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Alternative term, perniosis.
An idiopathic disorder characterized by red-purple papules, patches, or plaques that develop on the extremities in non-freezing cold or damp conditions.
A cold Induced inflammatory disorder that commonly affects acral sites and is frequently accompanied by a sensation of itching burning or pain.
Categorized as primary or secondary.
The primary process has been linked to abnormal neurovascular responses of dermal blood vessels in reaction to cold.
Affected patients develop vasoconstriction instead of protective vasodilatory responses in acral sites.
Diagnostic criteria include major criterion of localized erythema and swelling in acral sites that persists for greater than 24 hours.
In addition, one of the following minor criteria: on or worsening in cooler months, histopathologic findings of skin biopsy consistent with chilblains, without findings of lupus erythematosus, and response to conservative treatment of warming and drying.
Secondary perniosis is linked to several systemic processes including: cryoglobulinemia, auto immune connective tissue disease, leukemia, hyperviscosity syndrome, anti-phospholipid syndrome, and Covid-19.
Most well established to be associated with lupus erythematosus.
Lesions are more commonly seen on the feet than the hands.
Women are disproportionately affected.
Lesions usually are painful, but can burn or itch.
Associated with BMI less than 18 kg/m2.
Digital swelling can occur.
The most effective treatment is to keep the affected area warm and dry.
Primary perniosis is it self limited condition managed by the above recommendations of warm and dry skin, and maintaining body core temperature and avoid smoking.
Nifedipine can be used to reduce pain and facilitate healing.
Pentoxifylline may be considered.
If symptoms are severe or occur in warm months, chilblains lupus diagnosis is considered.
Consideration should also be given to an thromboembolic process, underlying autoimmune disease, or vasculitis.
