Pernio Clinical Presentation

  • Author: Michele S Maroon, MD; Chief Editor: William D James, MD   more...
 
Updated: Mar 1, 2012
 

History

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

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Physical

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

Close-up of erythematous macules and plaques on diClose-up of erythematous macules and plaques on distal plantar toes. Close-up of great toe bulla. Close-up of great toe bulla.
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Causes

The direct cause of pernio is cold exposure; specifically, exposure to both mild nonfreezing cold and humidity seems to be required.[2, 3] Chronic pernio may be secondary to various systemic diseases as follows:

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[8, 9] : 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.[10]
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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.

Specialty Editor Board

Craig A Elmets, MD  Professor and Chair, Department of Dermatology, Director, 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, and Society for Investigative Dermatology

Disclosure: Palomar Medical Technologies Stock None; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment; Abbott Laboratories Grant/research funds Independent contractor; UpToDate Salary Employment; Biogen Grant/research funds Independent contractor; Clinuvel Independent contractor; Covan Basilea Pharmaceutical Grant/research funds Independent contractor; ISDIN None Consulting; TenX BIopharma Grant/research funds Independent contractor

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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

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, and Texas Medical Association

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, 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 and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. 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].

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

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

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

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

  6. 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].

  7. 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].

  8. 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].

  9. 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].

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

  11. 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].

  12. 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].

  13. 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].

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

  15. 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].

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

  17. 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].

  18. Parra SL, Wisco OJ. What is your diagnosis? Perniosis (Chilblain). Cutis. Jul 2009;84(1):15, 27-9. [Medline].

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

  20. 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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Erythematous macules on distal toes of a 6-month-old girl with pernio.
Close-up of erythematous macules and plaques on distal plantar toes.
A 63-year-old man with pernio presenting as acral violaceous plaques with bullae.
Close-up of great toe bulla.
 
 
 
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