Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 22/04/2025
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Antonios Kanelleas and John Berth-Jones
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 (also called perniosis) are localized, inflammatory, erythematous lesions that are caused by exposure to cold ambient temperatures above freezing point. High humidity and wind, which exacerbate conductive heat loss, also play a significant part. They are thought to be caused by a persistent vasoconstriction of the deep cutaneous arterioles with accompanying dilatation of the small superficial vessels. The onset is usually in the autumn or winter. Chilblains are more common in temperate climates, when winters get rather cold and damp and people are not used to these conditions. Lesions occur acutely as single or multiple erythematous or dusky swellings that may occasionally ulcerate or blister. They are usually accompanied by pruritus or a burning sensation. Sites of predilection are the fingers, toes, heels, lower legs, thighs, nose, and ears. A specific subset occurs on the thighs of patients wearing tight-fitting, poorly insulating trousers (e.g., as worn by young horsewomen). Perniosis can also be a manifestation of eating disorders (anorexia nervosa, poor nutrition), and systemic diseases (lupus erythematosus and hematological malignancies).
The most important aspect is prophylaxis. This will be achieved through the use of warm clothing and warm, properly insulated housing. Avoidance of exposure in cold weather is, obviously, equally important. Once chilblains occur, they usually run a self-limiting course over a period of a few weeks. Treatment includes rest in a warm environment and possibly topical antipruritics, if needed. Vasodilator calcium channel blockers (nifedipine 20–60 mg daily, diltiazem 60–120 mg three times daily) have been shown to be an effective therapy and preventative measure in patients with idiopathic acral perniosis, and in those patients with perniosis associated with low body weight. Appropriate investigation to exclude myeloproliferative disorders, connective tissue diseases, and eating disorders is required. Particularly in children, chilblains have been linked with cold-sensitive dysproteinemia. In elderly patients and those with ulcerative lesions, peripheral vascular insufficiency must be excluded. The condition must be distinguished from chilblain lupus erythematosus (LE). The latter is a form of cutaneous LE manifesting with lesions resembling chilblains. Lesions develop in cold weather but tend to persist and ulcerate. Chilblain LE may be accompanied by discoid LE. Up to 20% of patients with chilblain LE will develop systemic LE.
Investigation is not routinely required in typical cases, but consider:
Full blood count
Autoimmune profile
Cryoglobulins
Cold agglutinins
Cryofibrinogen
Vascular imaging in elderly patients
Histology and immunofluorescence
Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, et al. Clin Rheumatol 2008; 27: 949–54.
This article reviews the clinical presentation, pathogenesis, diagnosis, and management of chilblain lupus erythematosus.
Kelly JW, Dowling JP. Arch Dermatol 1985; 121: 1048–52.
A series of four elderly men has been described in whom perniosis preceded the onset of chronic myelomonocytic leukemia.
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.
White KP, Rothe MJ, Milanese A, Grant-Kels JM. Paediatr Dermatol 1994; 11: 1–5.
St Clair NE, Kim CC, Semrin G, Woodward AL, Liang MG, Glickman JN, et al. Pediatr Dermatol 2006; 23: 451–4.
This is a case report of an adolescent girl in whom chilblains were the main presenting sign of celiac disease. A gluten-free diet resulted in both weight gain and resolution of chilblains.
Weston WL, Morelli JG. Pediatr Dermatol 2000; 17: 97–9.
A 10-year retrospective study of a pediatric clinic identified eight patients with perniosis, four of whom had cryoglobulins or cold agglutinins and two had positive rheumatoid factor.
De Silva BD, McLaren K, Doherty VR. Clin Exp Dermatol 2000; 25: 285–8.
Two cases of equestrian perniosis associated with cold agglutinins.
Rustin MH, Newton JA, Smith NP, Dowd PM. Br J Dermatol 1989; 120: 267–75.
Ten patients with severe recurrent acral perniosis were treated with 20 mg of nifedipine retard or placebo in a double-blind crossover trial for 12 weeks in total. No patients developed new lesions while on treatment and 70% were clear after a mean of 8 days. In the open study, 34 patients received up to 60 mg of nifedipine retard for 2 months; this was shown to be effective in reducing the healing time and symptoms of lesions.
Patra AK, Das AL, Ramadasan P. Indian J Dermatol Venereol Leprol 2003; 69: 209–11.
The authors compared two groups of patients with chilblains. Group A (12 patients) was treated with diltiazem 60 mg three times daily and group B (24 patients) with nifedipine 10 mg three times daily until complete relief, and then 20 mg twice daily for maintenance. They concluded that nifedipine has greater efficacy than diltiazem (80–90% of patients from group B showed relief by the 14th day of treatment).
Gaynor S. J Am Acad Dermatol 1983; 8: 13.
This author reports successful treatment of patients with perniosis using topical corticosteroids (0.025% fluocinolone cream) under occlusion nightly.
Topical corticosteroids are frequently used for the treatment of chilblains, but their use is not based on controlled trials.
Dowd PM, Blackwell V. In: Lebwohl, M, Heymann, W, Berth-Jones, J, Coulson, I, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies, 2nd edn. Chicago: Mosby, 2006; 39–40.
These authors reported use of 2% hexyl nicotinate in aqueous cream preparation and minoxidil 5% lotion applied three times a day, in a number of patients with chilblains and Raynaud’s phenomenon, and acidified nitrite cream (3% salicylic acid and 3% potassium nitrate) three times daily in patients with chilblains intolerant of calcium channel blockers. Low-dose (5 mg daily) tamoxifen proved useful in anorexia-associated perniosis.
Weismann K, Grønhøj Larsen F. Acta Dermatol Venereol 2006; 86: 558–9.
The authors describe two adolescent girls from Denmark who developed chilblains on their right hip. This was attributed to exposure to cold, associated with wearing tight-fitting jeans with a low waistband that left uncovered the upper part of the hip region. Both were treated with intense pulsed light (555–950 nm), 14 J/cm2. After three monthly treatments, the redness was reduced.
Noaimi AA, Fadheel BM. Saudi Med J 2008; 29: 1762–4.
Forty patients with chilblains were randomly divided into two equal groups. Group A received oral prednisolone 0.5 mg/kg/day, in two doses, and topical clobetasol ointment for 2 weeks. Patients in group B received pentoxifylline tablets 400 mg three times daily for 2 weeks. In group B, five of nine patients (55.5%) who completed the study showed a significant improvement, compared with three of 11 patients (27.2%) in group A with similar results.
Yang X, Perez OA, English 3rd JC. J Drugs Dermatol 2010; 9: 1242–6.
In this retrospective study hydroxychloroquine improved symptoms in four out of five patients with perniosis.
Langry JA, Diffey BL. Acta Dermatol Venereol 1989; 69: 320–2.
Anecdotally, UV light was used at the beginning of winter for the prophylactic treatment of chilblains, but this randomized double-blind study concluded that UV light was of no benefit.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Conservative management
Avoidance of further cold injury
Calcium channel blockers
Topical corticosteroids
Topical 2% hexyl nicotinate cream
Minoxidil 5% lotion
Acidified nitrate cream
Tamoxifen
Intense pulsed light
Oral pentoxifylline
Hydroxychloroquine
