Are these lesions a sign of a serious underlying illness?

Dermclinic Case:
This adolescent girl presents with painful purple papules that have developed on her toes. These papules are making it impossible for her to wear her "fashion" shoes to school in the late fall and early winter. She reports that her feet have been cold for as long as she can remember and that she is not bothered by it. She is otherwise healthy, takes no medications, and does not smoke.

Are these lesions a sign of a serious underlying illness? Why does the problem seem to be noticeable in the late fall and early winter?

Answer on next page.

Dermclinic–Answer

Chilblains (pernio) refers to a "benign" inflammatory process that produces painful, burning violaceous papules on the toes. This condition occurs in the setting of "cold" toes, which may or may not be erythrocyanotic. The trigger is cold, wet, nonfreezing weather that adolescents don't acknowledge and for which they don't change their footwear for protection. The acute form is typically seen in adolescence as repeated episodes of painful inflammation that last 10 to 14 days.

Histologic evaluation of these papules shows epidermal pallor, a superficial and deep perivascular lymphocytic infiltrate that occurs around the eccrine coils, and a characteristic papillary dermal edema. Classic chilblains is not usually associated with any detectable cold-associated proteins. The main differential diagnoses are chilblains lupus and cold-sensitive blood dyscrasias. Bloodwork looking for evidence of autoimmunity and cold-sensitive dysproteinemias should be considered for patients whose presentation is atypical or prolonged.

The best therapy for chilblains is to keep the feet warm with footwear appropriate for the climate. It is not surprising that this condition is most commonly seen in young women during the "shoulder seasons," when it is still not cold enough to require full protection. The challenge for us is to convince the patient to choose footwear that is more appropriate than fashionable. I also ask affected patients to wear "house shoes" and, possibly, socks at night so that they keep their feet continuously warm.

Your treatment will fail if you cannot convince these adolescents to keep their feet warm. If they can do so, high-potency topical corticosteroids will reduce the inflammation and the pain. In recalcitrant cases, nifedipine has been effective. The lesions resolve without scarring but will recur with further exposure to the cold.