Chilblain Lupus

Chilblain lupus is a rare type of lupus that mainly affects the skin of the fingers, toes, ears and nose. It causes red-purple, swollen, painful patches or small raised areas (papules and plaques) on skin that has been exposed to cold and damp weather. It is a form of chronic cutaneous lupus erythematosus, which means the body’s immune system is attacking its own skin over a long time. About 1 in 5 people with chilblain lupus may later develop full-body (systemic) lupus, but many people only ever have the skin form. DermNet®+1

Chilblain lupus (chilblain lupus erythematosus) is a rare skin type of lupus. It causes red-blue, swollen, painful or itchy patches on fingers, toes, heels, ears, or nose after cold and damp exposure. The spots can crack or ulcerate and may take weeks to heal. It is linked to problems in tiny blood vessels and the immune system. Some people with chilblain lupus later develop full (systemic) lupus, so careful follow-up with a rheumatologist or dermatologist is important.PMC+2

In chilblain lupus, cold and moisture trigger tiny blood vessel problems in the skin. These small vessels (microcirculation) become narrow or blocked, blood flow slows down, and the immune system becomes overactive in those areas. This leads to inflammation, swelling, color change, and sometimes sores or cracks. Because it is an autoimmune disease, the condition is not contagious and does not spread from person to person. DermNet®+1

Chilblain lupus often comes and goes in “flares”. Flares usually appear in winter or in cool, wet months and may improve in warmer seasons. With good skin protection from cold, appropriate medicines, and regular follow-up, most people can control their symptoms and reduce long-term damage. Cleveland Clinic+1

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Other names of chilblain lupus

Doctors use several names for the same condition. The most common names are:

• Chilblain lupus erythematosus

• Chilblain lupus erythematosus of Hutchinson

• Chilblain lupus (CHLE)

• Sometimes it is also called lupus pernio in older texts, although that term is now more often used for a different condition related to sarcoidosis, so many doctors avoid this older phrase to prevent confusion. Wikipedia+1

Knowing these names is helpful because different articles, doctors and hospitals may use different terms, but they usually mean the same skin type of lupus linked with cold exposure. PMC+1

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Types of chilblain lupus

Doctors often group chilblain lupus into broad types, based on cause and associated diseases.

1. Sporadic (idiopathic) chilblain lupus – This is the most common type. It happens in people with no known lupus gene mutation and no strong family pattern. Cold and moisture seem to trigger the lesions together with an overactive immune system. DermNet®+1

2. Familial chilblain lupus – This rare form runs in families. It is often linked to changes (mutations) in genes such as TREX1 or sometimes SAMHD1 or the STING pathway. These genes normally help clear extra DNA inside cells and control the body’s “type I interferon” immune response. When they are faulty, the immune system stays switched on and can attack the skin. ResearchGate+3PMC+3Cell+3

3. Chilblain lupus associated with systemic lupus erythematosus (SLE) – In some people, chilblain lupus appears together with full-body lupus. These patients may also have joint pain, kidney problems, fatigue, or other SLE features, and may carry antibodies like ANA and anti-dsDNA. PMC+1

4. Chilblain lupus associated with other cutaneous lupus types – Some people have chilblain lesions plus discoid lupus or other skin lupus patterns elsewhere on the body. The different skin patterns share similar autoimmune mechanisms and often similar antibodies. DermNet®+1

5. Childhood chilblain lupus – Children and teenagers can also develop this condition. Sometimes it is familial and linked to gene changes; sometimes it is sporadic. The disease pattern is similar, but early diagnosis is important to watch for later SLE. Synapse+1

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Causes of chilblain lupus

1. Cold exposure – The most important trigger is exposure to cold air or cold surfaces. Low temperature makes small blood vessels in the fingers, toes, ears and nose tighten (vasoconstriction). This reduces blood flow, causes tiny clots and injury in the skin, and in people with lupus-type immunity the damaged area becomes inflamed and forms chilblain lupus lesions. DermNet®+1

2. Damp or wet environments – Cold plus moisture (rain, snow, wet shoes or gloves) makes heat loss from skin much faster. This extra cooling and swelling of tissues further harms the microcirculation, making chilblain lupus more likely and more severe in winter or in damp climates. DermNet®+1

3. Chronic cutaneous lupus autoimmune activity – Chilblain lupus is a chronic cutaneous lupus variant. The immune system wrongly attacks skin cells and small vessels, leading to long-lasting inflammation in cold-exposed areas. This autoimmune base is the main reason lesions keep coming back over years. PMC+1

4. Systemic lupus erythematosus (SLE) – Some people with SLE develop chilblain lupus as part of their disease spectrum. They already have autoantibodies and an activated immune system, so cold-induced microvascular injury more easily turns into lupus-type skin inflammation. PMC+1

5. Autoantibodies such as ANA and anti-dsDNA – Many patients have antinuclear antibodies (ANA) and sometimes anti-double-stranded DNA (anti-dsDNA) or other lupus antibodies. These antibodies form immune complexes that can deposit in small skin vessels, causing inflammation and damage when blood flow is disturbed by cold. PMC+1

6. Anti-Ro/SSA and related antibodies – Studies show a strong link between chilblain lupus and antibodies to Ro/SSA antigens. These antibodies may directly injure skin cells or increase type I interferon signals, making the skin very sensitive to cold and inflammation. Wikipedia+1

7. Type I interferon pathway over-activation – In familial forms, genes that control the type I interferon response are abnormal. Too much interferon (a strong immune signal) keeps immune cells activated even without infection. This promotes chronic inflammation around blood vessels, especially after cold exposure. ResearchGate+3JAMA Network+3Frontiers+3

