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Chilblains

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Other Names

  • Pernio
  • Cold Sores
  • Idiopathic chilblains
  • Equestrian type chilblain
  • Perniones
  • Chill burns
  • Perniosis

Background

  • This page refers to Chillblains, sometimes called Pernio, superficial, localized inflammatory skin disorder resulting from a maladaptive vascular response to non-freezing cold[1]

History

  • First described by Chipman in 1912[2]
  • Chilblain: derived from Anglo-Saxon expression chil (cold) and -blain (sore) (need citation)
  • Pernio is derived from the latin word Perna, meaning "gammon of bacon" (need citation)

Epidemiology

  • Demographic
    • Most commonly affects children, women less than 40 years old
  • Prevalence
    • Varies between 0.9 per 1,000 and 1.7 per 1,000 in the Netherlands[3]
      • More common among women (0.9 to 2.1) than men (.06) per 1000

Pathophysiology

Typical chilblain violaceous lesions of toes[4]
  • General
    • Maladaptive vascular response to non-freezing cold causes an inflammatory skin disorder
    • Patients present with Lesions painful, itching discoloration and swelling for approximately 24 hours
    • Overall, the condition is poorly understood

Etiology

  • General
    • Etiology is poorly understood
    • Cold-induced vasodilatory reflex: protective physiologic response that intermittently opens blood flow to allow reperfusion and prevent ischemia
    • Hypothesized that cold induced vasospasm becomes dysfunctional, leading to hypoxemia and inflammation
    • Neurovascular instability with inappropriate neural responses to temperature has been proposed[5]
  • Gastrointestinal correlation
    • Can be seen in patients with anorexia, conditions causing weight loss, following bariatric surgery
    • Suggests thermoregulation plays a roll
  • Cold exposure
    • Tend to occur when daily temperatures drop below 12 °C to 15 °C[6]
  • Myelomonocytic leukemia[7]
    • Malignant cells, hypergammaglobulinemia may interfere with microcirculation
    • Subsequent hyperviscoscity, stasis leading to chilblains
  • Equestrian-type
    • Appears on the hips due to prolonged cold exposure, provoked by tight-fitting jeans[8]

Associated Conditions

Typical chilblains edematous and erythematous lesions on toes[4]
  • Secondary Chillblains may be related to:
    • Frostbite
    • Lupus
    • Cold urticaria
    • Acrocyanosis
    • Erythromelalgia
    • Raynaud phenomenon
    • Gangrene
    • Vasculitis
    • Cellulitis
    • Cold panniculitis
    • Cryofibrinogenemia
    • Cold agglutinin disease
    • Sarcoidosis
    • Blue toe syndrome
    • Aicardi-Goutières syndrome
    • Antiphospholipid syndrome

Histopathology

Chilblains violaceous papule and ulceration of 3rd right toe[4]
  • Idiopathic
    • Dermal edema with mixed immune infiltrate invading the papillary and/or reticular dermis
    • Inflammatory cells: mononuclear, mainly lymphocytes
    • Distribution surrounding sweat glands is a hallmark (perieccrine)
    • Spongiosis can be seen in epidermis, may contain necrotic keratoncytes
    • Vascular microthrombi are non-specific
  • Lupus
    • Immunopathology reveals skin deposits of immunoglobulins and complement[9]
    • Abundant dermal interstitial fibrin exudate and mucin is suggestive of lupus pernio.
    • Infiltrate composed of CD3, T cells, CD68+ macrophages, CD20+ B lymphocytes
    • CD123+ cells can be seen in idiopathic and lupus chilblains
  • Equestrian-Type
    • Perivascular and periadnexal, superficial and deep lymphoid cell infiltrate is present[10]
    • Dermal interstitial mucin involvement is common
    • Immunohistology shows CD3+ lymphocytes, few CD20+ cells, small clusters of CD123+

Risk Factors


Differential Diagnosis


Clinical Features

Lupus chilblain erythrocyanotic inflammatory lesions of fingers[4]
  • History
    • Patients most commonly report symptoms in hands, ears, lower legs, feet
    • They may report tingling, numbness, burning parasthesias
    • Pruritis is common
    • They may also endorse skin changes such as redness, swelling
    • Tender blue nodules can develop upon rewarming (lasting days)
  • Physical Exam
    • Uncommonly, blisters, erosions and ulcerations can be seen
  • Special Tests

