March 23, 2020
Years published: 1989, 1997, 2005, 2020
NORD gratefully acknowledges Amarachi Okafor, BS, PharmD Candidate, NORD Editorial Intern from the Keck Graduate Institute, and Julie Truong, PharmD, BCACP, MACM, Associate Professor of Clinical and Administrative Sciences at Keck Graduate Institute School of Pharmacy and Health Sciences, for assistance in the preparation of this report.
Perniosis is a seasonal inflammatory disorder that is triggered by prolonged exposure to cold and damp (humid) conditions. It usually occurs when the weather is cold and the humidity is high, especially during late fall and winter. It is a form of inflammation of the small blood vessels (vasculitis) that is characterized by painful, itchy, tender, skin injuries (lesions) on the lower legs, hands, toes, feet, ears and face. The lesions typically appear 12-24 hours after exposure to cold and usually last for two to three weeks. It may last for years if left untreated and cold exposure persists. Some individuals experience complete or partial resolution in the summer months, but in some individuals, symptoms may persist even into the warmer months.
Perniosis is characterized by inflammation of the small blood vessels caused by an abnormal reaction to the cold. It is characterized by a bluish-red discoloration of the skin that can cause pain, intense itching, burning/stinging, and swelling of the skin, especially as the body becomes warmer. The discoloration usually occurs on the fingers, toes, lower legs, heels, ears and nose; rarely it can appear on the thighs and buttocks. In severely affected individuals, there may be blister-like lesions (bullae) which may become ulcers if rubbed or irritated. This may result in infections or even scarring upon healing.
Perniosis of the thighs is a form of perniosis that more commonly affects young females who wear tight fitting pants. It is characterized by red or bluish patches (plaques) on the skin. These plaques are distributed on the upper hip region and on the outside of the thighs and can cause swelling, burning, itching and occasionally ulceration.
The exact cause of perniosis is unclear. It may be due to a root cause (primary), a consequence of a primary disease (secondary), such as a connective tissue disease or from abnormal proteins in the blood, or a disease of unknown causes (idiopathic). One hypothesis is that cold weather causes the small veins and arteries close to the skin to tighten or constrict. When the tissues are rewarmed, blood leaks into the tissue and causes the skin to swell. The swelling irritates the nerves and can cause pain. It is thought by some that the disorder may represent an allergic reaction or hypersensitivity to the cold. Prolonged exposure to the cold, insufficient protective clothing, and circulatory or cardiovascular diseases may also be causative factors. Tight clothing may decrease blood flow to the affected area causing a decrease in the skin’s temperature. Some cases are believed to be caused by genetic factors. Other suspected causes include nutrition, local infection, hormonal changes, and other systemic diseases. In the elderly, perniosis may be associated with an underlying systemic disease.
The incidence of perniosis is currently uncertain. Perniosis is seen more often in females than in males. More specifically, it is seen more often in women who are very thin or who have a low body mass index. Several articles in the medical literature focus on cases of perniosis in anorexic women. Individuals who smoke long-term or with poor circulation, are affected more frequently than the general population. This condition is more common in cold, damp climates than in dry ones. Many cases have been reported from Western Europe but cases have also been reported in the late winter to early spring in the coastal areas of North America. Symptoms usually begin before the age of 20 years. It does not commonly occur in children and elderly people.
Diagnosis is determined by ruling out other conditions or diseases.
Clinical Evaluations and Workup
The clinician will assess the individual for a history of prolonged cold exposure and conduct a physical examination, including a full skin examination to check for painful, itchy, red skin lesions on the extremities. An echocardiogram may be performed if an acute embolism is suspected. The clinician will also conduct a close examination of the process of rewarming the affected areas. The lesions should be self-limiting and resolve upon rewarming. The patient is also screened for autoimmune markers. Blood tests including a complete blood count, antinuclear antibodies, cryoglobulins, cryofibrinogen, cold agglutinins, antiphospholipid antibodies, and serum protein electrophoresis are performed to rule out other conditions. Skin biopsies are performed if the condition persists and an underlying systemic inflammatory disease is suspected.
First-line treatment and management of perniosis, consists of protecting the body from the cold and warming the affected areas slowly. For example, patients may benefit from wearing layered warm clothing, gloves, and socks. Individuals with perniosis should avoid scratching or rubbing the affected area to help avoid further damage to the skin.
Treatment with medications is second-line, and limited efficacy has been shown. The calcium channel blocker drug nifedipine (Adalat) may be an effective treatment for severe cases in decreasing the duration, severity, and recurrence of the lesions. Nifedipine works by widening the blood vessels. Patients given extended-release nifedipine, taken 20 mg three times daily, reported fast symptom improvement. Topical corticosteroids, such as topical mometasone or betamethasone, may help relieve the intense itching.
Treatment with intense pulsed light has been shown to reduce redness.
Patients are also encouraged to stop smoking because smoking decreases the amount of oxygen delivered to wounds and slows down the healing process.
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