8. TREX1 gene mutations – TREX1 is an enzyme that clears extra DNA inside cells. When TREX1 does not work properly, leftover DNA triggers constant antiviral immune signals. This leads to familial chilblain lupus and sometimes other autoimmune signs, such as SLE or brain involvement. PMC+2Cell+2

9. Other interferonopathy genes (SAMHD1, STING) – Mutations in SAMHD1 or gain-of-function changes in STING also cause strong type I interferon activation. These rare genetic problems can present with chilblain-like lesions, showing that faulty nucleic-acid handling and interferon signaling are key causes in some families. ScienceDirect+1

10. Small-vessel blood clots and stasis – Pathology studies show that chilblain lupus skin may have small blood clots, sluggish blood flow and swollen vessel walls. Cold-induced vasospasm and autoimmune attack together lead to this microvascular damage. PMC+1

11. Impaired microcirculation and vasospasm – People with chilblain lupus often have very reactive small blood vessels that narrow too strongly in response to cold. This vasospasm lowers oxygen delivery to skin and makes immune-mediated injury more likely in the affected areas. DermNet®+1

12. Raynaud phenomenon and other cold sensitivity disorders – Some patients have Raynaud-type color changes in their fingers or toes. When Raynaud and lupus coexist, repeated cold-induced vessel spasm can set the stage for chilblain lesions to form in acral skin. DermNet®+1

13. Smoking – Smoking damages blood vessels and reduces oxygen delivery to tissues. In people with lupus, smoking can worsen skin disease and make cold-induced vascular problems more severe, so it is considered a risk factor that can aggravate chilblain lupus. Wikipedia+1

14. Female sex and hormones – Lupus and many autoimmune diseases are more common in women, especially during child-bearing years. Sex hormones like estrogen may affect immune balance and blood vessels, which may partly explain why women are more often affected by chilblain lupus. PMC+1

15. Low body weight or poor insulation – People who are very thin or who do not wear warm clothes or shoes in winter lose heat quickly from hands and feet. This extra heat loss makes cold injury and vasospasm stronger, increasing the chance of chilblain lesions in someone with lupus-type immunity. Cleveland Clinic+1

16. Previous simple chilblains (pernio) – Some patients first have ordinary chilblains without lupus. Over time, if autoimmune antibodies appear, the pattern can shift into true chilblain lupus. Cold injury to vessels may act as a repeated trigger that contributes to this change. MDedge+1

17. Other autoimmune diseases – People with other connective tissue diseases (such as Sjögren syndrome, mixed connective tissue disease) sometimes develop similar acral lesions. Shared autoantibodies and interferon pathways may make chilblain lupus more likely in these groups. Wikipedia+1

18. Infections as triggers – Some case reports suggest that viral infections or immune stimulation can trigger flares in people with genetic or autoimmune risk. Infections may temporarily boost interferon and autoantibody production, which then worsens skin inflammation in cold-exposed areas. PMC+1

19. Certain immune-modulating medicines – Drugs that strongly stimulate or change immune responses can, in rare cases, lead to lupus-like skin reactions. In a person with underlying risk, this might help trigger chilblain-type lesions, although this is not a common cause. PMC+1

20. Genetic and environmental interaction – In many people, no single cause is found. Instead, chilblain lupus seems to result from a mix of inherited immune tendencies, autoantibodies, cold and damp exposure, and vessel reactivity. Together, these factors tip the balance toward chronic skin inflammation in acral areas. DermNet®+2PMC+2

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Symptoms of chilblain lupus

1. Red or purple skin patches on fingers and toes – The most typical sign is red-to-purple, flat or slightly raised patches on the backs or sides of the fingers and toes. These areas often appear or worsen after cold exposure and may last for weeks or months. DermNet®+1

2. Painful swollen plaques – The patches can become swollen plaques that feel tender or painful to touch. Pain may be a burning, throbbing or aching feeling, especially when the area warms up again after being cold. DermNet®+1

3. Itching (pruritus) – Many people feel strong itch in the affected skin before pain appears. The itching is often worse as the lesions first form and may reduce somewhat when the plaques are fully developed. DermNet®+1

4. Papules and nodules – Some lesions are small bumps (papules) or larger lumps (nodules) rather than flat patches. These can have a purple color and may have a slightly rough or hard surface. DermNet®+1

5. Ulcers or open sores – In more severe cases, the center of a plaque or nodule can break down and form a shallow ulcer. These sores can be painful and may get infected if not kept clean and protected. PMC+1

6. Fissures and cracks in skin – Repeated swelling and drying of skin, plus cold injury, can cause small splits or cracks (fissures). These usually appear on fingers, toes, or heels and can bleed or sting, especially when moving. DermNet®+1

7. Color changes with temperature – Skin over the lesions may change color with temperature, for example from red to purple to dusky or blue. Some patients also have Raynaud color changes (white-blue-red) in nearby fingers or toes. DermNet®+1

8. Warmth or cold sensitivity in lesions – The plaques may feel warmer than surrounding skin because of inflammation, or they may feel cold and numb when exposed again to low temperature. Either way, the skin in these areas is very sensitive to temperature change. DermNet®+1

9. Skin thickening and hardening over time – Repeated flares can lead to thicker, harder skin in the affected areas. This chronic change may leave plaques slightly raised and firm even between flares. DermNet®+1

10. Post-inflammatory pigmentation – After lesions heal, the skin often stays darker or sometimes lighter than normal. These color changes can last many months, especially on the knuckles and ears. Synapse+1

11. Lesions on ears, nose, and heels – Although fingers and toes are most common, some people also develop similar plaques on the ears, nose, heels, soles, or lower legs, especially if these areas are exposed to cold and damp. DermNet®+1

