Chilblain Lupus Erythematosus

Chilblain lupus erythematosus is a rare type of lupus that mainly affects the skin of fingers, toes, heels, ears, and nose. It causes red-purple, swollen, painful patches or small lumps that appear or get worse after exposure to cold and damp weather. DermNet®+1 This disease is a form of “cutaneous lupus”, which means the immune system attacks the skin by mistake. In chilblain lupus, tiny blood vessels in the skin become inflamed and damaged, so blood flow is reduced, and the skin reacts strongly to cold. PMC+1

Chilblain lupus erythematosus is a long-lasting form of cutaneous (skin) lupus where the immune system attacks small blood vessels in the skin, especially of the fingers, toes, ears, or nose. It usually appears after exposure to cold and damp conditions and causes reddish-purple, swollen, painful or itchy patches that can crack or ulcerate. Many patients also have or later develop systemic lupus erythematosus (SLE), so doctors always check for whole-body lupus signs and autoantibodies. Cleveland Clinic+2PMC+2

In CHLE, cold triggers spasm and damage of tiny vessels plus immune complex deposits in the skin. This leads to poor blood flow, inflammation, and tissue injury, which you can see as discolored, tender plaques or nodules, sometimes with crusts or sores. Because sunlight can also worsen cutaneous lupus, doctors often mix cold protection with strong photoprotection in treatment plans. PMC+2PMC+2

Chilblain lupus can exist alone or together with systemic lupus erythematosus (SLE), which is a more general form of lupus that can affect joints, kidneys, blood, lungs, and other organs. People with chilblain lupus need to be checked for signs of systemic lupus over time. Cleveland Clinic+1

There are two main patterns: a sporadic form, which usually appears in adults (often women), and a familial form, which can start in childhood and is linked to changes in a gene called TREX1 that affects how cells clear damaged DNA. DermNet®+1

Other names

Chilblain lupus erythematosus is also called “chilblain lupus”, “CHLE”, “chilblain lupus erythematosus of Hutchinson”, and sometimes “Hutchinson lupus”. Older articles may use “lupus pernio” for the same condition, but today that term is more often used for a skin form of sarcoidosis, so doctors now prefer “chilblain lupus”. DermNet®+1

Types

Doctors group chilblain lupus into a few practical types based on how it appears and why it happens. This helps them guess the risk of internal organ disease and choose how closely to monitor the patient. DermNet®+1

1. Sporadic chilblain lupus erythematosus – This is the most common type. It happens in people with no known family history of the same disease. It usually starts in young or middle-aged adults, more often in women, and is triggered by cold exposure plus an overactive immune system. DermNet®+1

2. Familial chilblain lupus erythematosus – This type runs in families and is often caused by a mutation in the TREX1 gene. It often starts in childhood, and family members may have similar cold-induced sores. Because it is genetic, symptoms may be more persistent and may appear even with mild cold exposure. DermNet®+1

3. Chilblain lupus associated with systemic lupus (SLE) – Some people with SLE develop chilblain-type lesions on top of their usual lupus symptoms such as joint pain, fatigue, or kidney problems. In these people, chilblain lupus is a sign that their immune system and blood vessels in the skin are highly active. PMC+1

4. Chilblain lupus without systemic disease (skin-limited CHLE) – In other people, only the skin is affected and no clear signs of internal organ involvement are found, at least at first. These patients still need follow-up, because a small number may later develop systemic lupus or other autoimmune diseases. PMC+1

Causes

Chilblain lupus is caused by a mix of triggers (such as cold) and internal factors (such as genes and autoantibodies) that together damage small blood vessels and activate the immune system in the skin. PMC+1

1. Cold and damp exposure – The main direct trigger is being in cold, damp conditions. Cold makes small blood vessels in fingers and toes tighten, causing poor blood flow; in chilblain lupus, this leads to strong inflammation and purple sores instead of only mild chilblains. Cleveland Clinic+1

2. Underlying cutaneous lupus erythematosus – Chilblain lupus is a variant of cutaneous lupus, so people who already have lupus affecting the skin are more likely to develop these cold-related lesions on exposed areas. DermNet®+1

3. Systemic lupus erythematosus (SLE) – Many patients with chilblain lupus also have or later develop SLE. The same autoantibodies and immune pathways that drive SLE also make skin vessels sensitive to cold and prone to inflammation. PMC+1

4. Overactive type I interferon pathway – Lupus, including chilblain lupus, often shows high activity of type I interferons, powerful immune messenger proteins that boost inflammation and attract immune cells into the skin, especially around small blood vessels. ScienceDirect+1

5. Anti-Ro/SSA and other autoantibodies – Many patients with chilblain lupus have antibodies such as anti-Ro/SSA that bind to parts of the body’s own cells. These autoantibodies can form immune complexes that deposit in vessel walls and skin, leading to rash and swelling. DermNet®+1

6. TREX1 gene mutation (familial CHLE) – In some families, chilblain lupus is caused by a mutation in the TREX1 gene. This mutation makes it harder for cells to clear damaged DNA, so the immune system sees this DNA as “foreign” and turns on interferon pathways, causing chronic inflammation in cold-exposed skin. DermNet®+1

7. Female sex and hormones – Lupus in general is more common in women, especially during child-bearing years. Female sex hormones are thought to influence the immune system and may help explain why chilblain lupus is reported more often in women. PMC+1

8. Family history of autoimmune disease – Even without a clear TREX1 mutation, people with close relatives who have lupus or other autoimmune diseases have a higher risk, showing the role of shared genes and immune traits in causing chilblain lupus. PMC+1

9. Poor circulation in fingers and toes – Conditions that narrow or damage small blood vessels, such as peripheral vascular disease or chronic smoking-related damage, reduce blood flow and make cold-induced skin injury and chilblain lupus more likely. Mayo Clinic+1

10. Smoking – Smoking constricts blood vessels and promotes clotting and inflammation. In people with lupus or a tendency to chilblains, smoking strongly increases the risk and severity of painful cold-induced lesions. MDedge+1

11. Raynaud’s phenomenon – Some patients with chilblain lupus also have Raynaud’s, where fingers and toes turn white, blue, then red in the cold. Sudden, repeated narrowing of blood vessels in Raynaud’s can further injure the skin and promote chilblain lupus lesions. PMC+1

