Overview Of Perniosis
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.
Commonly Associated With
- cold induced vascular disease
- erythema, pernio
Causes Of Perniosis
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.
Symptoms Of Perniosis
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.
Treatment Of Perniosis
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.
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.
Comparisons may be useful for a differential diagnosis:
Raynaud’s disease, or Raynaud’s phenomenon, is a vascular disorder. It is characterized by spasms of arterioles, especially in the fingers and toes due to exposure to cold. Occasionally other areas of the body such as the nose and tongue may be affected. The intermittent attacks of pallor or bluish color of the fingers or toes are precipitated by exposure to cold and intensified by emotional upsets. Raynaud’s may occur alone or be secondary to other conditions.
Chilblain lupus erythromatosus is a chronic and persistent form of lupus erythromatosus. It may be preceded by facial lesions, but most commonly affects the fingers, calves and heels of the feet. It is a disorder that affects mostly women and may progress to systemic lupus erythromatosus, which can affect the internal organs.
Vasculitis is a common disorder characterized by an inflammation of the blood vessel walls. This inflammation causes a narrowing of the inside of the vessel and can obstruct the flow of blood to the tissues (ischemia). The lack of blood may cause tissue death (necrosis), formation of blood clots (thrombosis), or the weakening and ballooning of an artery, which can possibly rupture (aneurysm).
Cold urticaria is a chronic, reactive skin disorder. It is probably the most common form of physical urticaria. Major symptoms may include abnormal reddening of the skin (erythema), hives, and itching after skin exposure to cold temperatures.
Cellulitis is a bacterial infection of the skin. The skin is painful, red, swollen, and warm to the touch. Usually, the lower legs are affected, but it is normally only on one side (unilateral). This condition is treated with systemic antibiotics.
Irritant contact dermatitis is a skin condition that primarily affects the hands. It is characterized by red, dry, rough, and chapped skin and can be caused by contact with an irritant (e.g. detergent), not by contact with the cold.
Acute embolism to the lower extremities is a blood clot that causes decreased blood flow (ischemia) to the legs and/or feet. As a result, the skin can be painful, pale, and warm as the clot worsens. In addition, individuals may show signs of decreased pulse, numbness, or even paralysis to the affected area.
Acrocyanosis is a condition that presents with chronic coolness and a purple discoloration of primarily the hands and feet. However, it may also affect the nose, ears, lips, and nipples. Cold temperatures can worsen symptoms.
Aicardi-Goutières syndrome is a rare genetic disorder, which is associated with a mutation in the TREX1 gene. It presents in infancy and primarily affects women. Approximately 40% of children with syndrome present with pernio-like skin lesions.
Cold panniculitis is inflammation of subcutaneous fat (adipose) tissue due to direct cold exposure, such as contact with popsicles. This condition is common in young children. It presents as red, hardened raised patches (plaques), which resolve in a few weeks. This disease may also be confirmed by removing a sample of tissue from an affected area and then testing the sample (biopsy).
Cryofibrinogenemia is a condition in which the blood plasma forms a precipitate during cooling conditions (cryoprecipitate). This disease is confirmed by the presence of proteins in the plasma called cryofibrinogen and the absence of circulating proteins called cryoglobulins. It may lead to the blockage of a blood vessel (secondary vascular occlusion), purple-colored spots (purpura), or skin cell death (necrosis) in areas such as the hands, feet, ears, and nose.