12. Nail changes – Rarely, chilblain lupus can affect the nails, leading to redness around the nail folds, nail ridging, or fragile nails. Nailfold changes can also reflect small vessel problems and lupus activity. www.elsevier.com+1

13. Recurrent winter flares – Lesions tend to appear repeatedly in cold seasons and improve or partly clear in warmer months. This yearly pattern is a typical symptom and helps distinguish chilblain lupus from some other skin diseases. DermNet®+1

14. General symptoms when SLE is present – In patients who also have systemic lupus, there may be tiredness, low-grade fever, joint pain, mouth ulcers, hair loss, or chest pain. These symptoms show that lupus is affecting more than just the skin. PMC+1

15. Emotional distress and reduced quality of life – Chronic pain, visible lesions on hands and face, and fear of flares in winter can cause worry, sadness, or embarrassment. This emotional burden is a real symptom that doctors should recognize and help manage. Cleveland Clinic+1

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Diagnostic tests for chilblain lupus

Doctors diagnose chilblain lupus by combining what they see on the skin, what the patient reports, and the results of laboratory and special tests. The Mayo Clinic diagnostic criteria include cold-related acral lesions plus histologic or immunofluorescent evidence of lupus in skin, and the exclusion of other cold-induced diseases. PMC+1

Physical examination tests

1. Visual inspection of skin lesions – The doctor carefully looks at the color, shape, size, and location of each lesion. Typical findings are red or purple plaques and papules on cold-exposed acral areas, sometimes with scaling, fissures or ulceration. The pattern (bilateral, on fingers and toes, worse in winter) strongly supports chilblain lupus. DermNet®+1

2. Palpation for warmth, tenderness and swelling – The doctor gently presses the lesions to feel if they are warm, firm, soft, or swollen. Pain or tenderness and a slightly raised, edematous texture fit with inflammatory plaques seen in chilblain lupus rather than only simple dry skin. DermNet®+1

3. Full skin examination for other lupus lesions – Because chilblain lupus is part of the lupus spectrum, the doctor checks the whole body for discoid lesions, malar rash, photosensitive rashes, or other cutaneous lupus signs. Finding additional lupus-type rashes makes the diagnosis more likely and can point to systemic disease. PMC+1

4. General physical examination for systemic signs – The doctor also checks joints, mouth, hair, chest and abdomen. Joint swelling, mouth ulcers, hair thinning, or signs of organ involvement can indicate systemic lupus along with the acral chilblain lesions. PMC+1

Manual tests

5. Capillary refill time test – The doctor presses on the fingertip or toe until it blanches white, then releases and counts how long it takes to turn pink again. Delayed refill suggests poor microcirculation, which can be part of cold-induced vascular problems seen in chilblain lupus. DermNet®+1

6. Cold exposure and rewarming assessment – In practice, doctors mainly use the history (“Do lesions appear after cold and improve with warmth?”). In some specialized settings, safe, mild cooling and rewarming of the skin may be observed to see how lesions or color changes behave. This helps link symptoms clearly to cold. DermNet®+1

7. Diascopy (glass slide pressure test) – The doctor presses a clear glass slide against the lesion to see if it blanches. In chilblain lupus, some redness may fade (due to blood in vessels), but a purplish or dusky color may remain, suggesting extravascular blood or deep inflammation. This helps distinguish from purely vascular or purpuric lesions. PMC+1

8. Range-of-motion and functional check of fingers and toes – The doctor asks the patient to bend and straighten affected digits. Pain or stiffness may be present due to skin tightness or underlying joint involvement from lupus, and this helps to assess how much daily function is affected. PMC+1

Laboratory and pathological tests

9. Complete blood count (CBC) and inflammatory markers (ESR, CRP) – CBC can show anemia, low or high white cells, or platelet changes that may point to systemic lupus or other diseases. ESR or CRP may be normal or mildly raised; abnormal results help assess overall inflammation and rule out infection. PMC+1

10. Antinuclear antibody (ANA) test – ANA is a screening blood test for autoimmune diseases like lupus. A positive ANA supports an autoimmune cause for the skin lesions and may prompt more detailed antibody testing. However, some chilblain lupus patients may have low or negative ANA, especially in purely cutaneous forms. PMC+1

11. Anti-dsDNA and anti-Sm antibodies – These more specific lupus antibodies are checked when SLE is suspected. Positive results support systemic lupus and may predict organ involvement, so they are important in managing patients who present with chilblain lupus lesions. PMC+1

12. Anti-Ro/SSA and anti-La/SSB antibodies – These antibodies are particularly associated with cutaneous lupus and have been linked to chilblain lupus in several studies. Their presence confirms a strong autoimmune background and can influence monitoring and counseling. Wikipedia+1

13. Complement levels (C3, C4) – Complement proteins can be low when lupus is active, especially in systemic disease. Measuring C3 and C4 helps assess immune complex activity and overall lupus activity in a person with chilblain lesions. PMC+1

14. Cryoglobulin and cold agglutinin tests – These blood tests look for abnormal proteins that clump in the cold and can cause other cold-related skin diseases. Negative cryoglobulin and cold agglutinin studies help fulfill diagnostic criteria for chilblain lupus by ruling out similar conditions like cryoglobulinemic vasculitis. Wikipedia+1

15. Skin biopsy with histology and direct immunofluorescence – A small skin sample from a lesion is examined under the microscope. Typical lupus features include interface dermatitis, perivascular lymphocytic infiltrate, thickened basement membrane, and mucin. Direct immunofluorescence may show deposits of immunoglobulins and complement at the dermo-epidermal junction and around vessels. These findings are central to confirming chilblain lupus. PMC+2SAS Publishers+2

