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Pernio/Chilblains

Treatment

First-line therapy: The main treatment involves getting the affected areas warm, and keeping them warm. Wet, constrictive clothing should be removed immediately. The affected skin should be kept warm and dry, with loose, layered clothing, and the subject should stay in a warm environment. The best way to maintain distal extremity warmth is to keep the core warm as well, and it is essential that patients wear warm, layered clothing proximally as well as distally.

First steps

  • Warm clothes
  • Avoid cold, damp environments

Ancillary steps

  • Calcium channel blockers, particularly nifedipine, may alleviate lesions and prevent recurrence.
  • There are reports of ultraviolet light phototherapy leading to decreased numbers of and severity of lesions, particularly when instituted prophylactically at the beginning of the cool season.

Subsequent steps

  • True idiopathic chilblain (pernio) is a self-limiting disease wherein lesions should all resolve completely, although in some cases that may take weeks to months.
  • Individual symptomatic lesions may benefit from topical over-the-counter antipruritic agents or weak anti-inflammatory agents, but there exists little evidence to support their use.
  • Persistent lesions should prompt an evaluation for systemic diseases, particularly chilblains lupus erythematosus or chronic myelomonocytic leukemia. The presence of thromboses should prompt consideration of thrombotic disorders including cryoglobulinemia or anti-phospholipid antibody syndrome.

Pitfalls

  • Chilblain lupus erythematosus may present with lesions identical to idiopathic chilblain (pernio). Patients with lesions that fail to resolve with warming or that persist for months, or patients with arthritis/arthralgia or other systemic symptoms should undergo a thorough review of systems and targeted laboratory workup.
  • The presence of retiform purpura or nonblanching purpuric lesions should prompt consideration for a thrombotic process such as cryoglobulinemia or anti-phospholipid antibody syndrome
  • Chronic myelomonocytic leukemia may present with lesions consistent with pernio on the extremities, including during blast crisis. The presence of other signs and symptoms suggestive of a hematologic disorder should prompt a systemic workup.