Evaluation

Childhood pernio erythematous to violaceous edematous lesion on the fingers of a 8 years-old boy[4]

Diagnosis

  • Diagnostic criteria proposed by Mayo Clinic[12]
    • Requires major criteria and at least 1 of 3 minor criteria (see table)
  • Major Criterion
    • Localized erythema and swelling involving acral sites and persistent for > 24 h.
  • Minor Criterion
    • Onset and/or worsening in cooler months (between November and March).
    • Histopathologic findings of skin biopsy consistent with pernio (e.g., dermal edema with superficial and deep perivascular lymphocytic infiltrate) and without findings of lupus erythematous.
    • Response to conservative treatments (i.e., warming and drying of affected areas).

Laboratory

  • Once the diagnosis is made, patient should be screened for underlying autoimmune disease
    • Complete blood count
    • Antinuclear antibodies
    • Complement levels
    • Cold agglutinin
    • Antiphospholipid antibodies
  • Other
    • Cryoglobulin levels do not appear to be associated with pernio[13][14]
    • Childhood pernio may be associated with cryoproteins
    • Consider Rheumatoid Factor

Biopsy

  • General
    • Considered controversial
    • Should be considered in patients who don't meet Mayo clinic criteria to search for other causes

Capillaroscopy

  • Not useful to diagnose pernio, as findings are too non specific
  • May be useful for excluding other conditions (e.g. connective tissue disease)[15]

Classification

  • Not applicable

Management

Main studies regarding chilblains treatment[4]
  • General
    • Overall, treatment remains unsatisfactory
  • Calcium Channel Blockers
    • Reported to be effective, causes peripheral vasodilation
    • Nifedipine is superior to diltiazem[16]
      • Dosed at 20-60 mg 3 times daily
    • Reduces healing time compared to placebo (8 days vs 24 in placebo)[17]
    • Reduced relapses, well tolerated
    • However not all studies confirm efficacy, remains controversial
      • Souwer et all found no difference between nifedipine and placebo for the treatment of chronic chilblains[18]
  • Pentoxifylline
    • Xanthine derivative used to treat muscle pain in people with peripheral artery disease
    • Noaimi et al found 5/9 patients improved compared to 3/11 with oral prednisolone and topical clobetasol[19]
    • Al-Sunday et al had a 110 patient RCT which found pentoxifylline was superior to placebo for therapeutic response, reduced development of new lesions[20]
  • Hydroxychloroquine
    • One small study suggested benefit in 4 of 5 patients[21]
  • Topical Nitroglycerine
    • Has shown promising results in a small trial of 22 patients[22]
  • Topical Steroids
    • Topical betamethasone often used but controversial
    • Souwer et al found no benefit compared to placebo at 6 weeks[23]
    • One small review showed benefit in 6 out of 8 patients[12]
  • Vitamin D
    • There was no benefit from vitamin D3 supplementation on the treatment of chronic chilblains[24]
  • Acupuncture
    • When combined with massage therapy, was found to be an effective treatment[25]
  • Laser Therapy
    • Limited to case reports only
  • Ionizing Radiation + Ultrasound
    • Supported by an Italian paper from 1968[26]
  • Neocutigenol
    • Ointment containing chlorhexidine diacetate, retinol palmitate
    • Suggested benefit, no puplications supporting use

Prevention

  • Avoid cold environments, rapid temperature changes
  • Keep extremities warm, dry
  • Heating sources should be available
  • Smoking cessation
  • Wear appropriate protective clothing (hat, scarf, shoes, gloves, socks)
  • Avoiding tight-fitting socks and shoes

Rehab and Return to Play

Rehabilitation

  • Needs to be updated

Return to Play/ Work

  • Return to play depends on severity of symptoms and sport
    • Typically can occur in days to weeks
  • Athletes competing in cold environments may require
    • Adjustments to clothing
    • Training modification
  • If symptoms are chronic
    • Preventative medications may be indicated

Complications and Prognosis

Prognosis

  • General
    • Tends to resolve spontaneously without treatment

Complications

  • Blisters, erosions and ulcerations
    • Typically self limited
    • Resolve spontaneously in 1 to 3 weeks[27]
  • Recurrence
    • Can occur during future cold exposures

