Henoch-Schönlein purpura (IgA vasculitis)
It nearly always starts the same way: within a few hours, dark red dots and blotches come up on the shins and buttocks, looking like small bruises or insect…
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Medicines commonly prescribed for Henoch-Schönlein purpura (IgA vasculitis)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: ORAL SOLUTION/SUSPENSION, 400 mgActive substance: ibuprofenManufacturer: Ferrer Internacional S.A.Prescription not requiredDosage form: TABLET, 400 mgActive substance: ibuprofenManufacturer: Farmalider S.A.Prescription not requiredDosage form: ORAL SOLUTION/SUSPENSION, 400 mgActive substance: ibuprofenManufacturer: Kern Pharma S.L.Prescription not required
It nearly always starts the same way: within a few hours, dark red dots and blotches come up on the shins and buttocks, looking like small bruises or insect bites, and a week or two earlier the person had an ordinary cold. This is inflammation of the smallest blood vessels in the skin: their walls start to leak blood, which is why the spots do not fade when you press on them. It mostly affects children between three and ten, less often adults, and in adults the illness tends to be more serious. In most people it settles by itself over a few weeks. The catch is that skin vessels are not the only ones inflamed, and that is why this rash is watched for far longer than it lasts.
What comes up on the skin, and what else hurts
The rash is recognisable. It is symmetrical, sits on the shins, feet, buttocks and the outer sides of the arms, and rarely climbs above the waist. The spots stand slightly proud of the skin — you can feel them with your eyes shut. The colour changes like a bruise: purple first, then brownish, then yellowish. New crops arrive in waves for about ten days, sometimes longer; each wave is alarming, but it only means the illness has not finished yet. On brown and black skin the spots are harder to make out, so look at the paler areas: palms, soles and the inside of the eyelids. In children under two, instead of a dense rash there may be swelling of the hands, feet, eyelids and scalp — it looks frightening and usually runs a milder course.
The skin is only the visible part. The same vessels become inflamed in three other places:
- Joints. Knees and ankles ache and swell, elbows and wrists less often. A child limps, sits down on the pavement halfway home, refuses to walk. The joint itself is not damaged: the pain goes without trace within a few days.
- The tummy. Cramping pain around the navel, nausea, vomiting, sometimes blood in the stools. The pain can arrive several days before the rash, in which case it is all put down to a stomach bug or appendicitis and the diagnosis only becomes clear afterwards.
- The kidneys. The quietest part of the illness and the most important. Urine darkens, or blood and protein show up only on testing; blood pressure may rise and the eyelids and ankles swell. The kidneys are not involved on day one: usually in the first weeks, but sometimes a month or two later, when the rash has long gone and everyone has stopped thinking about it.
Boys can develop swelling and pain in the scrotum. This cannot be told apart from a twisted testicle by looking, and with torsion the clock is measured in hours, so that pain always means an urgent examination rather than waiting at home.
Why the vessels start to leak
The fault lies with the immune system. The blood carries an excess of one class of antibody, immunoglobulin A; it clumps into complexes, settles in the walls of small vessels in the skin, bowel, joints and kidneys, and sets off inflammation there. The wall loses its integrity and blood seeps into the tissue. There is no infection inside the vessels themselves.
The trigger is usually a recent infection — a cold, a sore throat, a bout of gastroenteritis. Anything from a few days to three weeks passes between that and the rash, so the connection is easily missed. Less often the illness follows a medicine, an insect bite or a chill. In many people no cause is ever found, which does not mean it was looked for badly: this illness often turns up for no clear reason.
What is not on the list is contagion, or any fault of the parents. The purpura does not spread to others, has nothing to do with care or diet, is not a food allergy and is not inherited. Adults are a separate matter: past fifty, IgA vasculitis is occasionally the first sign of a tumour, which is why an adult is investigated more thoroughly than a child with the same rash. It is uncommon, but it is why the doctor asks questions that seem beside the point.
