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Purple hands and butterfly rash?
As unusual as they sound, they are actually symptoms of an autoimmune disease called systemic lupus erythematosus (SLE).
SLE is a long-term disease that can affect almost every part and organs in the body. As mentioned, SLE is an autoimmune disease; meaning that your body's immune system (which normally protects the body from infection) attacks your own tissues as though they were ‘bad guys’.
As a result, this can lead to pain, swelling, and damage to organs such as the kidneys and heart. SLE can be so debilitating that it affects one’s quality of life or worse, causes death. Below is a quick glance at what a SLE patient may experience:
Common signs and symptoms of systemic lupus erythematosus. Image via Häggström, Mikael (2014). "Medical gallery of Mikael Häggström 2014". WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.008. ISSN 2002-4436. Public Domain
The exact cause of SLE is poorly understood; it is likely to be linked to genetics but environmental factors are thought to play a role as well. Weirdly enough, SLE seems to overwhelmingly affect women, especially those in childbearing years. It is thought that this is because of the effect of oestrogen hormone in women and the fact that women have more copies of X chromosomes than men. However, it is important to note that SLE that occurs in men is slightly different from that in women, and men tend to suffer more than women if they have SLE.
People with SLE often have disease "flares," in which symptoms worsen, followed by periods of remission, in which symptoms improve. The frequency of these flares varies from person to person. Symptoms are mild in some people and life-threatening in others. However, treatments are available to relieve symptoms, reduce inflammation, and minimize organ damage.
Butterfly rash in SLE. Image via Doktorinternet, CC BY-SA 4.0 , via Wikimedia Commons
Majority of patients with SLE have skin problems at some point. The most common one being butterfly rash, which is the main tell-tale sign of SLE. It appears as redness in butterfly shape over the cheeks and nose after being in the sun. The rash usually lasts only a few days but often comes back.
In addition to that, some people with SLE also develop circular patches of raised, scaly skin (called discoid lupus erythematosus, DLE) which often leave a scar like these:
Patients with SLE are sensitive to UV light from the sun, a condition known as photosensitivity. Such sensitivity can lead to the development of skin rash after exposure to sunlight.
Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Image via https://emedicine.medscape.com/article/332244-overview
If you have SLE, make sure you do the following to minimize or avoid photosensitivity:
Apply sunscreen with a sun-protection factor (SPF) of 50 or greater every day, even if you don't plan to spend a lot of time outdoors. The sunscreen should be applied 20 minutes before going outside and should be reapplied every two hours.
Wear sun protective clothing outdoors.
Avoid sun exposure during the midday (between 10 AM and 4 PM).
Avoid medications that may make photosensitivity worse. Examples are NSAIDs, painkillers (especially piroxicam and ketoprofen), metformin (for diabetes), hydrochlorothiazide (a ‘water pill’) and amiodarone (for irregular heartbeat). However, you should not stop taking any medications without consulting your doctor first. If you’re worried the medications may worsen your SLE, speak to a doctor or pharmacist.
Other than the above preventive strategies, there are various topical treatments (topical means to be applied on the skin) available for managing skin conditions in SLE. They are:
Topical corticosteroids (a.k.a steroid creams/ointments) – Topical corticosteroids come in various types, strengths and potencies. In the case of SLE, more potent topical corticosteroids such as clobetasol propionate may be required. Common side effects of topical steroids are skin thinning and striae.
Topical calcineurin inhibitors – They’re commonly available in pharmacies as pimecrolimus 1% cream and as tacrolimus 0.03% or 0.1% ointment. They typically cause less side effects than topical corticosteroids, but are way more expensive.
Some patients with SLE can develop a phenomenon called Raynaud’s disease, which can cause the fingers and toes to turn white or purple-blue. It’s not as permanent as rashes in SLE, because individuals with SLE only experience it when they have an ‘attack’, which is usually triggered by cold temperatures or emotional stress.
Under normal circumstances, when a healthy person encounters a cold environment, the blood vessels in the skin are capable of ‘vasoconstriction’, i.e. decreases blood flow to the skin. This helps to minimize heat loss from the warm blood and therefore preserve a normal internal or "core" temperature in response to the cold environment.
However, people with Raynaud’s disease have an abnormal or defective vasoconstriction mechanism. As a result, the blood vessels constrict in an exaggerated way in response to cold and emotional or physical stress, leading to a Raynaud’s attack.
Most often, a Raynaud’s attack affects the blood vessels in the fingers. In a typical attack, the fingers (or toes) become suddenly cold as the blood vessels constrict. The skin color changes markedly and may become pale (called a "white attack") or a purple or blue color (called a "blue attack"). It usually begins in a single finger and then spreads to other fingers in both hands. The color changes often affect the whole finger from tip to base. The index, middle, and ring fingers are most commonly involved, while the thumb is often not affected. An attack can cause discomfort, including a "pins and needles" feeling, aching, numbness, or clumsiness of the affected hand(s).
Blood vessels supplying the skin of the ears, nose, face, knees, and nipples can also be affected, and the skin in these areas may become pale or bluish in color after cold exposure. Mottling (a bluish discoloration) of the skin of the arms and legs might also appear. Attacks affecting the toes are also common, although people tend to complain of these less frequently.
There are a few ways to reduce Raynaud’s attack on people who have them.
Avoid sudden cold exposure – Use strategies to keep the whole body warm and avoid rapidly shifting temperature, cold breezes, and damp cold conditions. These include dressing warmly, wearing layer clothing such as thermal underwear, donning a hat, and using mittens or gloves.
Quit smoking – Study has found that SLE patients who quit smoking are less sick and have fewer Raynaud’s attacks. If you’re interested in quitting smoking with the help of medication treatment (known as nicotine replacement therapy), consult a doctor or pharmacist.
Avoid medications that cause vasoconstriction – Examples are pseudoephedrine (for treating cold and allergic symptoms) and ergotamine (for treating migraine). If you have any doubts, talk to a healthcare professional.
Reduce emotional stress
To end a Raynaud’s attack, simply place the hands under warm water or in a warm place (such as in the armpits) or rotate the arms in a whirling windmill pattern. For more severe cases of Raynaud’s attack, doctors may prescribe a class of medication called calcium channel blocker, sildenafil (also used to treat erectile dysfunction in men) and/or nitroglycerins cream.
If you have any questions related to SLE, you can consult our professional doctors and healthcare professionals on Doc2Us. Doc2Us is a mobile application that allows you to talk to a doctor or any healthcare professionals via text chat at any time and from anywhere. For better communication, you can even send our online doctor images or voice messages related to your medical inquiry.
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UpToDate - Initial management of discoid lupus and subacute cutaneous lupus
UpToDate - Photosensitivity disorders (photodermatoses): Clinical manifestations, diagnosis, and treatment
Cover image credit: Profpedia at English Wikipedia, CC BY-SA 4.0 , via Wikimedia Commons
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