Lupus patients have many more types of rash than the commonly discussed butterfly rash and discoid lupus. After joints, the mucous membranes and rashes are the most commonly affected body parts in systemic lupus erythematosus (SLE).
Understanding these rashes is crucial for proper diagnosis and management. In this article, we will explore the various types of rash in lupus, their characteristics, and how they impact patients.
__________________________________________
NOTE: “The Lupus Encyclopedia” is published by Johns Hopkins Press, a non-profit publishing house whose proceeds go towards important endeavors like the “Muse Project.” Thank you for your support.
__________________________________________
Types of Rash in Lupus Patients: A Guide for Patients
Skin problems in lupus fall into two broad categories: lupus-specific rashes, unique to lupus, known as “cutaneous lupus,” and non-specific rashes found in various conditions, including in lupus patients. About two-thirds of people with lupus develop skin-related symptoms, including rashes. These rashes can be indicators of disease activity and may vary in appearance and severity.
Cutaneous lupus erythematosus (CLE) is categorized into acute, subacute, and chronic forms. When doing a biopsy and examining the skin under a microscope, these three types of rash in lupus have similar features.
If lupus-specific rashes are the only symptom, the diagnosis is “cutaneous lupus erythematosus” rather than SLE.
However, when cutaneous lupus erythematosus occurs alongside other organ involvement, it is considered part of SLE. In other words, the person has SLE and cutaneous lupus is one of the problems they are having from their SLE.
Acute Cutaneous Lupus Erythematosus (ACLE)
ACLE can affect the face, neck, and other areas, with the “butterfly” rash across the cheeks being one of the most common. The rash may be pink (on lighter skin) or dark (on darker skin), often worsened by sun exposure. It typically resolves without scarring but can cause post-inflammatory hyperpigmentation. A rare, severe form, toxic epidermal necrolysis-like ACLE, involves widespread painful blistering and requires urgent hospitalization and immunosuppressive treatments.
Malar Rash (Butterfly Rash)
The malar rash, also known as the butterfly rash, is one of the most recognizable signs of lupus. Though it is one of the best-known types of rash in lupus, it only occurs in one out of every SLE patients.
It appears as a red or purplish rash across the cheeks and the bridge of the nose, resembling the shape of a butterfly. The malar rash usually spares the nasolabial folds (the skin under the nose), which helps differentiate it from other facial rashes, such as rosacea and seborrheic dermatitis.
This rash can be triggered by sun exposure or other environmental factors. It may be accompanied by other lupus symptoms, such as joint pain or fatigue. The malar rash is often a sign of active lupus and can be a key indicator for diagnosis.
Chronic Cutaneous Lupus Erythematosus (CCLE)
CCLE are forms of cutaneous lupus that tend to occur for a long time (chronic) and tend to cause permanent scarring of the skin. Discoid lupus is the most common form of CCLE.
Other variants of CCLE include:
Lupus profundus (lupus panniculitis): Causes tender lumps under the skin that can lead to permanent indentations. It occurs when lupus causes inflammation of the fatty areas under the skin.
Lupus erythematosus tumidus (LET): Appears as swollen, pink-to-dark patches, highly sensitive to UV light, and does not typically progress to SLE (but it rarely can).
Chilblain lupus: Reddish, tender lesions on fingers and toes triggered by cold exposure.
Discoid Lupus Erythematosus Rash
Discoid lupus erythematosus (DLE) is the most common type of chronic cutaneous lupus erythematosus (CCLE). This rash appears as raised, red, and scaly patches that can leave scars. Discoid rashes are usually circular or coin-shaped, hence the name “discoid.” These rashes often occur on sun-exposed areas like the face, ears, and scalp but can also appear on other parts of the body.
Discoid lupus erythematosus (DLE), affects 15% of SLE patients. It is more common in smokers, women, and individuals with darker skin tones.
DLE confined to areas above the neck carries a 10% risk of progressing to SLE, while involvement below the neck increases this to 25%.