Electrodiagnostic tests

16. Nerve conduction studies (NCS) when neuropathy is suspected – NCS measure how fast and how well electrical signals travel in peripheral nerves. They are not routine for all patients, but in someone with numbness, tingling or suspected small-fiber or large-fiber nerve damage, NCS can show whether an additional neuropathy is present besides the skin lupus. PMC+1

17. Quantitative sensory or thermal threshold testing – Specialized electrodiagnostic methods can test sensitivity to cold and heat. These are mainly research or referral-center tools, but they can document abnormal cold pain or numbness in complex cases and help distinguish neuropathic from purely vascular symptoms. Frontiers+1

Imaging and instrument-based tests

18. Dermoscopy of skin lesions – A handheld dermoscope magnifies the skin and uses polarized light. In chilblain lupus, dermoscopy may show specific vessel patterns and colors that support a diagnosis of inflammatory lupus lesions rather than simple chilblains or vasculitis. ScienceDirect+1

19. Nailfold capillaroscopy (capillary microscopy) – Under magnification (sometimes using videocapillaroscopy), the tiny blood vessels at the base of the nails are examined. Changes such as dilated capillaries, dropouts, or hemorrhages can indicate connective tissue disease and help link chilblain lesions to lupus rather than to primary pernio alone. OUP Academic+1

20. Vascular ultrasound (Doppler) of digital arteries – In difficult cases or research settings, Doppler ultrasound can measure blood flow in small arteries of the hands and feet. It helps assess the degree of vasospasm, vessel narrowing, or other vascular disease contributing to chilblain lesions. While not required for diagnosis, it can help rule out other vascular problems. DermNet®+1

Non-Pharmacological Treatments

Note: These measures support medical treatment. They do not replace prescription medicines when needed.

1. Keeping the body warm
   Staying warm is the single most important step. Wear several thin layers of clothes, warm socks, gloves, hats and scarves. Keep feet and hands dry. This reduces cold-triggered spasm in small blood vessels and lowers the chance of new chilblain lupus lesions.DermNet®+1

2. Avoiding sudden temperature changes
   Rapidly going from very cold to very hot (for example, putting icy hands under very hot water) can damage fragile skin vessels. Slow, gentle warming helps blood flow return without extra injury.NCBI

3. Keeping the home and workplace comfortably warm
   Using safe heating, warm bedding, and draft-proofing can keep skin from repeatedly getting chilled, especially at night and early morning. This lowers flares in cold seasons.DermNet®+1

4. Protecting hands and feet from moisture
   Wet socks or gloves cool the skin very fast. Waterproof shoes and gloves, and changing out of wet clothes quickly, help prevent new painful spots.lupusuk.org.uk+1

5. Gentle regular exercise
   Walking, stretching, and moving fingers and toes help blood flow in small vessels. Exercise also supports heart health and reduces stiffness and fatigue that often come with lupus.NCBI+1

6. Stopping smoking (including vaping and smokeless tobacco)
   Nicotine narrows blood vessels and worsens circulation to the fingers and toes, making chilblain lupus and Raynaud-type attacks more severe. Stopping smoking is one of the most powerful ways to protect the skin and blood vessels.NCBI+1

7. Using gentle, fragrance-free moisturizers
   Regularly applying bland emollients (such as petroleum jelly or simple cream) keeps skin soft and less likely to crack. This also lowers the chance of infection entering through broken skin.lupusuk.org.uk+1

8. Avoiding harsh soaps and irritants
   Strong detergents, alcohol-based hand products, or rough scrubbing can damage the outer skin barrier. Using mild soap and lukewarm water protects fragile lupus skin.lupusuk.org.uk+1

9. Protecting skin from friction and pressure
   Tight shoes, tight rings, or long use of tools that press on fingers can worsen pain and slow healing of lesions. Choosing roomy footwear and taking breaks when using tools reduces mechanical stress.lupusuk.org.uk+1

10. Elevation of swollen limbs
    Gently raising the feet or hands on a pillow can reduce swelling and discomfort in affected areas by helping fluid drain back toward the heart.lupusuk.org.uk+1

11. Warm (not hot) water soaks
    Short soaks of hands or feet in comfortably warm water can ease pain and improve local circulation. Water should never be hot enough to cause redness or burns, because sensation may be reduced.lupusuk.org.uk+1

12. Good wound and ulcer care
    If ulcers form, careful cleaning with gentle solutions, non-stick dressings, and regular dressing changes are important. In some cases, advanced wound care methods like debridement or negative-pressure wound therapy may be used in hospital settings.PMC+2Wikipedia+2

13. Avoiding over-the-counter vasoconstrictor medicines without advice
    Some cold medicines and migraine drugs narrow blood vessels. In people with chilblain lupus or Raynaud-type problems, they can worsen circulation to fingers and toes. Always check with a doctor or pharmacist before using these medicines.NCBI+1

14. Stress management and good sleep
    Stress and poor sleep can worsen immune system imbalance and pain perception. Simple breathing exercises, relaxation apps, and regular sleep routines may reduce flare frequency and improve coping.PMC+1

15. Sun protection
    Many people with cutaneous or systemic lupus are very sensitive to ultraviolet (UV) light. Using broad-spectrum sunscreen, protective clothes, and shade helps prevent other lupus rashes and may reduce overall disease activity.PMC+1

16. Regular skin self-checks
    Checking hands, feet, ears, and nose each day helps you notice new color changes, cracks, or ulcers early. Early reporting to the doctor allows faster treatment and may prevent complications.lupusuk.org.uk+1

17. Foot and hand care by trained professionals
    Podiatrists or specialist nurses can trim nails safely, manage calluses, and give advice on footwear, which helps prevent small injuries that might turn into ulcers.Wikipedia+1

