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Therapeutic Strategies

Pernio/Chilblains

Timothy Berger Bruce Wintroub

Saturday, January 01, 2011

Pernio

Chilblains is a recurrent, localized erythema and swelling of the hands, feet, ears, and face. The reaction is an abnormal reaction to cold that occurs most frequently in women, children, and the elderly. Lesions tend to become worse in the elderly, and improve spontaneously in younger patients. The therapeutic strategy is to correct the conditions that result in chronic cold exposure and to employ therapeutic agents that increase peripheral circulation.

First Steps

The calcium channel blocker nifedipine 10 mg three times daily or 20 mg twice daily is very effective in both increasing the rate of resolution of pernio lesions and in preventing their appearance. This efficacy is due to the vasodilatory effect of this medication. While mild symptoms of peripheral edema and hypotension may occur, these symptoms rarely require discontinuation of the medication when used at low doses. Monitor blood pressure at the start of treatment and at return visits.

Ancillary Steps

  1. Keeping both the affected extremities and the core body warm are essential in preventing pernio. Patients should wear thick socks and shoes. For perniosis of the hands, gloves are recommended. The ambient temperature must be kept warm. These environmental changes are most critical in preventing recurrences.
  2. Keep feet dry. Moisture enhances cold injury.

Alternative Steps

  1. Nicotinamide 500 mg three times daily may be useful alone or in addition to calcium channel blockers.
  2. Hexylnicotinate 2% cream applied three times daily may be useful for patients intolerant of or unwilling to take oral medications.
  3. Amlodipine 2.5-5 mg once daily may be used as an alternative calcium channel blocker. The long half-life and consequent once daily dosing of this drug is beneficial when chronic therapy is required.
  4. Erythematous doses of ultraviolet light to affected areas 2 to 3 times a week at the start of winter may be preventive.

Pitfalls

  1. Pernio-like lesions occur in both discoid and systemic lupus erythematosus, as well as in sarcoidosis. The possibility of lupus should be excluded by appropriate laboratory tests and biopsy.
  2. Cryoglobulinemia, cryofibrinogenemia and other hypercoagulable states should be considered, especially in atypical or refractory cases. Most cases of classic perniosis are not associated with such conditions but are environmentally triggered.
  3. Chilblains may be accompanied by other clinical manifestations of cold sensitivity, such as acrocyanosis and/or erythrocyanosis.

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