12. Repeated minor trauma or pressure – Tight shoes, stiff gloves, or repeated friction on cold hands and feet can injure already fragile skin and vessels, so cold-exposed areas are more likely to develop chronic sores of chilblain lupus. DermNet®+1

13. Certain medicines that trigger lupus – A few drugs can trigger or worsen lupus in susceptible people (drug-induced lupus). In someone with cold sensitivity, a drug-related lupus flare may appear as or worsen chilblain lupus lesions. PMC+1

14. Viral or other infections – Infections can activate the immune system and increase interferon levels, which may flare underlying lupus and bring out chilblain-type lesions in people who already have immune and vessel abnormalities. PMC+1

15. Occupational cold exposure – Jobs that require long periods outdoors in cold, damp conditions (such as farming, fishing, or certain construction work) raise the risk that a person with lupus or a genetic tendency will develop chilblain lupus. Mayo Clinic+1

16. Other connective tissue diseases – Mixed connective tissue disease and related illnesses can share immune pathways with lupus and cause similar small-vessel problems, which in some cases present with chilblain-like lesions. PMC+1

17. Low body weight or little body fat – People who are very thin may lose heat quickly from fingers and toes and have less insulation, making them more sensitive to cold and more likely to develop chilblain-type damage. Mayo Clinic+1

18. Tight clothing or footwear in the cold – Tight socks, shoes, or gloves can reduce blood flow in cold conditions. In someone with lupus, this extra reduction in circulation adds to vessel inflammation and increases the chance of chilblain lesions. Mayo Clinic+1

19. Blood-clotting disorders such as antiphospholipid syndrome – Some lupus patients make antibodies that cause tiny clots in small vessels. When combined with cold-induced narrowing, this can produce persistent purple lesions that resemble and overlap with chilblain lupus. PMC+1

20. Small-vessel vasculitis – In some cases, chilblain-like lesions are part of a broader inflammation of small blood vessels (vasculitis). In chilblain lupus, biopsies often show lymphocytes around these vessels, reflecting vessel-focused immune damage in cold-exposed skin. PMC+1

Symptoms

The symptoms of chilblain lupus mainly affect cold-exposed skin, but some people also have general lupus symptoms such as tiredness and joint pain. Cleveland Clinic+1

1. Red or purple skin patches after cold exposure – The main sign is red-violet or dark purple patches or small raised areas (plaques) on fingers, toes, heels, or ears that appear or get worse a few hours after being in the cold. DermNet®+1

2. Swelling of fingers or toes – The affected areas often look swollen and puffy. This swelling is due to leaky and inflamed small blood vessels and can make the skin feel tight and uncomfortable. DermNet®+1

3. Burning or pain in the lesions – Many patients describe burning, aching, or sharp pain in the cold-induced plaques, which may worsen when the hands or feet warm up again, similar to severe chilblains. Cleveland Clinic+1

4. Itching (pruritus) – Itching is common and can be intense. Scratching may break the skin and increase the risk of crusting and infection, so it needs to be controlled with careful skin care and medical treatment. Wikipedia+1

5. Skin tenderness to touch – The lesions are usually tender if pressed or bumped. This tenderness reflects active inflammation in the skin and tiny nerves in the affected area. DermNet®+1

6. Small ulcers or breaks in the skin – In more severe or long-lasting cases, the surface of the plaques can crack or ulcerate, leading to open sores that may bleed or crust and may heal slowly in cold weather. DermNet®+1

7. Scaly or rough skin surface – Some lesions develop a dry, scaly top or thickened, rough skin (hyperkeratosis), especially if they keep coming back over many winters and are not well protected from the cold. Wikipedia+1

8. Color changes with temperature – The same fingers or toes may change from pale or white to blue and then red when moving from cold to warmth. In chilblain lupus, these color changes often overlap with or follow Raynaud’s episodes. PMC+1

9. Numbness or tingling in hands and feet – Damaged small vessels and inflamed skin can irritate or compress small nerves, causing numbness, tingling, or “pins and needles” feelings in affected digits. PMC+1

10. Stiffness of fingers or toes – Pain and swelling around joints can make it hard to move fingers and toes freely, especially when first warming up after cold exposure or in the morning. Cleveland Clinic+1

11. Nail changes – Some patients develop nail ridging, discoloration, or small pits, and the skin around the nails may become red or swollen, reflecting chronic inflammation of the tiny vessels near the nail bed. www.elsevier.com+1

12. Recurrent winter flares – A key feature is that lesions tend to recur each winter or whenever the weather is cold and damp. They may partly fade in warm months but can leave mild discoloration or scarring. DermNet®+1

13. Fatigue and feeling unwell – When chilblain lupus is part of systemic lupus, people may feel tired, feverish, or generally unwell during flares, showing that the immune system is active throughout the body. PMC+1

14. Joint pain or swelling – Joint symptoms in hands, wrists, knees, or other joints can appear along with the skin lesions if systemic lupus is present, reflecting inflammation of the joint lining. Cleveland Clinic+1

15. Other lupus signs (mouth ulcers, hair loss, sun-sensitive rash) – Some patients also notice painless mouth ulcers, hair thinning, or red rashes on sun-exposed skin, which are warning signs of more widespread lupus activity beyond the chilblain lesions. PMC+1

Diagnostic tests

Doctors diagnose chilblain lupus by combining the story (cold-related lesions), the skin exam, blood tests for lupus, and sometimes a skin biopsy. The Mayo Clinic diagnostic criteria and similar sets guide this process. PMC+1

1. General physical examination and medical history – The doctor asks when the lesions appear, how they relate to cold, and whether there are other symptoms like joint pain or mouth ulcers, then checks the whole body for signs of lupus and other diseases. Cleveland Clinic+1

2. Detailed inspection of cold-exposed skin – The skin of fingers, toes, heels, ears, and nose is examined under good light to see the color, shape, borders, and distribution of the lesions, to distinguish chilblain lupus from simple chilblains or frostbite. DermNet®+1

3. Palpation of lesions – The doctor gently presses and feels the plaques to judge warmth, thickness, tenderness, and whether there are nodules or ulceration, which helps assess depth of inflammation and risk of tissue damage. DermNet®+1