18. Compression or support stockings (only if advised)
    In some people with venous problems or leg swelling, medical-grade stockings can support circulation. They must be properly fitted and should not be used when arterial blood flow is poor unless a specialist recommends them.jvascsurg.org+1

19. Education and support groups
    Learning about chilblain lupus and general lupus, and connecting with support groups, can reduce anxiety and help people follow prevention and treatment plans.Cleveland Clinic+1

20. Regular follow-up with specialists
    Ongoing review by a rheumatologist and/or dermatologist helps detect any move from skin-only disease to systemic lupus, adjust drugs, and monitor for side effects.lupusuk.org.uk+1

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Drug Treatments –

Important safety note: Many of these medicines are strong and may have serious side effects. Doses and timing MUST be chosen by a doctor after checking your full health history. Some uses here are “off-label” (not specifically approved by FDA for chilblain lupus) but are supported by experience in cutaneous or systemic lupus and pernio.

1. Hydroxychloroquine
   Hydroxychloroquine is a disease-modifying antimalarial drug approved for systemic lupus. It helps calm overactive immune cells and can improve many skin lupus rashes, including chilblain lesions, over weeks to months. Typical adult doses are around 200–400 mg per day, adjusted to body weight and eye risk. Side effects can include stomach upset and, rarely, eye toxicity, so regular eye checks are essential.FDA Access Data+2FDA Access Data+2

2. Nifedipine (calcium-channel blocker)
   Nifedipine is a blood pressure medicine that relaxes smooth muscle in blood vessel walls. Doctors often use it off-label to treat severe chilblains and Raynaud attacks by improving blood flow to fingers and toes. Doses and timing vary; extended-release forms are commonly used once or twice daily. Side effects include headache, flushing, ankle swelling, and low blood pressure.FDA Access Data+2NHS Inform+2

3. Topical corticosteroids (e.g., clobetasol, mometasone)
   Strong steroid creams or ointments can reduce local inflammation, redness, and itching in chilblain lupus plaques. They are usually applied in thin layers once or twice daily to affected areas for limited periods. Long-term or improper use can thin the skin and cause stretch marks or visible blood vessels, so supervision is important.lupusuk.org.uk+1

4. Topical calcineurin inhibitors (tacrolimus, pimecrolimus)
   Tacrolimus and pimecrolimus creams modulate immune cells in the skin without being steroids. They are helpful on thin or sensitive areas (like face or fingers) to calm inflammation and reduce lesions. They are usually applied twice daily. Some people notice temporary burning or stinging at the start.PMC+1

5. Systemic corticosteroids (e.g., prednisone)
   In more severe or ulcerating chilblain lupus, short courses of oral steroids may be used to quickly reduce inflammation. Doses and tapering schedules are very individual. Long-term high-dose use has serious risks, such as weight gain, high blood pressure, infections, bone loss, and mood changes, so doctors try to keep the dose as low and short as possible.PMC+1

6. Mycophenolate mofetil
   Mycophenolate suppresses lymphocyte (immune cell) proliferation. It is widely used for systemic lupus, especially lupus nephritis, and sometimes for difficult skin lupus. It is taken by mouth, usually twice daily, with dose slowly increased. Common side effects are stomach upset and increased infection risk; regular blood tests are needed.PMC+1

7. Methotrexate
   Methotrexate interferes with DNA synthesis in rapidly dividing cells, including immune cells. Low weekly doses can help chronic inflammatory skin disease and lupus arthritis, and are sometimes used for resistant chilblain lupus. It is usually taken once weekly, with folic acid to reduce side effects like nausea and mouth ulcers. Blood tests monitor liver and blood counts.PMC+1

8. Azathioprine
   Azathioprine is an immunosuppressant that reduces lymphocyte activity. It can be used as a steroid-sparing drug in lupus with skin and joint involvement. It is taken once or twice daily. Side effects include low blood counts, liver irritation, and higher infection risk, so regular blood monitoring is needed.PMC+1

9. Dapsone
   Dapsone is an anti-inflammatory and antibacterial medicine used in some blistering and vasculitic skin diseases. It can help certain types of cutaneous lupus and pernio-like vasculitis. It is taken once daily, with dose chosen carefully. Side effects include anemia and rare severe reactions, so screening for G6PD deficiency and regular blood tests are essential.PMC

10. Thalidomide (very restricted use)
    Thalidomide strongly dampens inflammatory cytokines and can be effective for severe, treatment-resistant cutaneous lupus, including ulcerating lesions. However, it causes serious birth defects and nerve damage, so it is used only in highly controlled programs, with strict pregnancy prevention and close monitoring.PMC+1

11. Pentoxifylline
    Pentoxifylline improves red blood cell flexibility and micro-circulation. It has been used off-label for ischemic skin problems and pernio. It is taken orally several times per day with food. Common side effects include stomach upset and dizziness; it should be used only under medical supervision.NCBI+1

12. Sildenafil (PDE-5 inhibitor)
    Sildenafil relaxes blood vessels by increasing nitric oxide signaling and is approved for erectile dysfunction and pulmonary hypertension. Off-label, it may help severe Raynaud-type digital ischemia and ulcers by improving blood flow. Doses for vascular problems differ from erectile dysfunction doses and must be doctor-guided. Side effects include headache, flushing, and low blood pressure.Springer+1

13. Bosentan (endothelin-receptor antagonist)
    Bosentan is approved for pulmonary arterial hypertension. It blocks endothelin, a strong vessel-narrowing molecule. Case reports describe benefit in refractory chilblain lupus and digital ulcers, but it has important liver and pregnancy risks and is restricted to specialist use. Dosing is twice daily, with frequent liver blood tests.OUP Academic+2FDA Access Data+2

