History: The patient was referred by his pediatrician with complaints of red, itchy lesions on his toes for two to three weeks. The pediatrician was concerned because some of the lesions looked vasculitic. The patient is in good general health. A non-smoker. He recollected as the interview progressed that he'd had similar but less severe problems in the past.
Physical Exam: Erythematous macules, some hemorrhagic in appearance on toes. One lesion slightly necrotic.
Photos:
Lab: CBC, ANA and other tests ordered at pediatric office
Pathology: none
Diagnosis: Chilblains (Lupus Pernio)
Reasons Presented and Questions: Similar patients are seen two or three times a year in my office. The typical case is a child or young adult with pruritic erythematous macules or papules on the toes or fingers. The lesions can look necrotic and sometimes ulcerate. The person is otherwise healthy. Pernio is most commonly seen in fall or winter, but I've seen it in spring, too. The lesions are worrisome in appearance to physician, parent, patient. The index case here was worried that "my toes will rot off." His mother was worried about "lupus." I reassured them that this is a common, self-limited problem. While nifedipine may help, it's not necessary. Wear warm socks. Give it time. There's a good review on emedicine.com. It says: "The direct cause of pernio is cold exposure. Chronic pernio may be secondary to various systemic diseases as follows:
Chronic myelomonocytic leukemia
Anorexia nervosa
Dysproteinemias
Macroglobulinemia
Cryoglobulinemia, cryofibrinogenemia, cold agglutinins
Antiphospholipid antibody syndrome
Raynaud disease"
But it's usually easy to differentiate which ones are worrisome and the vast majority will be simple chilblains. One may need to use some clinical judgment. Not rush to unnecessary testing. In my experience, and from what I've read, this occurs more when it is cold and damp in thin individuals. Not bone chilling cold, but the cold of early winter. When it is really cold, good socks and footwear may be more the norm and can protect a susceptible individual from chilblains.
11/29/07 -- Patient just seen:
These are the toes of a 70 yo man who has had chilblains for the past 30 winters. His toes are uncomfortable, itchy and occasionally painful. He's had no chronic illnesses and enjoys good health.
Here is an excellent full text reference: "Pernio in Children" from the journal, Pediatrics.
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