Perniosis

Key Points
Type of localized Vasculitis typically affecting children and elderly persons
Caused by the constriction of blood vessels due to cold
Several factors contribute to development of the condition
Consists of purple or red bumps which may be accompanied by itching or tenderness

Perniosis (Chiblains) is a type of localized Vasculitis which typically affects children and elderly persons. The condition presents as purple or red bumps which may be accompanied by itching or tenderness. Lesions may form in various locations, including the nose, ears, fingers, toes, thighs, lower legs, and on the wrists in young children. Lesions typically present within a few hours after exposure and may take up to 2 weeks to resolve. Blistering, scabs, ulceration and pustules may be present in severe cases. Rarely, lesions may be ringlike or thickened. Thickened lesions often endure for months.

Perniosis is caused by the constriction of blood vessels due to exposure to cold. When vessels become warm again, blood leaks into the tissue, causing lesions. A variety of factors may contribute to the development of the condition, including heredity, nutritional deficiency/low body weight, hormone level shifts, and conditions which affect the bone marrow, vascular system, and connective tissue. In children, the condition is typically intermittent and recovery complete. In the elderly, the condition worsens with age. The condition is much less common in cold climates.

Differential Diagnosis (Other conditions with similar appearance)
Erythema multiforme
Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Sarcoidosis

Diagnosis
Key Points
Diagnosis based on the appearance of the affected area
Laboratory testing may be performed to confirm diagnosis and rule out other conditions

Perniosis is typically diagnosed based on the appearance of the affected area. Laboratory testing may be necessary to confirm the diagnosis and to rule out similar appearing conditions.

Treatment
Keeping warm and maintaining circulation are key in preventing development of lesions
Lesions typically have little response to treatment
*Therapies which may be effective include topical corticosteroids and Antibiotics

The primary methods of preventing development of lesions are keeping warm during cold weather and maintaining proper circulation. Smoking can seriously restrict bloodflow and it is recommended that affect persons avoid it. Unfortunately, lesions respond very poorly to therapy. Therapies which may be effective include the use of topical steroids and topical or oral antibiotics.