14. Non-steroidal anti-inflammatory drugs (NSAIDs, e.g., ibuprofen, naproxen)
    NSAIDs relieve pain and reduce mild inflammation in joints and skin. They do not treat the underlying autoimmune cause but can ease discomfort from chilblain lesions. They are used for short periods at the lowest effective dose. Side effects include stomach irritation, kidney strain, and increased bleeding risk.Medical News Today+1

15. Topical nitroglycerin ointment (specialist use)
    Very small amounts of nitroglycerin ointment can be applied near ischemic digits to widen blood vessels and improve circulation in severe Raynaud-like disease. It may cause local irritation and headaches and must be used carefully to avoid low blood pressure.NCBI+1

16. Antibiotics for secondary infection
    If skin cracks or ulcers become infected (more pain, pus, warmth, or fever), doctors may prescribe topical or oral antibiotics such as flucloxacillin or cephalexin, depending on local guidelines. These drugs aim to clear infection and prevent deeper tissue damage. Course length and choice depend on culture results and severity.Cleveland Clinic+1

17. Simple analgesics (paracetamol/acetaminophen)
    Paracetamol does not treat inflammation but helps with pain and fever. It is often chosen because it has fewer stomach and kidney side effects than NSAIDs when used at correct doses. Overdose can damage the liver, so maximum daily dose must not be exceeded.Medical News Today+1

18. Topical anesthetic creams (lidocaine/prilocaine)
    Short-term use of numbing creams on very painful intact skin can ease discomfort from chilblain patches while other treatments take effect. They are applied in thin layers for limited times. Overuse can lead to skin irritation or, rarely, systemic side effects, so instructions must be followed.Wikipedia+1

19. Belimumab (biologic for systemic lupus)
    Belimumab is an antibody that blocks a B-cell survival factor (BLyS). It is FDA-approved for active systemic lupus and lupus nephritis. By reducing autoreactive B cells, it can improve overall lupus control and may indirectly help persistent cutaneous lesions in severe cases, always in combination with standard therapy.FDA Access Data+2FDA Access Data+2

20. Anifrolumab (biologic for moderate–severe SLE)
    Anifrolumab blocks type I interferon signaling, a key pathway in lupus. It is approved for adults with moderate to severe SLE on standard therapy. Better systemic control can allow some skin manifestations, including chilblain-type lesions, to improve. It is given as an IV infusion every 4 weeks under specialist care.FDA Access Data+2PubMed+2

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Dietary Molecular Supplements

Supplements can support general health but do not replace medical treatment. Doses below are general adult ranges from common guidance; your doctor may advise different amounts.

1. Vitamin D
   Many people with cutaneous and systemic lupus have low vitamin D, partly because they avoid sun. Vitamin D helps regulate immune cells and bone health. Doctors often use 600–2000 IU daily, or higher short-term under monitoring, to correct deficiency. Blood tests guide dosing, because too much can harm kidneys and calcium balance.Wiley Online Library+2ScienceDirect+2

2. Omega-3 fatty acids (fish or algal oil)
   Omega-3 fats from fish oil or algae may reduce inflammatory markers and improve some lupus disease scores in studies. Typical doses are around 1–3 g/day of EPA+DHA, taken with meals to reduce stomach upset. They may thin the blood slightly, so they must be used carefully with anticoagulants.ClinicalTrials+2ScienceDirect+2

3. Vitamin C
   Vitamin C supports collagen formation, wound healing, and antioxidant defense. Usual doses are 100–500 mg/day from food and supplements. Very high doses can cause diarrhea and may be risky in kidney disease, so “more” is not always better.PMC+2News-Medical+2

4. Vitamin E
   Vitamin E is an antioxidant that protects cell membranes from oxidative damage. Supplemental doses are often in the range of 100–400 IU/day when used, depending on diet and medical supervision. Very high doses may increase bleeding risk, especially with blood thinners.News-Medical+1

5. Zinc
   Zinc is essential for immune function and skin repair. Moderate supplementation (often 8–15 mg/day in adults when diet is low) can support wound healing and immunity. Too much zinc can actually weaken immunity and cause copper deficiency, so medical guidance is important.PMC+2News-Medical+2

6. Selenium
   Selenium is a trace element in antioxidant enzymes. Typical supplement doses are 50–100 µg/day when needed, considering intake from food. High doses can be toxic, so it should only be used under professional advice.News-Medical+1

7. Curcumin (from turmeric)
   Curcumin has anti-inflammatory and immunomodulatory properties and has been studied in many inflammatory conditions. Common oral doses in studies are about 500–1000 mg/day of standardized extract, often split with meals. It may interact with blood thinners and can cause stomach upset in some people.EatingWell+3PMC+3ScienceDirect+3

8. Probiotics
   Probiotic supplements (for example, Lactobacillus or Bifidobacterium strains) may support gut barrier integrity and immune balance. Doses vary, but many products provide 1–10 billion CFU daily. Evidence in lupus is still emerging, so they should be considered supportive rather than primary treatment.PMC+1

9. Green tea polyphenols (EGCG)
   Green tea contains catechins with antioxidant and mild anti-inflammatory effects. One to three cups of brewed green tea per day or standardized extracts may be used. Concentrated extracts can rarely affect the liver, so medical advice is needed before high-dose use.PMC+1

10. L-arginine (specialist supervision only)
    L-arginine is an amino acid used by the body to make nitric oxide, which widens blood vessels. In some vascular conditions, supplements are studied to improve blood flow. Doses in research vary widely (often grams per day), and it may interact with blood pressure drugs, so it must only be used under specialist care.NCBI+1

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Immunity-Modulating, Regenerative and Stem-Cell-Related Therapies

These are specialist treatments for severe or systemic lupus and are not routine for mild chilblain lupus.

