eMedicine Specialties > Dermatology > Environmental

Pernio

Author: Michele S Maroon, MD, Program Director, Department of Dermatology, Geisinger Medical Center
Coauthor(s): David Hensley, MD, Mullanax Dermatology Associates, Arlington Memorial Hospital
Contributor Information and Disclosures

Updated: Jan 29, 2009

Introduction

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.

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 lesions in otherwise appropriate weather conditions. The response to vasodilator drugs varies. Keeping acral areas warm and dry best prevents pernio.

Frequency

United States

The true incidence is unknown, as pernio frequently is unrecognized or misdiagnosed.

International

Rates of pernio vary with climate. England, with its cool damp climate, has an annual incidence of 10%.

Mortality/Morbidity

Most cases resolve without any adverse reactions.

Sex

Women are affected more frequently than men.

Age

Pernio is most frequent in young and middle-aged women and in children.

Clinical

History

Most patients present with a history of recurrent painful and/or pruritic, erythematous, violaceous papules or nodules on the fingers and/or toes. Most cases resolve within 2-3 weeks. Elicit a history of cold exposure or repeated episodes of cold exposure.

Physical

Pertinent findings are limited to the skin. Cutaneous lesions present 12-24 hours after cold exposure as red or violaceous macules, papules, nodules, or plaques, which may form vesicles or ulcerate. They occur on acral areas, are associated with burning or pruritus, and last 1-3 weeks.

Causes

The direct cause of pernio is cold exposure. Chronic pernio may be secondary to various systemic diseases as follows:

  • Chronic myelomonocytic leukemia1
  • Anorexia nervosa2
  • Dysproteinemias
  • Macroglobulinemia
  • Cryoglobulinemia, cryofibrinogenemia, cold agglutinins
  • Antiphospholipid antibody syndrome
  • Raynaud disease3
  • Variants
    • 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 erythematosus4,5 : 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: Sulindac induced cases have been reported.6

More on Pernio

Overview: Pernio
Differential Diagnoses & Workup: Pernio
Treatment & Medication: Pernio
Follow-up: Pernio
Multimedia: Pernio
References
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References

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

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

  3. 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].

  4. 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].

  5. 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].

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

  7. 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].

  8. 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].

  9. 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].

  10. Carruthers R. Chilblains (perniosis). Aust Fam Physician. Nov 1988;17(11):968-9. [Medline].

  11. 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].

  12. Goette DK. Chilblains (perniosis). J Am Acad Dermatol. Aug 1990;23(2 Pt 1):257-62. [Medline].

  13. 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].

  14. Simon TD, Soep JB, Hollister JR. Pernio in pediatrics. Pediatrics. Sep 2005;116(3):e472-5. [Medline].

  15. Spittell JA Jr, Spittell PC. Chronic pernio: another cause of blue toes. Int Angiol. Jan-Mar 1992;11(1):46-50. [Medline].

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Further Reading

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Keywords

pernio, chilblains, perniosis

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Contributor Information and Disclosures

Author

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, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

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, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham
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, and Society for Investigative Dermatology
Disclosure: Palomar Medical Technologies Stock None; Merck Consulting fee Independent contractor; Tronox Consulting fee Independent contractor; Amgen Consulting fee Review panel membership; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

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, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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