4. Joint and musculoskeletal examination – Joints in the hands, wrists, knees, and feet are checked for swelling, pain, and range of movement to look for arthritis or tendon involvement that would support a diagnosis of systemic lupus. PMC+1

5. General examination for systemic lupus signs – The doctor checks for mouth ulcers, hair loss, lymph node swelling, breathing problems, or leg swelling to see if other organs may be affected, which changes the overall diagnosis and treatment plan. PMC+1

6. Capillary refill time test – By pressing a fingertip or toe and watching how quickly color returns, the doctor can check how well small blood vessels are working; delayed refill suggests poor circulation that can worsen chilblain lesions. Mayo Clinic+1

7. Cold stimulation (cold challenge) test – In some cases, a controlled cold test is used, where a finger is briefly exposed to cold and then observed. Strong color change or lesion worsening supports a diagnosis of cold-sensitive vascular disease like chilblain lupus. PMC+1

8. Peripheral pulse palpation – Pulses at the wrist, ankle, and foot are felt to make sure larger arteries are open. Reduced or absent pulses may point to other blood-vessel diseases that can mimic or worsen chilblain lesions. Mayo Clinic+1

9. Nailfold capillary examination with a hand lens – The tiny blood vessels at the base of the nails are inspected with a simple magnifier. Abnormal shapes or patterns can suggest connective tissue disease and support lupus-related small-vessel problems. PMC+1

10. Complete blood count (CBC) – This blood test measures red cells, white cells, and platelets. Abnormal counts (such as anemia or low white cells) can support systemic lupus and may influence how safely certain medicines can be used. PMC+1

11. ESR and C-reactive protein (CRP) – These blood tests measure general inflammation. They may be normal or mildly raised in chilblain lupus but can rise more when there is wider lupus activity or infection in ulcerated lesions. PMC+1

12. Antinuclear antibody (ANA) test – ANA is a key screening test for lupus. A positive ANA suggests an autoimmune process, and its pattern can guide further testing; many patients with chilblain lupus show a positive ANA. PMC+1

13. Anti-Ro/SSA and other autoantibody panel – More specific tests look for anti-Ro/SSA and other extractable nuclear antigens, which are often present in chilblain lupus and help confirm that the lesions are part of the lupus spectrum. DermNet®+1

14. Complement levels (C3 and C4) – Complement proteins are often low when lupus is active, because they are consumed in immune reactions. Low C3 or C4 in a patient with chilblain-type lesions supports the diagnosis of lupus-related disease. PMC+1

15. Basic metabolic panel and urinalysis – Blood and urine tests check kidney function and look for protein or blood in the urine. This helps detect silent lupus kidney disease (lupus nephritis) in patients who present first with skin findings. PMC+1

16. Skin biopsy with routine histology – A small sample of affected skin is removed under local anaesthetic and examined under the microscope. In chilblain lupus, doctors usually see inflammation around blood vessels, thickened basement membrane, and damage to the skin’s junction zone. PMC+1

17. Direct immunofluorescence of skin biopsy – Special stains are used on the biopsy to detect deposits of immunoglobulins and complement along the dermo-epidermal junction (the “lupus band”), which strongly supports the diagnosis of cutaneous lupus, including chilblain lupus. PMC+1

18. Electrocardiogram (ECG) – If systemic lupus is suspected, an ECG can be done to look for heart rhythm or conduction problems caused by inflammation or damage to the heart, helping to assess overall disease impact beyond the skin. PMC+1

19. Nerve conduction studies – In patients with numbness, tingling, or suspected nerve damage in hands and feet, nerve conduction tests can check how well nerves carry signals and distinguish chilblain-related discomfort from true neuropathy linked to lupus. PMC+1

20. Doppler ultrasound and dermoscopy of lesions – Doppler ultrasound can measure blood flow in small vessels of the hands and feet, while dermoscopy (a hand-held skin microscope) allows detailed view of surface vessels and structures, helping to separate chilblain lupus from other vascular and skin conditions. mjrheum.org+1

Non-pharmacological (non-drug) treatments

1. Strict protection from cold

The most basic treatment is to keep hands, feet, ears, and nose warm and dry using gloves, thick socks, hats, and closed shoes. The purpose is to stop blood vessels from tightening suddenly when exposed to cold, which reduces oxygen and worsens lesions. The mechanism is simple: stable warmth lowers vasospasm and improves circulation, so inflammation and skin damage are less likely to flare. PMC+1

2. Avoiding damp and sudden temperature changes

People with CHLE should avoid standing or sitting in cold, wet places and avoid rapid moves from very warm to very cold environments. The purpose is to prevent repeated “shock” to already fragile small vessels. By keeping the environment more stable, the mechanism is reduced vessel constriction and less micro-trauma to the skin, which lowers the chance of new chilblain lupus spots forming. OUP Academic+1

3. Layered clothing and thermal socks/gloves

Using multiple clothing layers traps warm air close to the body and allows easy adjustment if the weather changes. The purpose is better temperature control without overheating. Mechanistically, layered garments act as insulation, keeping skin temperature more constant and lowering the vasospasm that contributes to chilblain and CHLE lesions. PMC+1

4. Gentle regular exercise

Low-impact exercise such as walking, light cycling, or stretching helps the heart pump blood more effectively to fingers and toes. The purpose is to improve overall circulation and vascular health. The mechanism is increased cardiac output and better endothelial function, which can reduce episodes of cold-induced vasospasm and support healing of skin lesions. Dr.Oracle+1

5. Elevating hands and feet when swollen

When lesions are swollen, resting with hands or feet slightly raised on a pillow can help fluid drain. The purpose is to reduce edema and discomfort. Mechanistically, elevation uses gravity to improve venous and lymphatic return, which can ease pressure in small vessels and reduce pain, throbbing, and risk of ulceration. PMC+1

6. Bland emollients and barrier creams

Simple fragrance-free moisturizers or barrier creams (like petrolatum-based products) keep the skin soft and reduce cracking. The purpose is to protect the outer skin barrier and lower infection risk. Mechanistically, emollients reduce transepidermal water loss and micro-fissures, so irritants and microbes cannot enter easily, which supports healing of lupus-damaged skin. PMC+1