DLE lesions can rarely lead to skin cancer, particularly in sun-exposed areas. Diligently using sunscreen on DLE and not smoking reduces the chance for cancer.
Discoid lupus rashes can cause significant scarring and permanent hair loss if they occur on the scalp (scarring alopecia, discussed below). They are usually not as strongly associated with systemic lupus as the malar rash but can still indicate lupus activity, particularly in the skin.
Subacute Cutaneous Lupus Erythematosus (SCLE)
SCLE frequently affects the chest, back, arms, and buttocks in either scaly (psoriasiform) or ring-shaped (annular) patterns. It is highly ultraviolet light-sensitive, often linked to anti-SSA antibodies, and can leave pigment changes. Drug-induced SCLE is common, triggered by medications like proton pump inhibitors, calcium channel blockers, thiazides, and improves with drug discontinuation. SCLE can also rarely signal underlying cancer, especially in smokers and the elderly.
SCLE is strongly associated with sun sensitivity, and patients often find that their rashes worsen with sun exposure. This type of rash can be persistent and may flare up during periods of increased sun exposure. It is important for individuals with SCLE to practice rigorous sun protection to prevent flare-ups.
Photosensitivity and Lupus Rashes
Photosensitivity, or an increased sensitivity to sunlight, is a common feature in lupus. Most lupus patients find that exposure to ultraviolet (UV) light can trigger or worsen their rashes. This photosensitivity can lead to the development of new rashes or exacerbate existing ones.
Photosensitive rashes in lupus often appear on sun-exposed areas of the body, such as the face, neck, and arms. These rashes can vary in appearance, from a mild redness to more severe rashes resembling SCLE or malar rashes. The photosensitivity seen in lupus is not limited to rashes; it can also lead to systemic symptoms, including fatigue and joint pain.
Managing photosensitivity in lupus involves avoiding excessive sun exposure, wearing protective clothing, and using broad-spectrum sunscreens. These measures can help prevent rashes and other lupus-related complications triggered by UV light. Download the free handout on UV light protection on the “Lupus Secrets” page.
Diagnosing and Managing Cutaneous Lupus Erythematosus Rashes
Diagnosing Lupus Rashes
Cutaneous lupus erythematosus is diagnosed via physical examination and sometimes with a skin biopsy. A biopsy reveals characteristic features that confirm cutaneous lupus. Proper diagnosis is crucial to avoid mistaking lupus rashes for other skin conditions, such as rosacea.
Accurately diagnosing the types of rash in lupus is essential for effective treatment. A dermatologist or rheumatologist usually diagnoses lupus rashes based on their appearance, distribution, and any accompanying symptoms. Blood and urine tests may be performed to confirm the diagnosis and assess disease activity.
Treatment Options for Lupus Rashes
Treating lupus rashes involves managing both the skin symptoms and the underlying lupus activity. Common treatments include topical corticosteroids, which reduce inflammation, and antimalarial drugs like hydroxychloroquineand quinacrine, which help control skin and systemic lupus symptoms. For more severe rashes, systemic steroids (like prednisone) or immunosuppressive medications (like methotrexate) may be necessary.
In addition to medical treatments, lifestyle modifications can play a crucial role in managing lupus rashes. Sun protection is paramount, as sun exposure can trigger or worsen rashes. Patients should also avoid triggers such as certain medications or stress, which can lead to lupus flare-ups.
Treatment focuses on controlling inflammation, preventing progression, and addressing cosmetic concerns. Key strategies include:
Topical Treatments:
Small lesions are treated with cortisone creams or injections, or non-steroidal topical agents like pimecrolimus and tacrolimus.
Antimalarials:
Hydroxychloroquine (HCQ) is the first-line systemic treatment, reducing both skin and systemic disease activity. If HCQ is insufficient, additional antimalarials or alternative medications like chloroquine and quinacrine are considered.
