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Keys To The Evaluation And Treatment Of Pernio

Pernio, also known as chilblains, is an inflammatory skin disorder, which occurs after exposure to cold (subfreezing), damp environments. Pernio occurs most commonly in young to middle-aged women, with a mean age of diagnosis of 38.3 years and a 2:1 ratio of occurrence in female versus male patients.1 Low body mass index and hyperhidrosis are also associated with an increased incidence of pernio.2 The skin lesions of pernio are red to violaceous macules or plaques, which may form vesicles or ulcerate. The lesions are commonly painful or pruritic. The cutaneous findings are localized to acral areas, most commonly the dorsal toes followed by the fingers. The pathological process in pernio is an abnormal neurovascular response to cold skin temperatures.3

In the majority of cases, pernio is a benign, idiopathic condition with skin lesions resolving in one to three weeks.4 Chronic lesions may indicate an association with an underlying systemic disease including systemic lupus erythematosus, sarcoidosis, anti-phospholipid antibody syndrome, cryoglobulinemia, Raynaud’s phenomenon and chronic myelomonocytic leukemia.5

Chilblain lupus erythematosus occurs in patients with systemic lupus erythemaotsus and presents as violaceous plaques on the distal digits and dorsal interphalangeal joints.6 These skin lesions are very similar to those one would see in patients with idiopathic pernio. Clinicians can distinguish between these lesions through histologic analysis and workup for systemic lupus.7 Lupus pernio occurs secondary to sarcoidosis and is characterized by violaceous nodules and plaques on the central face.8 Cryoglobulemia and anti-phospholipid antibody syndrome are systemic thromboembolic diseases that can produce acral skin lesions similar to idiopathic pernio, but with more sharply angulated, non-blanching distal lesions or retiform purpura.1,9 

Clinicians have reported pernio in patients with chronic myelomonocytic leukemia. Skin lesions associated with this malignancy often have extensive or atypical cutaneous lesions, cellular atypia on histological exam or systemic symptoms of malignancy.10

Raynaud’s phenomenon is characterized by vasoconstriction and vasospasm leading to a classic red, white and blue discoloration of entire digits. However, this condition can be associated with the more distally localized lesions of pernio.11 Histological analysis of idiopathic perinio is characterized by interface dermatitis and one can use this histologic presence to distinguish it from cases of pernio with underlying systemic disease.5 Histologic or systemic workup is indicated in patients with severe or chronic pernio, and in patients with symptoms of systemic disease.

Prevention is the mainstay in the treatment of pernio. Preventative modalities include warm clothing, avoidance of cold/moist environments, hand and toe warming heat packs, warm water soaks and avoidance of nicotine.12 Ultraviolet light, which can damage small vessels and minimize their ability to constrict, has been advocated as a treatment modality to prevent pernio. However, a double-blind study by Langtry and colleagues found no significant effect with the use of ultraviolet therapy to prevent pernio.13

Several topical and oral medications have proven to be effective in the treatment of idiopathic pernio. While clinicians have utilized topical and oral corticosteroids in the treatment of pernio, the corticosteroids had low success rates.14 Nitroglycerine ointment is a potent vasodilator that has shown to be a successful treatment modality for pernio. Verma examined the use of 0.2% nitroglycerin ointment for the treatment of pernio and found 82% of patients had clinical remission within one week of using this topical medication.15 Similarly, a case study by Verma and coworkers showed successful treatment of pernio in a 6-month-old boy with topical nitroglycerin ointment.16 

Pentoxifylline is a phosphodiesterase inhibitor that improves red blood cell deformity and blood viscosity. Noaimi and Fadheel found oral pentoxifylline to be a safe and effective drug for the treatment of pernio with a success rate of 55.5 percent.14 They additionally found pentoxifylline to be superior to a combination of oral and topical glucocorticoids.

Calcium channel blockers are peripheral vasodilators that have been shown to be effective treatment modalities for pernio. Nifedipine is a calcium channel blocker that inhibits vascular smooth muscle contraction, producing vasodilation of the systemic arteries. Typical dosage for nifedipine in the management of pernio is 20 mg two to three times per day.17 Patra and colleagues showed that nifedipine was successful for the treatment of pernio with 80 to 90 percent resolution in one to two weeks.17 Diltiazem, a newer calcium channel blocker, is also reportedly effective for pernio but to a lesser extent than nifedipine.17 Side effects of peripheral vasodilators can include hypotension and dizziness. 

Pernio is an inflammatory disorder of the skin resulting from abnormal vascular response to subfreezing, moist environments. Pernio may be idiopathic or can result from underlying systemic disease. Prevention is paramount in the management of pernio. Once skin lesions develop, the research has shown that nifedipine is the most successful treatment modality for this condition.

References

1.         Cappel JA, Wetter DA. Clinical characteristics, etiologic associations, laboratory findings, treatment, and proposal of diagnostic criteria of pernio (chilblains) in a series of 104 patients at Mayo Clinic, 2000 to 2011. Mayo Clinic Proceedings. 2014;89(2):207-215.

2.         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;71(3):265-269.

3.         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;231(3):274-279.

4.         Vano-Galvan S, Martorell A. Chilblains. CMAJ. 2012;184(1):67.

5.         Akkurt ZM, Ucmak D, Yildiz K, Yuruker SK, Celik HO. Chilblains in Turkey: a case-control study. An Bras Dermatol. 2014;89(1):44-50.

6.         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;54(6):395-399.

7.         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;80(3):180-188.

8.         Consensus development conference on diabetic foot wound care. 7-8 April 1999, Boston, MA. American Diabetes Association. Adv Wound Care. 1999;12(7):353-361.

9.         Lutz V, Cribier B, Lipsker D. Chilblains and antiphospholipid antibodies: report of four cases and review of the literature. Br J Dermatol. 2010;163(3):645-646.

10.       Kelly JW, Dowling JP. Pernio. A possible association with chronic myelomonocytic leukemia. Arch Dermatol. 1985;121(8):1048-1052.

11.       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;83(8):495-496.

12.       Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008;10(2):128-135.

13.       Langtry JA, Diffey BL. A double-blind study of ultraviolet phototherapy in the prophylaxis of chilblains. Acta Derm Venereol. 1989;69(4):320-322.

14.       Noaimi AA, Fadheel BM. Treatment of perniosis with oral pentoxyfylline in comparison with oral prednisolone plus topical clobetasol ointment in Iraqi patients. Saudi Med J. 2008;29(12):1762-1764.

15.       Verma P. Topical nitroglycerine in perniosis/chilblains. Skinmed. 2015;13(3):176-177.

16.       Verma P, Singal A, Yadav P. Perniosis in an infant treated with topical nitroglycerine. Pediatr Dermatol. 2013;30(5):623-624.

17.       Patra AK, Das AL, Ramadasan P. Diltiazem vs. nifedipine in chilblains: a clinical trial. Indian J Dermatol Venereol Leprol. 2003;69(3):209-211.

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