7. Good wound care for ulcers

If CHLE lesions break down into sores, careful wound care (gentle cleansing, non-stick dressings, and medical review) is very important. The purpose is to prevent secondary bacterial infection and deeper tissue damage. The mechanism is removal of debris and protection with clean dressings, which lowers bacterial load and gives the immune-damaged tissue a better chance to repair. Cleveland Clinic+1

8. Sun avoidance and high-SPF sunscreen

Although lesions are cold-related, CHLE is a type of cutaneous lupus, and ultraviolet (UV) light can trigger or worsen disease. The purpose of broad-spectrum sunscreen, hats, and shade is to minimize UV-induced immune activation in skin. Mechanistically, less UV exposure reduces DNA damage and autoantigen release that can drive lupus flares. WHO+1

9. Stopping smoking

Smoking strongly harms small blood vessels and lowers oxygen delivery, so it can worsen both chilblains and lupus. The purpose of smoking cessation is to improve micro-circulation and immune balance. Mechanistically, stopping nicotine reduces vasoconstriction, oxidative stress, and endothelial injury, which may decrease frequency and severity of CHLE lesions. PMC+1

10. Avoiding tight shoes and gloves

Tight footwear or gloves can compress already fragile vessels and skin. The purpose of loose, comfortable shoes and socks is to avoid pressure points and friction. Mechanistically, reducing mechanical stress prevents local ischemia and micro-trauma that can turn mild lesions into painful cracks or ulcers. PMC+1

11. Gentle hand and foot massage (with care)

Light massage with warm hands or oil can encourage blood flow, but it must be gentle and not on open sores. The purpose is to support local circulation and relaxation. Mechanistically, soft massage stimulates vasodilation and venous return, which helps warm tissues and may reduce persistent redness and swelling. Dr.Oracle+1

12. Biofeedback or thermal biofeedback training

Some centers teach patients to consciously warm their hands using relaxation and biofeedback devices that show skin temperature. The purpose is to give patients more control over vasospasm. Mechanistically, relaxation and focused attention can decrease sympathetic nervous system activity, allowing small vessels to dilate, which may reduce frequency of cold-induced flares. Dr.Oracle+1

13. Stress-reduction techniques

Chronic stress can worsen autoimmune activity and vasospasm. Practices like deep breathing, meditation, or gentle yoga aim to lower stress levels. The mechanism is reduced stress hormones and sympathetic tone, which can help stabilize immune responses and circulation in people with lupus. BMJ Ard+1

14. Occupational therapy for hand function

Occupational therapists can suggest adaptive tools, splints, and routines to protect the hands while still doing daily tasks. The purpose is to maintain independence and avoid trauma. Mechanistically, using ergonomic tools and splints reduces repeated micro-injury and pressure on affected fingers, giving lesions a better chance to heal. Cleveland Clinic+1

15. Physiotherapy and graded activity for stiffness

Physiotherapists can design gentle exercise plans to keep joints and muscles around affected areas flexible. The purpose is to control stiffness and pain without overloading tissues. Mechanistically, graded activity improves blood flow, muscle strength, and joint range of motion, which may decrease secondary problems like disuse and chronic pain. Australian Prescriber+1

16. Patient education and self-monitoring

Teaching patients to watch for early color changes, new lesions, or signs of infection is a key supportive treatment. The purpose is early detection and fast treatment of flares. Mechanistically, quick response (warming, resting, or contacting the doctor) can prevent a small inflammatory spot from progressing to a deep ulcer. Cleveland Clinic+1

17. Avoiding trauma, scratching, and strong chemicals

People with CHLE should avoid scratching lesions and avoid harsh soaps or chemicals. The purpose is to reduce extra irritation of already inflamed skin. Mechanistically, less mechanical and chemical trauma means fewer breaks in the barrier and lower risk of superinfection and scarring. PMC+1

18. Using mild, pH-balanced cleansers

Gentle, non-soap cleansers help clean the skin without stripping natural oils. The purpose is hygiene with minimal irritation. Mechanistically, pH-balanced products preserve the acid mantle and microbiome, which supports barrier repair in cutaneous lupus lesions. PMC+1

19. Psychological support and counseling

Living with a chronic, visible skin disease can cause anxiety or depression. Counseling or support groups help people cope emotionally. The mechanism is better mental health and adherence to treatment, which can indirectly improve disease control and quality of life. BMJ Ard+1

20. Regular follow-up with dermatology and rheumatology

Scheduled visits allow doctors to adjust treatment, check blood tests, and screen for internal lupus. The purpose is early detection of organ involvement and side effects. Mechanistically, continuous monitoring helps match therapy intensity to disease activity, which is important because CHLE can coexist with or progress toward systemic lupus. PMC+1

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Drug treatments

Very important: These medicines are powerful. Many are immunosuppressants and have serious side effects. Never start, stop, or change dose without your specialist.

1. Topical high-potency corticosteroids (e.g., clobetasol 0.05% ointment)

High-potency steroid ointments are often first-line for localized CHLE lesions. The doctor may ask you to apply a thin layer once or twice daily for a limited time. The purpose is to reduce local inflammation and itching. The mechanism is strong suppression of inflammatory chemicals and immune cells in the skin, helping plaques flatten and redness fade. PMC+1

2. Medium-potency topical steroids (e.g., mometasone 0.1% cream)

On thinner skin or for longer use, doctors may prefer a mid-strength steroid such as mometasone, usually used once daily. The purpose is similar control of inflammation with a lower risk of thinning the skin. The mechanism is moderate glucocorticoid action at the local level, which decreases swelling and pain while trying to minimize adverse effects. WHO+1

3. Topical tacrolimus ointment (Protopic)

Tacrolimus 0.03% or 0.1% ointment is a calcineurin inhibitor that can be used off-label on CHLE lesions, especially on delicate areas where steroids are risky. It is usually applied twice daily. The purpose is steroid-sparing control of inflammation. Mechanistically, tacrolimus blocks calcineurin in T cells, reducing inflammatory cytokine production in the skin. PMC+2FDA Access Data+2

4. Topical pimecrolimus cream (Elidel)

Pimecrolimus 1% cream is another topical calcineurin inhibitor used twice daily after other topicals fail. The purpose is to control redness and itching without causing steroid-induced thinning. Mechanistically, it decreases T-cell activation and inflammatory mediator release in the superficial skin layers. WHO+2FDA Access Data+2