Systemic Therapies:
Severe or widespread lesions may require systemic immunosuppressants such as Saphnelo, Benlysta, methotrexateor mycophenolate mofetil. Emerging treatments include anifrolumab (Saphnelo) and belimumab (Benlysta), both of which show promise in improving cutaneous lupus.
Lifestyle Modifications:
Sun Protection: Daily sunscreen use, protective clothing, and limiting UV exposure are essential.
Smoking Cessation: Smoking worsens lupus symptoms and reduces treatment efficacy.
Additional Treatments:
Options for refractory cases include IVIG, retinoids (isotretinoin, acitretin), and biologics like ustekinumab. Severe cases may benefit from lenalidomide, though its use is limited due to significant side effects like nerve damage and blood clots.
Many other types of rash can be seen in lupus. All of these look differently under the microscope than does cutaneous lupus erythematosus. For example, though bullous lupus only occurs in lupus patients (and no one else), it is not considered “cutaneous lupus” since it looks differently under the microscope.
Other rashes in this section can be considered “nonspecific rashes” since they not only occur in lupus patients but also in people who do not have lupus.
Mucosal Ulcers and Mucositis
Mucosal ulcers affect lubricated surfaces like the mouth, nose, and vagina, occurring in up to 45% of SLE patients. These ulcers are often painless. Mouth ulcers (oral ulcers) may spread to the lips in severe cases. Treatments such as antimalarials, belimumab (Benlysta), anifrolumab (Saphnelo), immunosuppressants, and topical steroids can help.
Scarring Alopecia: Permanent hair loss, often from discoid lupus erythematosus (DLE), requires early treatment to prevent progression.
Non-Scarring Alopecia: Temporary hair loss linked to disease activity often regrows with treatment.
Other causes of hair loss include alopecia areata, thyroid disorders, or medications. Dermatological consultation is essential for correct diagnosis and treatment.
Bullous Lupus Erythematosus (BLE)
BLE, a blistering rash unique to SLE, is often linked to kidney inflammation and low blood counts. It is only seen in people who have lupus, but the biopsy appears differently than does cutaneous lupus erythematosus (like the malar rash, discoid lupus, and subacute cutaneous lupus). Dapsone is the primary treatment, with other options available if needed.
Urticaria and Angioedema
SLE-related hives and deeper tissue swelling (angioedema) can occur, often triggered by sun exposure. These typically improve with lupus treatments.
Skin Vasculitis (Cutaneous Vasculitis)
Skin vasculitis in SLE presents as bruised areas, especially on the lower legs. Vasculitis can also cause skin ulcers and red palms. Severe forms may require immunosuppressants. Some types may involve other organs and need further investigation.
Skin Ulcers
Skin ulcers expose underlying tissue and may result from vasculitis or antiphospholipid antibodies. Proper wound care and specialist consultation are essential to prevent complications.
Bruises
Bruising (purpura) can result from low platelets, vasculitis, or medications like prednisone and blood thinners. Lupus can also cause non-specific bruised areas of skin during periods of increased disease activity. Nonessential supplements contributing to bruising should be discontinued.
Livedo Reticularis
This net-like skin discoloration (or mottled skin) is due to reduced blood flow and is sometimes linked to antiphospholipid antibodies. It may indicate a condition called Sneddon syndrome if associated with a stroke.
Telangiectases
Dilated blood vessels causing red spots on the skin are called telangiectasiaand are common in SLE. They are usually permanent and do not improve with treatment.
Periungual Erythema
Reddened skin near fingernails and eyelids can occur due to dilated blood vessels. These changes are generally harmless and resistant to treatment.
Palmar Erythema
Red palms are due to increased blood flow and are not typically problematic. Severe cases may indicate cutaneous vasculitis.
Erythromelalgia
Erythromelalgiacauses red, hot, and painful hands or feet, worsening in warm temperatures. Low-dose aspirin and immunosuppressants are effective treatments.
