Chilblains (CHILL-blayns) are the painful inflammation of small blood vessels in your skin that occur in response to repeated exposure to cold but not freezing air. Also known as pernio, chilblains can cause itching, red patches, swelling and blistering on your hands and feet. Chilblains are small lesions caused by the inflammation of tiny blood vessels after exposure to cold air. They’re often painful and tend to affect the skin on your hands and feet. Other names for this condition include pernio, perniosis, and cold-induced vascular disorder. Chilblains are small red itchy patches that can appear on toes and fingers after you’ve been in the cold, particularly in winter. They have a distinctive ‘dusky pink’ appearance and can be very tender and itchy. Sometimes they can look a bit like a bruise and sometimes toes can become quite swollen.
This condition has some similarities to Chilblains in that it is related to restricted blood flow to the extremities. In the case of Raynaud’s, blood flow to the extremities is restricted or interrupted by a constriction of the blood vessels called vasospasm. Triggers for this can be cold or emotional stress.
To ensure treatment, a course of vitamin B, especially nicotinic acid, helps improve circulation and may completely treat the chilblains. Soaking the feet in warm water with Epsom salts for 15 to 20 minutes, three to four times a day will help in thawing the chill
Rewarming affected skin gently, without massaging, rubbing or applying direct heat. Avoid cold exposure whenever possible. Keeping your affected skin dry and warm, but away from sources of heat. Applying lotion to alleviate itching.
Other Names
- Pernio
- Cold Sores
- Idiopathic chilblains
- Equestrian type chilblain
- Perniones
- Chill burns
- Perniosis
Pathophysiology
- 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
Causes
- 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
- 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
- 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
- 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
- Dermal interstitial mucin involvement is common
- Immunohistology shows CD3+ lymphocytes, few CD20+ cells, small clusters of CD123+
- Cold, non-freezing wet weather
- Female gender
- Age young to middle aged
- Low Body Mass Index
- Tobacco Use Disorder
- Family history in chronic cases
- General
- Hypothermia
- Freezing
- Frostbite
- Non-Freezing
- Chilblains (Pernio)
- Cold Induced Urticaria
- Trench Foot
- Cold Injury Mimics
- Pressure Necrosis including Acute Compartment Syndrome
- Cellulitis
Diagnosis
- 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
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 nonspecific
- May be useful for excluding other conditions (e.g. connective tissue disease)[15]
Treatment
- Resist the urge to scratch, as this will further damage the skin.
- Use calamine lotion or witch hazel to soothe the itching.
- Rub Deep Heat or similar onto the feet to promote heart and circulation.
- Wear woolen or cotton socks.
- Keep your whole body warm.
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, which 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