5. Oral hydroxychloroquine (Plaquenil and generics)

Hydroxychloroquine is an antimalarial and disease-modifying drug considered a core treatment for many forms of cutaneous and systemic lupus. Typical adult doses for SLE are 200–400 mg per day, adjusted by weight and eye risk. The purpose is to reduce disease activity, flares, and skin lesions over months. Mechanistically, it interferes with antigen presentation and toll-like receptor signaling in immune cells, dampening autoantibody-driven inflammation. FDA Access Data+3WHO+3FDA Access Data+3

6. Oral chloroquine

Chloroquine is an older antimalarial sometimes used when hydroxychloroquine is not available, but its eye toxicity risk is higher, so many guidelines prefer hydroxychloroquine. Doses and monitoring are carefully tailored. The purpose and mechanism are similar: modulation of lysosomal pH and immune signaling, which reduces lupus skin activity, but safety concerns limit its use. WHO+1

7. Quinacrine (mepacrine)

Quinacrine is a second-line antimalarial used off-label for stubborn cutaneous lupus, often combined with hydroxychloroquine at low doses. The purpose is extra benefit when one antimalarial is not enough. Mechanistically, it also affects lysosomes and DNA-binding of immune complexes, further calming the autoimmune reaction in skin. PMC+1

8. Oral prednisone (systemic corticosteroid)

Prednisone is a strong oral steroid used for short periods when lesions are severe or when there is systemic lupus activity. Doses can range from about 5–60 mg daily depending on severity, then slowly tapered. The purpose is fast control of inflammation. Mechanistically, glucocorticoids suppress many inflammatory genes and immune cell functions but can cause weight gain, infections, osteoporosis, and many other side effects. Australian Prescriber+2FDA Access Data+2

9. Nifedipine (calcium channel blocker)

Nifedipine, an oral calcium-channel blocker, is widely used for cold-induced vasospasm such as perniosis and Raynaud, and can help CHLE by improving blood flow. Doses depend on the formulation (e.g., extended-release tablets once daily). The purpose is to dilate small arteries in fingers and toes. Mechanistically, nifedipine blocks L-type calcium channels in vascular smooth muscle, relaxing the vessel wall and increasing skin perfusion. OUP Academic+2FDA Access Data+2

10. Amlodipine (calcium channel blocker)

Amlodipine is another long-acting calcium channel blocker that some clinicians use similarly to nifedipine in vasospastic disorders. It is usually taken once daily and adjusted for blood pressure and side effects. The purpose and mechanism are to reduce peripheral vascular resistance and improve circulation, which may lessen cold-related skin ischemia. OUP Academic+1

11. Pentoxifylline

Pentoxifylline is a blood “rheology” drug that makes red cells more flexible and reduces blood thickness; it has been used in primary chilblains and other vascular problems. The purpose is to enhance micro-circulation and reduce inflammation. Mechanistically, it improves erythrocyte deformability, decreases blood viscosity, and has mild anti-TNF effects, potentially aiding healing in CHLE. OUP Academic+1

12. Sildenafil (PDE-5 inhibitor, off-label)

Sildenafil is a phosphodiesterase-5 inhibitor used for pulmonary hypertension and erectile dysfunction, and sometimes off-label for severe digital vasospasm. The purpose in this context is to improve blood flow to fingers and toes that do not respond to more standard therapies. Mechanistically, it increases nitric-oxide–mediated vasodilation by preserving cyclic GMP levels. OUP Academic+1

13. Mycophenolate mofetil (CellCept and generics)

Mycophenolate is an oral immunosuppressant often used for systemic lupus, especially kidney disease, and sometimes for refractory cutaneous lupus. Typical doses for SLE are divided twice daily under specialist care. The purpose is to reduce immune overactivity so fewer autoantibodies and inflammatory cells damage blood vessels and skin. Mechanistically, it blocks inosine monophosphate dehydrogenase, which lymphocytes need to make DNA, thus selectively reducing active T and B cells. FDA Access Data+4Lupus+4FDA Access Data+4

14. Methotrexate

Methotrexate, taken once weekly at low doses, is widely used for autoimmune diseases and sometimes for refractory cutaneous lupus. The purpose is steroid-sparing long-term control of inflammation. Mechanistically, low-dose methotrexate affects folate metabolism and increases anti-inflammatory adenosine, leading to reduced activation of T cells and other immune cells, but it requires strict monitoring for liver and blood toxicity. FDA Access Data+3Lupus+3FDA Access Data+3

15. Azathioprine (Imuran)

Azathioprine is another systemic immunosuppressant sometimes used for SLE and cutaneous disease when antimalarials are not enough. It is given orally once or twice daily, with dose adjusted to body weight and TPMT/NUDT15 enzyme status. The purpose is maintenance suppression of autoimmune activity. Mechanistically, it is a purine analogue that interferes with DNA synthesis in proliferating lymphocytes, but it carries risks of bone-marrow suppression and malignancy. FDA Access Data+3Lupus+3FDA Access Data+3

16. Cyclophosphamide

Cyclophosphamide is a strong cytotoxic immunosuppressant used mainly for severe organ-threatening SLE, not for mild skin-only CHLE. It is given as pulses or oral courses in hospital. The purpose is to rapidly control life-threatening autoimmune inflammation. Mechanistically, it cross-links DNA, killing rapidly dividing immune cells, but it has serious toxicities including infection, infertility, and bladder damage, so it is reserved for selected cases. Lupus+1

17. Belimumab (Benlysta)

Belimumab is a monoclonal antibody that targets BLyS (BAFF), a survival factor for B cells, and is FDA-approved as add-on therapy for active autoantibody-positive SLE from age 5 years. It is given by intravenous infusion or subcutaneous injection at specific schedule doses. The purpose is to reduce flares and overall disease activity. Mechanistically, it lowers the number of autoreactive B cells and autoantibodies that contribute to lupus and possibly cutaneous manifestations. Lupus+4FDA Access Data+4FDA Access Data+4

18. Anifrolumab (Saphnelo)

Anifrolumab is a monoclonal antibody against the type I interferon receptor and is FDA-approved for adults with moderate–severe SLE on standard therapy. It is infused intravenously, usually every four weeks. The purpose is to control disease in patients whose lupus remains highly active. Mechanistically, it blocks type I interferon signaling, a key pathway in lupus pathogenesis, thereby reducing downstream inflammatory gene activation. Lupus+3FDA Access Data+3FDA Access Data+3

