Pernio

Pernio

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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.

Pernio is due to an abnormal vascular response to cold exposure, [1] and it 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. Hyperhidrosis and low lody mass index are suggested associations. [2] The response of pernio to vasodilator drugs varies. Keeping acral areas warm and dry best prevents pernio.

The direct cause of pernio is cold exposure; specifically, exposure to both mild nonfreezing cold and humidity seems to be required. [3, 4] Chronic pernio may be secondary to various systemic diseases as follows:

Chronic myelomonocytic leukemia [5]

Anorexia nervosa [6] : Low body mass index may predispose to pernio. [3, 7]

Dysproteinemias

Macroglobulinemia

Cryoglobulinemia, cryofibrinogenemia, cold agglutinins

Antiphospholipid antibody syndrome

Raynaud disease [8]

Celiac disease [9]

Variants include the following:

Kibes (equestrian cold panniculitis): Erythrocyanotic plaques occur on the upper lateral thighs of women who ride horses. Histology is characterized by an intense perivascular infiltrate extending into subcutaneous fat.

Chilblain lupus erythematosus [10, 11] : Violaceous “pernio” plaques appear prominent over dorsal interphalangeal joints, often with positive antinuclear antibody (ANA) or rheumatoid factor (RF). Histologic and immunofluorescent evidence of lupus is present in the skin lesions. Half of the patients have associated facial discoid lupus lesions, and 15% develop systemic lupus.

Drug-induced pernio: Sulindac-induced cases have been reported. [12]

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. [13]

Women are affected by pernio more frequently than men.

Pernio is most frequent in young and middle-aged women and in children. Note the image below.

Prognosis is good. Recurrences may be observed annually with onset of cold weather. [7] Long-term follow-up of patients with chronic recurrent pernio is advised because this may reveal connective-tissue disease (lupus erythematosus). Most cases of pernio resolve without any adverse reactions.

Avoid exposure to cold.

Keep extremities warm and dry.

Cease smoking.

Shahi V, Wetter DA, Cappel JA, Davis MD, Spittell PC. Vasospasm Is a Consistent Finding in Pernio (Chilblains) and a Possible Clue to Pathogenesis. Dermatology. 2015 Sep. 231 (3):274-9. [Medline].

Singh GK, Datta A, Grewal RS, Suresh MS, Vaishampayan SS. Pattern of chilblains in a high altitude region of Ladakh, India. Med J Armed Forces India. 2015 Jul. 71 (3):265-9. [Medline].

Prakash S, Weisman MH. Idiopathic chilblains. Am J Med. 2009 Dec. 122(12):1152-5. [Medline].

Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008 Apr. 10(2):128-35. [Medline].

Kelly JW, Dowling JP. Pernio. A possible association with chronic myelomonocytic leukemia. Arch Dermatol. 1985 Aug. 121(8):1048-52. [Medline].

White KP, Rothe MJ, Milanese A, Grant-Kels JM. Perniosis in association with anorexia nervosa. Pediatr Dermatol. 1994 Mar. 11(1):1-5. [Medline].

Akkurt ZM, Ucmak D, Yildiz K, Yürüker SK, Celik HÖ. Chilblains in Turkey: a case-control study. An Bras Dermatol. 2014 Jan-Feb. 89 (1):44-50. [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. 1990 Aug. 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). 2010 Jan 28. [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. 1994 Dec. 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). 2001 May. 80(3):180-8. [Medline].

Reinertsen JL. Unusual pernio-like reaction to sulindac. Arthritis Rheum. 1981 Sep. 24(9):1215. [Medline].

Chan Y, Tang WY, Lam WY, et al. A cluster of chilblains in Hong Kong. Hong Kong Med J. 2008 Jun. 14(3):185-91. [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].

Verma P. Topical Nitroglycerine in Perniosis/Chilblains. Skinmed. 2015 May-Jun. 13 (3):176-7. [Medline].

Dowd PM, Rustin MH, Lanigan S. Nifedipine in the treatment of chilblains. Br Med J (Clin Res Ed). 1986 Oct 11. 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. 1989 Feb. 120(2):267-75. [Medline].

Michele S Maroon, MD Program Director, Department of Dermatology, Geisinger Medical Center

Michele S Maroon, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

David Hensley, MD Mullanax Dermatology Associates, Arlington Memorial Hospital

David Hensley, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Pennsylvania Medical Society, Southern Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.

Craig A Elmets, MD Professor and Chair, Department of Dermatology, Director, Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: University of Alabama at Birmingham; University of Alabama Health Services Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary from Massachusetts Medical Society for employment; Received salary from UpToDate for employment. for: Astellas.

Pernio

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