Perniosis - Causes, Symptoms, Diagnosis, and Treatment

Introduction:

Perniosis is an inflammatory condition of the skin due to extreme cold exposure. During cold temperatures, small blood vessels get inflamed, causing localized vasculitis. Characteristic features of perniosis include painful, itching lesions with bluish discoloration on the skin. In severe conditions, along with painful lesions, bullae are present. Sometimes bullae lead to secondary infections and scarring on healing. Perniosis is a seasonal inflammatory cutaneous condition mostly reported in cold weather conditions. The exact etiology of perniosis is unknown. Perniosis is most often present in females than in males.

What Is Perniosis?

Perniosis is a cold-induced vascular disease characterized by an inflammation of the blood vessels causing cyanotic skin discoloration. Skin lesions cause burning pain, itching, and tingling sensation. Due to the abnormal reaction of cold, reddish-blue skin discoloration is seen in various body parts. The common areas in perniosis are the lower extremities, hands, toes, ears, feet, and face. The onset of the perniosis condition is usually 12 to 24 hours after cold exposure, and the symptoms last for 2 to 3 weeks. Symptoms due to cold weather are resolved in the summer months. It is also observed that the signs remain persistent in warmer conditions. Perniosis is also known as chilblain, pernio,cold-induced vascular disease, and erythema.

Other names of perniosis: are chilblain, pernio, erythema, and cold-induced vascular disease.

What Causes Perniosis?

The exact etiology of perniosis is not known. The primary factor for the occurrence of perniosis is extreme cold temperature. Perniosis is associated with other connective tissue disorders or may be due to abnormal proteins. Perniosis is sometimes idiopathic. Idiopathic conditions are those that occur with a disease of unknown cause. It is also stated that, by tight clothing, skin gets decreased blood flow, causing a cyanotic response. Perniosis might occur with various factors, including nutritional deficiency, local infection, hormonal changes, cardiovascular dysfunction, and systemic diseases.

Mentioned below are the causes of perniosis:

• Cold exposure.

• Insufficient clothing.

• Tight clothing.

• Connective tissue diseases.

• Cardiovascular disorders.

• Circulatory dysfunction.

• Hormonal changes.

• Nutritional deficiency.

• Local infection.

• Systemic diseases.

What Are the Signs and Symptoms of Perniosis?

Perniosis is characterized by cyanotic discoloration of the skin. Symptoms include burning pain, itching, and tingling sensation in the affected area. The reddish-blue discoloration is seen on the lower extremities, hands, feet, toes, heels, fingers, ears, and nose. Perniosis with reddish-blue discoloration rarely appears in the thighs and buttocks region. Females wearing tight jeans are highly subjected to perniosis, and the symptoms are seen in the thigh region. Young to middle-aged women are the most commonly affected population. Reddish-blue patches are seen in the affected area, occasionally leading to swelling, burning pain, and ulceration. Perniosis is a benign condition, and the symptoms last for two to three weeks.

Mentioned below are a few of the signs and symptoms of perniosis.

• Color: Reddish-blue discoloration.

• Affected Area: Lower legs, hands, fingers, toes, ears, nose, and thighs.

• Gender: Females are more susceptible than males.

• Affected Population: Young to middle-aged women.

• Symptoms: Swelling, pain, itching, tingling sensation in the affected area.

• Complications: Blisters, bullae, ulceration, and scarring.

Who Is at Risk of Perniosis?

Perniosis affects all age groups. It is most common among young to middle-aged women.

Some risk factors for perniosis development are mentioned below.

• Smoking.

• Poor nutrition.

• Low level of Body Mass Index or BMI.

• Outdoor workers in cold weather also include fishermen, swimmers, and farmers.

• People who ice skate.

• People wearing tight clothes.

• Systemic diseases such as chronic myelomonocytic leukemia.

• Individuals with connective tissue disorders, especially systemic lupus erythematosus or SLE

• Associated conditions like acrocyanosis, Raynaud's phenomenon.

• Familial cause.

Complications of Perniosis

In severe cases, skin lesions coalesce to form bullae. Bursting or irritating the blisters will cause ulceration and other complications.

The complication of perniosis include,

• Blisters.

• Painful swelling.

• Ulceration.

• Scarring.

How Is Perniosis Diagnosed?

A proper history and physical examination are mandatory in diagnosing perniosis. The clinical examination must include a complete skin examination to look for reddish-blue discoloration in the affected area. Other symptoms are also observed to know the severity of perniosis. Perniosis due to the abnormal blood supply is examined with an echocardiogram. Perniosis is a self-limiting condition that resolves symptoms in warm conditions. In the case of perniosis with persistent symptoms, the underlying disease is diagnosed with specific measures.

Mentioned below are a few of the diagnostic tools that are used to diagnose perniosis.

• Proper history taking.

• Physical examination.

• Complete blood count.

• Autoimmune markers are evaluated.

• Echocardiogram for acute embolism.

• Antinuclear antibodies.

• Cryoglobulins.

• Cryofibrinogen.

• Cold agglutinins

• Serum protein electrophoresis is done.

• Skin biopsy.

What Is the Differential Diagnosis of Perniosis?

Symptoms of perniosis are chilblains that can be similar to the following disorders. Comparisons of symptoms are helpful in the differential diagnosis.

The possible symptoms of perniosis often mimic the following skin condition.

• Raynaud's disease.

• Chilblain lupus erythematosus.

• Vasculitis.

• Cold urticaria.

• Cellulitis.

• Irritant contact dermatitis.

• Acute embolism.

• Acrocyanosis.

• Aicardi-Goutieres syndrome.

• Cold panniculitis.

• Cryofibrinogenemia.

What Is the Treatment of Perniosis?

Perniosis is a self-limiting condition. The first line of treatment is done to increase the body temperature. Systemic therapy is advised in perniosis with underlying disease.

Various measures for the treatment of perniosis are given below.

• Protecting the body from extreme cold.

• Warming the affected areas.

• The second line of therapy.

• Calcium channel blockers: Nifedipine helps in vasodilation.

• Topical corticosteroids

• Topical mometasone.

• Topical betamethasone helps in relieving itching and burning sensations.

• Erythema is reduced with intense pulse light.

How to Avoid Perniosis at Home?

Certain measures can be taken at home to reduce the cold temperature and get a healthy lifestyle through lifestyle modifications.

The following methods can be performed to avoid the perniosis condition at home.

• Cessation of smoking.

• Avoid tight clothes.

• Protecting the skin from cold conditions with layered clothes, socks, and gloves.

• Healthy, nutritious diet.

• Always keep the skin dry and warm.

• Reduce or limit cold exposure.

• Exercise regularly.

• Avoid a sedentary lifestyle.

• Rooms and workplaces can be equipped with heaters to maintain warm conditions.

• Use footwear with leather or canvas.

Food Tips for Perniosis

Perniosis occurs due to impaired blood circulation in the skin peripheries. Some foods help to improve blood circulation. Such foods have beneficial effects on people with perniosis.

Foods to consume for perniosis or chilblains include:

• Cayenne.

• Pepper.

• Ginger.

• Turmeric.

• Cinnamon.

• Garlic.

• Onion.

• Fatty fish.

• Sesame seeds.

• Oats.

• Almonds.

• Vitamin niacin supplements (vitamin B3)

Conclusion:

Perniosis or chilblains is a benign cutaneous condition. Perniosis is a self-limiting condition requiring no treatment in the primary type. Avoiding or limiting cold exposure resolves the symptoms. In severe pain, get medical help from the physician. The doctor advised treatment will prevent the chance of complications. Poor nutrition should be corrected with a good diet for a healthy lifestyle.