What causes perniosis?

Perniosis is thought to be caused by an abnormal response of the blood vessels to cold temperatures. In some instances, perniosis can occur in an individual for a long time and/or may be recurring. In these cases, the condition could be related to an underlying disease including:

Last updated on 05-01-20

How is perniosis and Raynaud phenomenon distinguished?

There are no specific laboratory tests used to distinguish perniosis and Raynaud phenomenon. The conditions are diagnosed by their signs and symptoms.

While both perniosis and Raynaud phenomenon are triggered by cold, perniosis causes red or purple bumps on the skin that can be itchy and tender. Severe cases can cause blistering, scabs, pustules, and ulcers. Affected skin may thicken as it heals.

Raynaud phenomenon does not cause bumps or thickened skin. In people with Raynaud phenomenon, the skin often becomes numb and paper white when exposed to the cold. After warming, the skin may turn blueish or red before returning to its normal color. Raynaud phenomenon tends to last for minutes, while perniosis-related skin bumps can last for weeks.

If perniosis is suspected, but the symptoms are unusual, biopsy may be considered. A biopsy can help rule out other causes of the skin findings. It is possible to have both perniosis and Raynaud phenomenon. In addition, both perniosis and Raynaud phenomenon can occur in association with other underlying conditions (such as an autoimmune disorder, blood disorder, or cancer). If another condition is suspected (due to the presence of additional signs and symptoms), testing should be offered to to rule out this possibility.

Last updated on 05-01-20

How might perniosis be treated?

The main method of treatment for perniosis focuses on avoiding exposure to cold. Individuals with this condition may be advised to keep the affected areas of their body warm by wearing insulated clothing, gloves, and footwear. The use of ultraviolet light at the beginning of cold, damp season may help prevent outbreaks. It is also suggested that people with this condition do not use cigarettes or nicotine. The use of certain medications, such as topical steroids and nifedipine, a calcium channel blocker, have been effective in treating some individuals.

Last updated on 05-01-20

Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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