Laboratory Studies
The following laboratory tests may be needed:
- CBC count and sedimentation rate: These should be obtained to rule out associated leukemia.
- Antiphospholipid antibody panel: Review of patients presenting with pernio shows an increased incidence of antiphospholipid antibody syndrome.
- Cryoglobulins, cryofibrinogen, and cold agglutinin testing: These generally are absent but should be considered as part of the laboratory evaluation in a patient with chronic pernio. Because of occasional false-negative cryoprecipitate screening results, consider hepatitis C antibody screening or even rheumatoid factor (RF) as a marker for cryoglobulinemia in select cases.
- Antinuclear antibody (ANA): Pernio lesions can occur in the setting of lupus erythematosus.
- Serum protein electrophoresis (SPEP) and quantitative immunoglobulins: Dysproteinemias and macroglobulinemia, causing increased serum viscosity, may be associated with pernio.
Histologic Findings
Pernio can often be diagnosed on the basis of clinical findings. Biopsy may be indicated to rule out other inflammatory processes in difficult chronic cases. Punch biopsy is adequate. There is variable epidermal spongiosis or necrosis. Intense papillary dermal edema is present. A superficial and deep perivascular lymphocytic infiltrate is seen, with the described "fluffy edema" of vessel walls. Lymphocytic vasculitis may be present.
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].