1. Belimumab (Benlysta)
   Belimumab is a monoclonal antibody that blocks a B-cell survival signal (BLyS). It lowers autoreactive B cells and reduces lupus disease activity in many patients. It is given by IV infusion or subcutaneous injection on a fixed schedule, in addition to standard drugs. Side effects include infections and infusion reactions.FDA Access Data+2FDA Access Data+2

2. Anifrolumab (Saphnelo)
   Anifrolumab blocks the type I interferon receptor, a key driver of lupus immune activation. It is approved for moderate–severe SLE not controlled by other medicines. It is given IV every four weeks. It can increase risk of upper respiratory infections and shingles, so vaccination and monitoring are important.FDA Access Data+2PubMed+2

3. Other biologic or targeted therapies (research use)
   Other biologics and small molecules that affect B cells, T cells, or cytokines (for example, rituximab in selected cases) are sometimes used off-label for difficult lupus. They aim to “reset” the immune system more precisely than broad immunosuppressants, but they also carry infection and infusion risks.PubMed+1

4. Hematopoietic stem cell transplantation (HSCT)
   Autologous HSCT uses strong chemotherapy to wipe out the existing immune system and then re-infuses the patient’s own stem cells to rebuild it. It has been tried in very severe, treatment-resistant SLE, with some periods of remission but also significant risk, including infection and treatment-related death. It is reserved for selected cases in expert centers.PMC+2Springer+2

5. Mesenchymal stem cell (MSC) therapy (experimental)
   MSC therapy uses stem cells from bone marrow, umbilical cord, or other tissues to modulate immune responses and promote tissue repair. Studies suggest benefit in some refractory lupus patients, but results are mixed and long-term safety is still being studied. It is currently an experimental option in clinical trials, not routine care.Longdom+3ClinicalTrials+3PMC+3

6. Emerging RNA and cell-based therapies (research only)
   New RNA-engineered T-cell and mRNA-based cell therapies are being studied for lupus. These treatments aim to very precisely target harmful immune cells while sparing healthy ones. At present they are available only in clinical trials and carry unknown long-term risks.Reuters+1

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Surgeries and Procedures

Surgery is rare in chilblain lupus and is used mainly for complications like severe ulcers or threat to fingers or toes.

1. Surgical debridement of ulcers
   In deep or infected ulcers that do not heal with dressings, surgeons may carefully remove dead or infected tissue (debridement). This helps healthy tissue grow and allows dressings or advanced wound therapies to work better. It is usually done with local anesthesia and repeated as needed.PMC+1

2. Skin grafting for non-healing wounds
   If an area of skin is badly damaged and cannot heal on its own, a small piece of healthy skin from another body site can be transplanted to cover the wound. This protects underlying structures and speeds closure. It is used only when blood flow is stable and infection is controlled.Wikipedia+1

3. Digital sympathectomy for critical ischemia
   In extreme cases with severe Raynaud-type ischemia and risk of losing a finger or toe, a hand or vascular surgeon may perform digital periarterial sympathectomy. The outer layer of the artery is stripped to interrupt nerve signals that keep vessels in spasm, improving blood flow and healing.PubMed+2PubMed+2

4. Amputation of dead tissue (very rare, last resort)
   If a finger or toe has irreversible gangrene despite all other measures, limited amputation may be needed to remove dead tissue, relieve pain, and prevent infection spread. The goal is always to save as much tissue and function as possible.MDedge+1

5. Diagnostic skin biopsy
   A small skin sample from a lesion may be taken to confirm chilblain lupus and rule out other vasculitic or lymphoproliferative diseases. The biopsy is done under local anesthetic, and the wound is closed with stitches or left to heal with dressings. Results guide long-term treatment choices.OUP Academic+1

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Prevention Tips

1. Keep your whole body warm and dry in cold, damp weather.

2. Avoid sudden changes between very cold and very hot environments.

3. Stop smoking and nicotine products if you use them.

4. Use gentle daily moisturizers and protect skin from friction and tight shoes or gloves.

5. Protect yourself from sun with clothing and sunscreen to reduce overall lupus activity.

6. Have regular check-ups with a rheumatologist and/or dermatologist.

7. Treat small cuts, cracks, or blisters early with gentle cleaning and dressings.

8. Ask your doctor before taking over-the-counter decongestants, migraine pills, or supplements that might affect blood flow.

9. Keep vaccinations up to date (as advised) to lower infection risk while on immune-modifying drugs.

10. Maintain a balanced diet, regular gentle exercise, and good sleep to support immune balance and healing.lupusuk.org.uk+2PMC+2

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When to See a Doctor

You should see a doctor (and urgently or in emergency care if severe) if:

• Painful red-blue patches appear after cold exposure and do not improve within a few days.

• You notice ulcers, black spots, or open sores on fingers, toes, ears, or nose.

• There are signs of infection: more redness, warmth, pus, bad smell, or fever.

• You develop joint pain, swelling, mouth ulcers, chest pain, shortness of breath, or new fatigue and fevers, which can suggest systemic lupus.

• Medicines cause new problems such as vision changes, severe stomach pain, yellow eyes/skin, shortness of breath, easy bruising, or very low mood.

• Your fingers or toes stay very pale, blue, or black and extremely painful even after warming.