19. Antibiotics for secondary skin infection

When cracked or ulcerated lesions become infected, doctors may prescribe topical or oral antibiotics such as dicloxacillin or cephalexin, depending on local practice and cultures. The purpose is to clear bacterial infection and prevent deeper tissue involvement. Mechanistically, antibiotics kill or stop growth of bacteria like staphylococci, allowing the immune system and skin to recover. Cleveland Clinic+1

20. NSAIDs (e.g., ibuprofen) for pain and inflammation

Non-steroidal anti-inflammatory drugs like ibuprofen may be used for short-term pain relief and to reduce mild inflammation around lesions, if kidney and stomach status allow. The purpose is symptom control while other disease-modifying drugs take effect. Mechanistically, NSAIDs inhibit cyclo-oxygenase enzymes and lower prostaglandin production, which can reduce pain and swelling but may raise risks of gastric, renal, and cardiovascular side effects. Australian Prescriber+1

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Dietary molecular supplements

These are not cures. They may support general health or inflammation control. Always ask your doctor before starting any supplement, especially if you take immunosuppressants or blood thinners.

1. Vitamin D

Many people with lupus have low vitamin D levels, partly because they avoid sun, and studies suggest that correcting deficiency can improve disease activity and fatigue. Typical supplements may be 800–2000 IU daily, but doses are set by blood tests. The purpose is to support bone health and modulate the immune system. Mechanistically, vitamin D affects T cells and interferon pathways, and higher levels have been linked to lower lupus activity scores in trials. ScienceDirect+4PMC+4MDPI+4

2. Omega-3 fatty acids (fish oil, EPA/DHA)

Omega-3s from fish oil or algae have anti-inflammatory properties and have shown benefits in autoimmune conditions, including reductions in lupus disease activity and inflammatory markers in some studies. Common supplemental doses in research are about 1–3 g of EPA+DHA daily, decided by a doctor. The purpose is to reduce chronic inflammation and possibly improve vascular health. Mechanistically, omega-3s shift eicosanoid production towards less inflammatory mediators and may help resolve inflammation. Frontiers+4PubMed+4MDPI+4

3. Calcium (with vitamin D when needed)

Patients on steroids or with low vitamin D are at high risk of osteoporosis, so calcium plus vitamin D is often advised if diet is insufficient. Doses are usually tailored so total daily calcium intake (food + supplements) is around guideline levels. The purpose is to keep bones strong and prevent fractures. Mechanistically, calcium supports bone mineralization and works with vitamin D to counter steroid-induced bone loss. PMC+2MDPI+2

4. Curcumin (turmeric extract)

Curcumin is a component of turmeric with anti-oxidant and anti-inflammatory effects studied in various inflammatory conditions. Typical supplement doses in studies range from about 500–1000 mg daily of standardized extract, though quality varies. The purpose is to gently reduce inflammation and oxidative stress. Mechanistically, curcumin can affect NF-κB and other inflammatory signaling pathways, which might support background control of autoimmune activity. MDPI+1

5. Selenium

Selenium is an essential trace element that supports antioxidant enzymes and immune function. Some lupus patients may have low intake. Low-dose supplements (for example, 50–100 mcg/day, as decided by clinicians) can correct deficiency. The purpose is to support antioxidant defense and thyroid health. Mechanistically, selenium is needed for glutathione peroxidase and other selenoproteins, which help control oxidative stress linked to vascular and immune damage. MDPI+1

6. Zinc

Zinc is important for wound healing, skin integrity, and normal immune responses, and low intake has been reported in many chronic diseases. Modest supplementation may be considered if laboratory tests show deficiency. The purpose is to support skin repair and balanced immunity. Mechanistically, zinc is a cofactor in DNA synthesis and enzyme systems; deficiency can worsen skin lesions and immunity, so normalizing levels can aid healing. MDPI+1

7. Folate (folic acid)

Patients on methotrexate often take folic acid to reduce side effects like mouth ulcers and liver enzyme rises. Typical doses are once daily except on methotrexate day, but exact regimen is set by the prescriber. Its purpose in this setting is protective rather than disease-modifying. Mechanistically, folate replenishes folate pools partially blocked by methotrexate, improving tolerability while keeping immunomodulatory benefit. FDA Access Data+2FDA Access Data+2

8. Probiotics

Probiotics may help support gut health, which can influence immune function. There is limited but growing research in autoimmune diseases. The purpose is to improve digestive balance and possibly reduce systemic inflammation. Mechanistically, probiotics can modulate the gut microbiome and intestinal barrier, which in turn may affect immune cell activation and cytokine production. PubMed+1

9. Antioxidant-rich polyphenols (e.g., berries, green tea extract)

Instead of high-dose pills, many experts encourage polyphenol-rich foods or moderate supplements (as advised by a doctor). The purpose is to lower oxidative stress that damages vessels and tissues. Mechanistically, polyphenols scavenge free radicals and may modulate signaling pathways involved in inflammation, which is relevant to chronic autoimmune skin disease. PubMed+1

10. Low-glycemic index dietary pattern

Rather than a single supplement, a low-GI eating style acts like a metabolic “molecular” intervention. Studies in lupus show low-GI diets can reduce weight and fatigue. The purpose is to stabilize blood sugar and lower chronic inflammation. Mechanistically, lower post-meal glucose levels reduce oxidative stress and inflammatory markers that can feed into autoimmune pathways. PubMed+1

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Immune-modulating and regenerative / stem-cell-related therapies

These are specialist treatments, often used only in difficult cases or research settings.

