Perniosis or Chillibains
Also known as chilblains, this has been a very common problem during these past few winter months. Presentation can range from mild erythema all the way to severe blistering. Sensations experienced are also variable, including pain as well as itch. It is often not associated with a preceding history of Raynaud’s phenomenon.
Perniosis is due to prolonged cold injury. This explains the usual distribution affecting the toes and the dorsal surface of the fingers. Skin seems to be even more vulnerable to injury if it is also wet when exposed to cold.
Interestingly, patients will often not associate the cold as a cause, as their symptoms are often more apparent when the affected areas are rewarmed. So much so that they will take there feet out from under their blankets at night. (It is important however not to confuse this with erythromelalgia, which can be associated with a myelodysplastic disorder. In erythromelalgia, there is palmar or plantar erythema with a burning sensation which is almost instantly relieved when plunged into cold water.)
In chilblains, a biopsy of involved skin will demonstrate a lymphocytic vasculitis. Although a biopsy is not often necessary to establish a diagnosis, it can be helpful in the more difficult cases to exclude differential diagnoses such as a leukocytoclastic vasculitis, cholesterol thromboembolic vasculitis, disseminated intravascular coagulation, lupus, dermatomyositis, etc.
It is important to look for a possible underlying connective tissue disease such as lupus if there are any other associated symptoms, especially if the chilblains are more widespread (eg if there is involvement of the ears and nose as well) or more severe (eg if there is blistering or ulceration). Investigations looking for cryoglobulins should also be included.
Management is relatively straightforward. Two pairs (thin cotton inner layer, thicker outer layer) of gloves or socks to keep the hands and feet warm are essential. It is also important to keep the entire arms and legs warm. This is not always that easy, especially when this might impact on the patient’s ability to work. When the skin lesions are symptomatic, topical potent corticosteroids such as betamethasone dipropionate can be very helpful. If a patient is on a peripheral vasoconstrictor such as a beta-blocker for blood pressure control, it might be worthwhile to switch them over to a vasodilator such as a calcium channel blocker or an ACE inhibitor. Other options include applying glyceryl trinitrate to the wrists to promote improved blood flow.
Unfortunately, with repetitive cold injury, there is evidence to suggest that the skin becomes even more sensitive to further cold injury, and so it is extremely important to emphasize the importance of keeping the extremities warm.