These signs mean you need medical review to adjust treatment and prevent serious complications.Cleveland Clinic+2PMC+2

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What to Eat and What to Avoid

1. Eat: Plenty of vegetables and fruits
   Colorful produce provides vitamins and antioxidants that support immune balance and skin repair.News-Medical+1

2. Eat: Whole grains and high-fiber foods
   Oats, brown rice, and whole-grain bread help steady blood sugar and support heart and gut health.News-Medical

3. Eat: Lean protein (fish, beans, lentils, eggs)
   Protein is needed for wound healing and maintaining muscle strength, especially when illness or steroids are present. Fatty fish also supplies omega-3 fats.ScienceDirect+1

4. Eat: Healthy fats (olive oil, nuts, seeds, avocado)
   These fats help reduce inflammation when they replace saturated and trans fats.ScienceDirect+1

5. Eat: Foods rich in vitamin D and calcium (fortified milk, yogurt, certain fish)
   These support bone health, which is important in lupus, especially if you use steroids and avoid sun.ScienceDirect+1

6. Avoid or limit: Highly processed and fried foods
   These often contain trans fats, excess salt, and sugar that may worsen inflammation, blood pressure, and weight gain.News-Medical+1

7. Avoid or limit: Sugary drinks and sweets
   Too much sugar can worsen weight, diabetes risk, and possibly inflammation, especially with steroid use.News-Medical

8. Avoid or limit: Excess salt
   High salt intake can raise blood pressure and may interact with kidney and heart involvement in lupus.News-Medical+1

9. Avoid or limit: Excess alcohol
   Alcohol can irritate the stomach, interact with many lupus medicines, and strain the liver, especially with methotrexate or acetaminophen.News-Medical+1

10. Avoid or limit: Unsupervised herbal or “immune boosting” products
    Many herbal mixes interact with immunosuppressive or blood-thinning drugs. Always ask your doctor or pharmacist before starting any new product.PMC+2MDPI+2

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Frequently Asked Questions (FAQs)

1. Is chilblain lupus the same as ordinary chilblains?
   No. Ordinary chilblains are cold-induced skin injuries that usually heal quickly. Chilblain lupus has a lupus-related immune cause and often recurs every winter, may last longer, and can be linked to systemic lupus.PMC+1

2. Can chilblain lupus turn into full systemic lupus?
   Yes, some people with chilblain lupus later develop systemic lupus, while others never do. Regular follow-up and blood tests help doctors detect early signs and start treatment if needed.lupusuk.org.uk+1

3. Will my chilblain lupus go away completely?
   For some people, lesions improve or disappear with good cold protection and medicines. Others have repeated flares in cold months. Long-term control is often possible, even if the condition does not fully vanish.lupusuk.org.uk+1

4. Are home remedies alone enough?
   Warm clothes, dry skin, and good wound care are very important, but they are usually not enough for moderate or severe chilblain lupus. Medical assessment is strongly recommended to check for lupus and choose safe treatment.lupusuk.org.uk+1

5. How long do lesions take to heal?
   Simple chilblain lesions may improve within 1–3 weeks if the area is kept warm and dry. In chilblain lupus, lesions may last much longer, especially if ulcers or infection occur.lupusuk.org.uk+1

6. Is hydroxychloroquine safe to use for a long time?
   Hydroxychloroquine is often used for years in lupus and is considered relatively safe when doses are adjusted to body weight and eyes are checked regularly. Eye exams and blood tests help detect rare side effects early.FDA Access Data+1

7. Why do I need eye checks with hydroxychloroquine?
   Very rarely, hydroxychloroquine can damage the retina after long use. Modern screening (eye scans and field tests) can detect early changes and allow dose changes or stopping the drug before serious vision loss.FDA Access Data+1

8. Can I use steroids on my skin every day forever?
   No. Long-term, continuous use of strong steroid creams can thin the skin and cause other problems. Doctors usually limit duration, suggest breaks or milder steroids, or use non-steroid creams like tacrolimus for maintenance.PMC+1

9. Is chilblain lupus contagious?
   No. Chilblain lupus comes from your own immune system and blood vessel reactions. It cannot spread to other people by touch or sharing items.PMC+1

10. Can children or teenagers get chilblain lupus?
    Yes. Chilblain lupus can appear in young people as well as adults. In young patients, doctors may be especially careful about monitoring for systemic lupus and choosing safe long-term medicines.lupusuk.org.uk+1

11. Does diet alone cure chilblain lupus?
    No diet alone can cure lupus. However, a balanced anti-inflammatory pattern (plenty of vegetables, fruits, whole grains, healthy fats, and adequate vitamin D) can support general health and may help your body cope with the disease and medicines.News-Medical+2ScienceDirect+2

12. Are supplements like vitamin D or omega-3 always safe?
    They can be helpful when used correctly, but high doses or mixing with other medicines can cause problems. Blood levels, kidney function, and drug interactions should be considered, so always talk with your doctor before starting or changing supplements.PMC+2ScienceDirect+2

13. Can stress make chilblain lupus worse?
    Stress does not cause lupus, but it can worsen symptoms and make coping harder. Stress can disturb sleep and hormones, which may flare pain and immune activity. Stress-reduction techniques are a helpful part of care.PMC+1

14. Is pregnancy possible if I have lupus with chilblain lesions?
    Many people with lupus have successful pregnancies, but it requires careful planning and specialist care. Some drugs (for example, mycophenolate, methotrexate, thalidomide, bosentan) are unsafe in pregnancy and must be changed well before conception.FDA Access Data+2FDA Access Data+2

15. Who should coordinate my care?
    Usually a rheumatologist (for lupus) and a dermatologist (for skin disease) work together. Your primary care doctor or pediatrician also plays a key role in vaccinations, screening, and everyday health issues. Sharing information between all of them gives the best outcome.Cleveland Clinic+1

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: December 31, 2025.