1. Belimumab as an immune-modulating “booster in balance”

Belimumab does not “boost” immunity; instead, it balances it by lowering abnormal B-cell activity while preserving much normal defense. Its purpose in resistant lupus is to reduce flares and allow lower steroid doses. Mechanistically, by blocking BLyS, it reduces survival of autoreactive B cells and autoantibody production, which may indirectly help severe or widespread cutaneous lupus including CHLE. Lupus+3FDA Access Data+3FDA Access Data+3

2. Anifrolumab as targeted interferon pathway blockade

Anifrolumab targets the type I interferon receptor and is used for moderate–severe SLE on standard therapy. Its purpose is to control disease when more common drugs are not enough. Mechanistically, it blocks a central lupus pathway, decreasing expression of interferon-stimulated genes, which can reduce skin and systemic inflammation, as shown in clinical trials. Lupus+3FDA Access Data+3JAAD Case Reports+3

3. Low-dose methotrexate as long-term immune remodeling

In low weekly doses, methotrexate acts more as an immune “modulator” than a cytotoxic chemotherapy. The purpose in lupus and cutaneous disease is to gradually reduce abnormal immune activation and need for steroids. Mechanistically, low-dose methotrexate increases anti-inflammatory adenosine and influences T-cell and cytokine profiles over time, which can be seen as slow functional immune remodeling. FDA Access Data+3Lupus+3FDA Access Data+3

4. Mycophenolate as lymphocyte-selective immunosuppression

Mycophenolate selectively blocks a pathway that lymphocytes heavily rely on, so it can be viewed as a “targeted” immune depressor. The purpose is strong disease control with more specificity than some older drugs. Mechanistically, by inhibiting inosine monophosphate dehydrogenase, it restricts DNA synthesis in proliferating T and B cells, which can calm severe skin and organ disease and indirectly give tissues time to repair. FDA Access Data+4Lupus+4FDA Access Data+4

5. Experimental mesenchymal stem cell (MSC) therapy

In some research centers, mesenchymal stem cells from bone marrow or umbilical cord are being studied for severe, refractory SLE. They are given by intravenous infusion at doses and schedules defined in clinical trials. The purpose is to “reset” or calm the immune system and promote tissue repair, but this is not standard care and should only be done in trials. Mechanistically, MSCs release anti-inflammatory cytokines and growth factors and interact with T cells, B cells, and dendritic cells to shift the immune response to a more tolerant state. BMJ Ard+1

6. Autologous hematopoietic stem cell transplantation (HSCT)

For extremely severe, life-threatening autoimmune disease not controlled by any drugs, some centers have used HSCT, where high-dose chemotherapy wipes out most immune cells and then the patient’s own stem cells are reinfused. The purpose is a “deep reset” of the immune system, reserved only for very high-risk cases. Mechanistically, aggressive immunoablation followed by stem cell rescue can lead to re-formation of a less autoreactive immune repertoire, but risks include infection, infertility, and treatment-related death, so it is not routine for CHLE. BMJ Ard+1

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Surgeries and procedures

1. Diagnostic skin biopsy

A punch or excisional biopsy takes a small piece of affected skin under local anesthesia. The purpose is to confirm CHLE and rule out other conditions like vasculitis or cancer. Mechanistically, histology and immunofluorescence show typical lupus changes (interface dermatitis, immune deposits), guiding correct diagnosis and treatment. PMC+1

2. Debridement of necrotic tissue

If ulcers form and part of the tissue dies, surgical or bedside debridement may be performed to remove dead tissue. The purpose is to reduce infection risk and speed healing. Mechanistically, removing necrotic tissue decreases bacterial load and allows healthy granulation tissue and new skin to grow. Cleveland Clinic+1

3. Sympathectomy (very selected cases)

In extremely resistant digital ischemia with repeated ulcers or threatened loss of fingers or toes, surgeons may cut or block parts of the sympathetic nerves to the limb (surgical or chemical sympathectomy). The purpose is to reduce vasospasm and improve blood flow. Mechanistically, interrupting sympathetic signals stops chronic vessel constriction, which can improve perfusion, but this is rare and reserved for severe failure of medical therapy. OUP Academic+1

4. Reconstructive or plastic surgery

After severe or long-standing lesions, some patients are left with scars or deformities that affect function or appearance. Plastic surgery procedures, such as skin grafts or local flaps, may be used. The purpose is to restore skin coverage and improve cosmetic and functional outcome. Mechanistically, healthy tissue is moved to cover damaged areas, providing better blood supply and more normal skin structure. Cleveland Clinic+1

5. Digital amputation (last resort)

In rare, very severe cases with irreversible gangrene of a toe or finger, partial amputation may be needed. The purpose is to remove dead tissue that cannot recover and to prevent life-threatening infection. Mechanistically, surgery excises non-viable tissue and reshapes the area so it can heal and accommodate footwear, but every effort is made to prevent reaching this stage. OUP Academic+1

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Prevention tips

1. Keep extremities warm and dry – Daily use of gloves, warm socks, and proper shoes in cool seasons helps prevent cold-triggered lesions. PMC+1

2. Avoid sudden temperature changes – Try not to go directly from a very hot shower into cold air; gradual adjustment is safer for small vessels. OUP Academic+1

3. Protect from damp – Change wet socks or gloves quickly and avoid staying in cold, humid environments to reduce chilblain formation. PMC+1

4. Use sun protection every day – Daily broad-spectrum SPF 50+, clothing, and hats lower UV-triggered cutaneous lupus flares. WHO+1

5. Do not smoke or vape – Stopping nicotine improves blood flow and may lower overall lupus activity. PMC+1

6. Follow medications exactly as prescribed – Taking hydroxychloroquine and other drugs regularly helps prevent flares and long-term damage. FDA Access Data+2WHO+2

7. Monitor for early changes – Contact your doctor early if you see new discolored or painful spots, especially with cold exposure. Cleveland Clinic+1

8. Maintain a balanced, anti-inflammatory diet – A pattern rich in plants, omega-3s, and adequate vitamin D and calcium may support disease control. PubMed+2MDPI+2

9. Keep regular follow-up appointments – Routine labs and visits allow early adjustment of therapy and monitoring of side effects. Australian Prescriber+1

10. Manage stress and sleep – Good sleep and stress-reduction strategies can help reduce flares and improve quality of life. BMJ Ard+2PubMed+2

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When to see a doctor

You should see a doctor—ideally a dermatologist and rheumatologist—if you develop painful or itchy reddish-purple patches on fingers, toes, ears, or nose that appear after cold exposure and do not go away with simple warming, or if they repeatedly return each winter. Also seek medical care urgently if lesions become very painful, turn black, form deep ulcers, show pus or spreading redness, or if you develop systemic symptoms such as fever, severe fatigue, joint swelling, chest pain, shortness of breath, mouth ulcers, or kidney-related signs like dark or foamy urine. These may suggest systemic lupus or serious complications that need fast specialist assessment and blood tests. Lupus+3Cleveland Clinic+3PMC+3