Erythema Nodosum (EN)
Tender lumps under the skin, often on the shins, result from fat inflammation. EN typically resolves with standard SLE treatments.
Cutaneous Mucinosis
Dark bumps caused by mucin deposits under the skin are rare and usually improve with SLE management.
Subcutaneous Nodules
Firm lumps under the skin, often associated with rheumatoid arthritis, can diminish with effective SLE treatment. Cortisone injections are an option for larger nodules.
Sclerodactyly
Thickened skin on the fingers, often linked to Raynaud’s phenomenon, can progress to hardening (scleroderma). Immunosuppressants may help in the early stages, but advanced cases are resistant to treatment.
Digital Gangrene
Loss of blood flow to fingers or toes can lead to tissue death. Immediate treatment with steroids, blood thinners, and vasodilators is crucial to minimize damage.
Calcinosis Cutis
Calcium deposits under the skin appear as hard lumps, often in pressure-prone areas. Surgical removal is the only effective option in lupus.
Xerosis (Dry Skin)
Dry, itchy skin is prevalent in SLE. Moisturizers and proper skin care are effective remedies.
Neutrophilic Dermatosis
Rare inflammatory skin conditions involving neutrophils include Sweet syndrome and pyoderma gangrenosum. These require immunosuppressive treatment.
Erythema Multiforme (EM)
Target-like lesions are a hallmark of EM, often associated with chilblain lupus. SLE-related EM, known as Rowell syndrome, occurs with anti-SSA antibodies and may need additional management.
Nail Changes
SLE can cause nail abnormalities, including discoloration and ridges. HCQ may darken nails but does not necessarily lead to complications. Dermatological evaluation is recommended to assess severe conditions.
Conclusion
Cutaneous lupus significantly impacts patients’ quality of life, but early diagnosis, effective treatment, and preventive measures can minimize its effects. Collaboration between dermatologists and rheumatologists is vital for optimal management. It is best to ask to see a dermatologist who specializes in “medical dermatology” to get the best care.
By recognizing and addressing the types of rash in lupus, patients and healthcare providers can work together to minimize symptoms and prevent complications.
For more in-depth information on Types of Rash in Lupus: A Comprehensive Guide:
I was diagnosed with sub acute cutaneous lupus rash. Nothing has worked to date. I was on hydroxychoroquine and cortisone ointment for over a year and it did not clear up my rash. i was then prescribed Protopic/Tacromilus. It helped with the pain but did not clear the rash. My Rheumatologist then prescribed Methotrexate but unfortunately, I had a bad liver blood test and it was cancelled. The reason I am writing you is to ask if what has happened to me normal. The reason I was going to be on Methotrexate was because both my hands broke out with blisters. My last count was 69 blisters. On one of my knuckles, I had 3 blisters, one on top of the other which cause a pretty big sore. That sore then went on to create a hole in my knuckle where I could see inside and see my (?) muscle, tendon or a nerve move back and forth. I cannot heal this hole and when I try using polysporin on it and wrapping it up, the hole seems to get bigger. I have an appointment with a Dermatologist on May 12th, 2025 (1 year wait time). I would really like a way to close this hole as it is very painful.
My goodness, May is too long to wait for un open, nonhealing sore! I am not sure how things work in Canada, but if someone told me this here in the US, my advice would be to “call the appointment line every morning and ask, ‘do you have any last minute cancellation slots I can take?’ and patients usually can get in quite quickly as we hate to see open, unused slots.
This sort of case, I’d have to completely get the help of a good medical dermatologist, as you are doing. If it is a blistering lupus rash, dapsone usually works very well. For SCLE that doesn’t respond to HCQ alone, I typically add quinacrine. If the SCLE is part of systemic lupus, I go to anifrolumab (Saphnelo) which typically works amazingly well in most patients.
Good luck, Carole, I hope you can get in faster so you can get the help you need.
Leave a comment