See Also


References

  1. Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008;10(2):128–35.
  2. Chipman ED. Chilblains. Cal State J Med. 1912;10(12):512–3.
  3. Souwer IH, Bor JH, Smits P, Lagro-Janssen AL. Nifedipine vs Placebo for Treatment of Chronic Chilblains: A Randomized Controlled Trial. Ann Fam Med. 2016;14(5):453–9.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 Nyssen, Astrid, et al. "Chilblains." Vasa (2019).
  5. George R, Fulchiero GJ Jr, Marks JG Jr, Clarke JT. Neurovascular instability syndrome: a unifying term to describe the coexistence of temperature-related vascular disorders in affected patients. Arch Dermatol. 2007;143(2):274–5.
  6. Prakash S, Weisman MH. Idiopathic chilblains. Am J Med. 2009;122(12):1152–5.
  7. Nazzaro G, Genovese G, Marzano AV. Idiopathic chilblains in myelomonocytic leukemia: not a simple association. Int J Dermatol. 2018;57(5):596–8.
  8. Weismann K, Larsen FG. Pernio of the hips in young girls wearing tight-fitting jeans with a low waistband. Acta Derm Venereol. 2006;86(6):558–9.
  9. Viguier M, Pinquier L, Cavelier-Balloy B, de la Salmoniere P, Cordoliani F, Flageul 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):180–8.
  10. Yang AY, Schwartz L, Divers AK, Sternberg L, Lee JB. Equestrian chilblain: another outdoor recreational hazard. J Cutan Pathol. 2013;40(5):485–90.
  11. Souwer IH, Smaal D, Bor JH, Knoers N, Lagro-Janssen AL. Phenotypic familial aggregation in chronic chilblains. Fam Pract. 2016;33(5):461–5.
  12. 12.0 12.1 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 Clin Proc. 2014;89(2):207–15.
  13. Yang X, Perez OA, English JC III. Adult perniosis and cryoglobulinemia: a retrospective study and review of the literature. J Am Acad Dermatol. 2010;62(6):e21–e22.
  14. Cohen SJ, Pittelkow MR, Su WP. Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients. J Am Acad Dermatol. 1991;25(1 Pt 1): 21–7.
  15. Ozmen M, Kurtoglu V, Can G, Tarhan EF, Soysal D, Aslan SL. The capillaroscopic findings in idiopathic pernio: is it a microvascular disease? Mod Rheumatol 2013;23(5):897–903.
  16. Patra AK, Das AL, Ramadasan P. Diltiazem vs. nifedipine in chilblains: a clinical trial. Indian J Dermatol Venereol Leprol. 2003;69(3):209–11.
  17. 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;120(2):267–75.
  18. Souwer IH, Bor JH, Smits P, Lagro-Janssen AL. Nifedipine vs Placebo for Treatment of Chronic Chilblains: A Randomized Controlled Trial. Ann Fam Med. 2016;14(5):453–9.
  19. 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):1762–4.
  20. Al-Sudany NK. Treatment of primary perniosis with oral pentoxifylline (a double-blind placebo-controlled randomized therapeutic trial). Dermatol Ther. 2016;29(4):263–8.
  21. Yang X, Perez OA, English JC III. Successful treatment of perniosis with hydroxychloroquine. J Drugs Dermatol. 2010;9 (10): 1242–6.
  22. Verma P. Topical Nitroglycerine in Perniosis/Chilblains. Skinmed. 2015;13(3):176–7.
  23. Souwer IH, Bor JH, Smits P, Lagro-Janssen AL. Assessing the effectiveness of topical betamethasone to treat chronic chilblains: a randomised clinical trial in primary care. Br J Gen Pract. 2017;67(656):e187–e193.
  24. Souwer IH, Lagro-Janssen AL. Vitamin D3 is not effective in the treatment of chronic chilblains. Int J Clin Pract. 2009;63(2): 282–6.
  25. Xiang F, Wang Y, Xiao YB. [Clinical observation on 136 cases of chilblains treated by acupuncture combined with massage]. Zhongguo Zhen Jiu. 2005;25(3):171–2.
  26. Calzavara F, Rossetto S, Scarpis U. Association of ionizing radiations and ultrasonics in the therapy of chilblains. Minerva Radiol. 1968;13(3):162–8.
  27. Vano-Galvan S, Martorell A. Chilblains. CMAJ. 2012;184(1):67.
Created by:
John Kiel on 30 June 2019 22:54:52
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Last edited:
1 April 2022 13:01:09
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