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What to eat and what to avoid

1. Eat more fruits and vegetables – Colorful produce provides antioxidants and fiber that may help lower inflammation and support gut health. PubMed+2MDPI+2

2. Choose omega-3-rich foods – Fatty fish (like salmon, sardines), flaxseed, and walnuts provide omega-3 fats that support vascular and immune health. PubMed+2ScienceDirect+2

3. Include vitamin-D-rich foods – Fortified milk or plant drinks, eggs, and oily fish help maintain vitamin D along with supplements if prescribed. PMC+2MDPI+2

4. Use whole grains and low-GI carbohydrates – Oats, brown rice, and whole-grain bread help stabilize blood sugar and may reduce fatigue and inflammation. PubMed+1

5. Choose lean proteins – Beans, lentils, tofu, poultry, and fish support muscle repair without too much saturated fat, helpful for heart health in lupus. PubMed+2MDPI+2

6. Limit highly processed and sugary foods – Frequent sugary drinks, sweets, and fast food can increase weight and inflammatory markers, which is unhelpful in autoimmune disease. PubMed+2MDPI+2

7. Reduce very salty foods – High salt intake may worsen blood pressure and possibly influence autoimmune activity, so it is wise to limit very salty snacks and instant foods. MDPI+1

8. Avoid excess alcohol – Alcohol can interact with many lupus medicines and further damage the liver, so intake should be low or zero depending on your doctor’s advice. Australian Prescriber+2PubMed+2

9. Be careful with herbal supplements – Some herbs can stimulate the immune system or interact with drugs; always check with your doctor before using them. PubMed+1

10. Stay hydrated with water – Adequate fluid intake supports kidney function and overall health, especially when some medicines can affect kidneys. Australian Prescriber+2PubMed+2

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Frequently asked questions (FAQs)

1. Is chilblain lupus erythematosus the same as regular chilblains?

No. Regular chilblains (perniosis) happen after cold in people without autoimmune disease, while CHLE is a form of cutaneous lupus with immune-mediated blood-vessel and skin damage. They may look similar, but CHLE often lasts longer and is linked to lupus antibodies. PMC+2PMC+2

2. Can CHLE turn into systemic lupus?

Some patients with CHLE already have systemic lupus or develop it later, while others stay with mainly skin disease. Doctors check blood tests and organs regularly because early detection of systemic lupus allows earlier treatment. PMC+2DermNet®+2

3. Will my lesions go away completely?

Many lesions improve with warmth, topical therapy, and systemic drugs, but CHLE can be chronic and may leave dark marks or scars. The chance of full clearing depends on how active your lupus is, how early treatment starts, and how well triggers are controlled. PMC+2PMC+2

4. Is CHLE contagious?

No. CHLE is an autoimmune condition and cannot be spread from person to person. It is your own immune system reacting against your tissues, not an infection. Cleveland Clinic+1

5. Does everyone with CHLE need strong immunosuppressants?

Not always. Some people respond well to lifestyle changes, topical steroids or calcineurin inhibitors, and hydroxychloroquine. Stronger drugs such as mycophenolate, methotrexate, or biologics are usually reserved for people with severe or resistant disease or with organ involvement. PMC+2PMC+2

6. How long does hydroxychloroquine take to work?

Hydroxychloroquine usually works slowly; patients often need several weeks to months before seeing full benefit in skin and joint symptoms. Because it helps prevent flares and long-term damage, doctors often recommend staying on it long term if tolerated. Lupus+3FDA Access Data+3FDA Access Data+3

7. Why are eye checks important with hydroxychloroquine?

At high cumulative doses or in people with risk factors, hydroxychloroquine can damage the retina. Regular eye exams detect early changes before vision is affected, so the drug can be stopped in time. This balance allows many patients to use its benefits safely. FDA Access Data+2FDA Access Data+2

8. Can diet alone treat CHLE?

Diet can support overall health and maybe reduce inflammation, but it cannot replace medical treatment for CHLE. It works best combined with prescribed medicines, cold protection, and sun safety under specialist guidance. PubMed+2MDPI+2

9. Is it safe to exercise with CHLE?

Yes, most people are encouraged to do gentle regular exercise, as long as they protect their extremities from cold and avoid trauma. Exercise boosts circulation, mood, and heart health, all of which are helpful in lupus. Australian Prescriber+2BMJ Ard+2

10. Can CHLE affect my nails?

Yes. Some patients develop nail fold changes or nail dystrophy due to chronic vascular and immune damage around the nail matrix. This is another reason dermatologists may examine nails and sometimes biopsy near that area. scielo.org.co+1

11. Will I always have to avoid the sun?

People with cutaneous lupus, including CHLE, are usually advised to be careful with sun for life, especially midday and intense UV. However, with good photoprotection and disease control, many can still enjoy outdoor activities at safer times or with proper clothing and sunscreen. WHO+1

12. Are biologic drugs like belimumab or anifrolumab safe for teenagers?

Belimumab is approved for SLE in patients 5 years and older in some regions, while anifrolumab is approved for adults. Safety and use in young people depend on local rules and specialist judgment. A pediatric rheumatologist weighs benefits and risks carefully for each person. FDA Access Data+3FDA Access Data+3FDA Access Data+3

13. Can CHLE damage internal organs by itself?

CHLE mainly affects the skin and small vessels in the skin. Internal organ damage usually comes from systemic lupus rather than CHLE alone, which is why doctors evaluate you for systemic signs and antibodies when CHLE is diagnosed. PMC+2DermNet®+2

14. Will my children definitely get lupus if I have CHLE?

No. Lupus is influenced by genes plus environment, so relatives have higher risk than the general population, but most family members never develop lupus. There is no simple way to predict it for a child, and regular healthy lifestyle and medical check-ups are sensible. BMJ Ard+1

15. What is the most important thing I can do every day?

The most important daily actions are usually: keep warm and dry, avoid sun without protection, take your medicines exactly as prescribed, and stay in touch with your doctors if you notice changes. These simple, regular steps, along with a healthy lifestyle, often make the biggest difference over time. EatingWell+3PMC+3WHO+3

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.