Background
Pernio is an inflammatory skin condition presenting after exposure to cold as pruritic and/or painful erythematous-to-violaceous acral lesions. Pernio may be idiopathic or secondary to an underlying disease. Note the image below.
A 63-year-old man with pernio presenting as acral violaceous plaques with bullae. Pathophysiology
Pernio is due to an abnormal vascular response to cold exposure and is most frequent when damp or humid conditions coincide. Minor trauma also may predispose the acral parts to symptomatic pernio lesions in otherwise appropriate weather conditions. The response of pernio to vasodilator drugs varies. Keeping acral areas warm and dry best prevents pernio.
Epidemiology
Frequency
United States
The true incidence of pernio is unknown because pernio frequently is unrecognized or misdiagnosed.
International
Rates of pernio vary with climate. England, with its cool damp climate, has an annual incidence rate of pernio of 10%. A clustering of pernio cases has been reported from Hong Kong during January and February, with resolution of most cases within a few weeks when the weather warmed.[1]
Mortality/Morbidity
Most cases of pernio resolve without any adverse reactions.
Sex
Women are affected by pernio more frequently than men.
Age
Pernio is most frequent in young and middle-aged women and in children. Note the image below.
Erythematous macules on distal toes of a 6-month-old girl with pernio. Chan Y, Tang WY, Lam WY, et al. A cluster of chilblains in Hong Kong. Hong Kong Med J. Jun 2008;14(3):185-91. [Medline].
Prakash S, Weisman MH. Idiopathic chilblains. Am J Med. Dec 2009;122(12):1152-5. [Medline].
Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. Apr 2008;10(2):128-35. [Medline].
Kelly JW, Dowling JP. Pernio. A possible association with chronic myelomonocytic leukemia. Arch Dermatol. Aug 1985;121(8):1048-52. [Medline].
White KP, Rothe MJ, Milanese A, Grant-Kels JM. Perniosis in association with anorexia nervosa. Pediatr Dermatol. Mar 1994;11(1):1-5. [Medline].
Rustin MH, Foreman JC, Dowd PM. Anorexia nervosa associated with acromegaloid features, onset of acrocyanosis and Raynaud's phenomenon and worsening of chilblains. J R Soc Med. Aug 1990;83(8):495-6. [Medline].
Kearby R, Bowyer S, Scharrer J, Sharathkumar A. Case Report: Six-Year-old Girl With Recurrent Episodes of Blue Toes. Clin Pediatr (Phila). Jan 28 2010;[Medline].
Su WP, Perniciaro C, Rogers RS 3rd, White JW Jr. Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria. Cutis. Dec 1994;54(6):395-9. [Medline].
Viguier M, Pinquier L, Cavelier-Balloy B, et al. Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus. Medicine (Baltimore). May 2001;80(3):180-8. [Medline].
Reinertsen JL. Unusual pernio-like reaction to sulindac. Arthritis Rheum. Sep 1981;24(9):1215. [Medline].
Langtry JA, Diffey BL. A double-blind study of ultraviolet phototherapy in the prophylaxis of chilblains. Acta Derm Venereol. 1989;69(4):320-2. [Medline].
Dowd PM, Rustin MH, Lanigan S. Nifedipine in the treatment of chilblains. Br Med J (Clin Res Ed). Oct 11 1986;293(6552):923-4. [Medline].
Rustin MH, Newton JA, Smith NP, Dowd PM. The treatment of chilblains with nifedipine: the results of a pilot study, a double-blind placebo-controlled randomized study and a long-term open trial. Br J Dermatol. Feb 1989;120(2):267-75. [Medline].
Carruthers R. Chilblains (perniosis). Aust Fam Physician. Nov 1988;17(11):968-9. [Medline].
Crowson AN, Magro CM. Idiopathic perniosis and its mimics: a clinical and histological study of 38 cases. Hum Pathol. Apr 1997;28(4):478-84. [Medline].
Goette DK. Chilblains (perniosis). J Am Acad Dermatol. Aug 1990;23(2 Pt 1):257-62. [Medline].
Jacob JR, Weisman MH, Rosenblatt SI, Bookstein JJ. Chronic pernio. A historical perspective of cold-induced vascular disease. Arch Intern Med. Aug 1986;146(8):1589-92. [Medline].
Parra SL, Wisco OJ. What is your diagnosis? Perniosis (Chilblain). Cutis. Jul 2009;84(1):15, 27-9. [Medline].
Simon TD, Soep JB, Hollister JR. Pernio in pediatrics. Pediatrics. Sep 2005;116(3):e472-5. [Medline].
Spittell JA Jr, Spittell PC. Chronic pernio: another cause of blue toes. Int Angiol. Jan-Mar 1992;11(1):46-50. [Medline].

