raynaud's phenomenon by duration: Raynaud's presents with sharply demarcated pallor and cyanosis lasting hours, while perniosis lesions persist for days with intense pain, itching, or burning 2

Differentiate from frostbite which involves actual tissue freezing and necrosis rather than the inflammatory response to non-freezing cold seen in perniosis 2

Monitor for chronic perniosis which develops with repeated cold exposure and persistence of lesions, requiring more aggressive preventive strategies 2

Expect seasonal variation as lesions typically begin in fall/winter and resolve in spring/early summer among susceptible individuals 2

Treatment Duration and Follow-up

• Continue nifedipine therapy throughout cold weather months with clinical reassessment at 3 weeks to confirm symptom relief 1
• For topical nitroglycerin, expect response within 1 week for most patients, though those with longer disease duration may require 2-3 weeks for complete regression 4
• Anticipate excellent prognosis with proper treatment, though recurrence may occur with subsequent cold exposure requiring repeat courses of therapy 2, 4