Chilblains

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Chilblains (synonyms: perniosis, pernio) are localized, inflammatory lesions that are caused by exposure to cold, damp, non-freezing conditions. The pathogenesis is unknown, but chilblains are thought to represent an abnormal inflammatory response of vascular origin (vasospasm) to cold.

The onset is usually in the autumn or winter, with women, children, and the elderly being most frequently affected.

Lesions occur acutely as single or multiple erythematous to blue-violaceous macules, papules, or nodules. They are usually accompanied by pruritus, burning sensation, or pain and rarely in severe cases may blister or ulcerate. The lesions are often symmetrical with a predilection for fingers, toes, heels, nose, and ears.

Chilblains usually resolve spontaneously within 1–3 weeks, although they may be prolonged in elderly patients.

Perniosis may be idiopathic or represent a clinical manifestation of systemic diseases (connective tissue disorders, cold-induced syndromes, hematological malignancies) or eating disorders (anorexia nervosa, poor nutrition). During the COVID-19 pandemic pernio-like lesions, called ‘Covid toes’, occurred and were attributed to an associated coagulopathy.

Management Strategy

Clinical findings and history suggest the diagnosis and further investigations are seldom required. However, in patients with atypical and/or persistent lesions and in the elderly, investigations should be performed for underlying causes. The condition must be distinguished from chilblain lupus , a form of cutaneous lupus in which lesions develop in cold but tend to persist and ulcerate, and from lupus pernio , which is a form of cutaneous sarcoidosis that usually affects the face/nose.

The treatment of perniosis remains unsatisfactory.

For chilblains with no underlying pathology, preventive measures such as adequate clothing and heating and insulation, avoidance of cold, and avoidance of smoking are important.

Treatment includes use of vasodilator calcium channel blockers (nifedipine 20–60 mg daily, diltiazem 60–120 mg TID) , which have been shown to be effective in the treatment and prophylaxis of perniosis in patients with idiopathic acral perniosis and in those with low body weight. Pentoxifyllin e provides a safe alternative approach. A range of topical vasodilators and other treatments have been deployed with a largely anecdotal evidence base.

Specific Investigations

Investigations are not routinely required in typical cases, but consider:

CBC
Autoimmune profile (ANA, complement)
Antiphospholipid antibodies
Cryoglobulins
Cold agglutinins
Cryofibrinogen
Histology and immunofluorescence
Vascular assessment in the elderly
Serum protein electrophoresis

Nyssen A, Benhadou F, Magnée M, et al. Vasa 2020; 49(2): 133–40.

A comprehensive literature review regarding differential diagnosis and management of chilblains.

Chilblain lupus erythematosus – a review of literature

Hedrich CM, Fiebig B, Hauck FH, et al. Clin Rheumatol 2008; 27: 949–54.

This article reviews the clinical presentation, pathogenesis, diagnosis, and management of chilblain lupus erythematosus.

Pernio as the presenting sign of blast crisis in acute lymphoblastic leukemia

Park KK, Tayebi B, Uihlein L, et al. Pediatr Dermatol 2018; 35(1): e74–5.

A case of a 5-year-old girl with pernio as the presenting symptom of acute B-cell lymphoblastic leukemia.

Anorexia nervosa associated with acromegaloid features, onset of acrocyanosis and Raynaud’s phenomenon and worsening of chilblains

Rustin MH, Foreman JC, Dowd PM. J Roy Soc Med 1990; 83: 495–6.

Two patients are reported who developed severe perniosis in association with anorexia nervosa.

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