Signs that cannot wait
Not every rash that fails to fade is Henoch-Schönlein purpura. Meningococcal blood poisoning produces the same picture, and that kills within hours. You can check at home: press the side of a clear glass firmly against the spots and look through it — if the spots are still visible, they are not fading. On its own the sign appears in harmless purpura too; what matters is how the person is otherwise.
Call an ambulance (in Europe, the single number 112) if a rash that does not fade under glass comes with any of the following:
- high fever, shivering, ice-cold hands and feet, grey or mottled skin, drowsiness you cannot rouse them from;
- severe headache, a stiff neck, pain from bright light, vomiting;
- the rash spreading in front of your eyes, with new spots appearing within minutes;
- severe unrelenting tummy pain, repeated vomiting, blood in the stools or black tarry stools;
- a child drawing the legs up to the tummy in bouts, settling in between and lying pale and floppy in the gaps — this is how one length of bowel sliding inside another shows itself, and it is a surgical emergency;
- no urine passed for half a day or more, with obvious swelling of the face and legs.
Do not drive yourself in that situation: nobody can be treated at the wheel. The same day, though without an ambulance, you should be seen if bearable tummy pain appears, if the urine turns the colour of strong tea, if joints swell and hurt, or if the scrotum becomes painful.
What confirms the diagnosis
There is no single test for this illness. The diagnosis rests on the pattern: the typical rash on legs and buttocks plus at least one of three things — tummy pain, joint inflammation, or changes in the urine. Everything else is there to rule out look-alikes and to see whether the kidneys are involved:
- a urine test, the single most important one; it is done on the first day and repeated afterwards, because a dipstick picks up blood and protein long before anything is noticeable;
- a blood pressure reading, in children with a correctly sized cuff;
- a full blood count with a platelet count: here the platelets are normal, and if they are low the rash looks the same but the illness is a different one;
- blood creatinine, which shows how the kidneys are working;
- an abdominal ultrasound scan if there is pain: it shows a thickened bowel wall and one loop of bowel slipping inside another;
- a skin or kidney biopsy — not for everyone, but where the picture is unusual or the kidneys are seriously affected.
What helps while it runs its course
There is no medicine that cuts the vasculitis short, and that is not as bad as it sounds: the body deals with it unaided in most cases, and treatment is there to ease symptoms and to catch complications. What genuinely helps:
- Rest during the worst days. Long periods of standing and walking make the leg rash worse — it really is denser by the evening. This is not dangerous, but a couple of days lying down with the legs raised are worth it.
- Pain relief. Paracetamol is usually enough for joint pain. Anti-inflammatory painkillers such as ibuprofen should not be taken without the doctor's agreement: they add to the load on the kidneys and can worsen bleeding from the bowel, and those are precisely the two organs at stake here.
- A short course of corticosteroids. These are not given to everyone, only for severe tummy pain or badly affected joints; they change neither the rash nor the outlook for the kidneys.
- Hospital admission if the tummy pain is severe, if there is bleeding from the bowel or if the kidneys are seriously involved. Once the kidneys are inflamed a kidney specialist takes over and the treatment is a different order of thing: steroids and drugs that damp down the immune system.
No diet, no fluid restriction and no creams are needed. The illness is not catching: a child goes back to school and an adult back to work as soon as they feel up to it, not after some fixed interval.
The kidneys: why six months of check-ups
This is the main reason a harmless-looking rash is not left unwatched. Kidney inflammation develops in roughly a third of people and is almost always silent: the person feels perfectly well while protein is already leaking into the urine. It can appear months after the recovery.
So urine and blood pressure are checked to a schedule: often in the first weeks, less often later, over six months to a year in all, and for longer if anything turns up. Missed appointments are the commonest mistake in this illness, because by then everything looks finished. In most people the urine settles on its own; in a small minority the inflammation drags on and, untreated, can leave permanent kidney damage — and it is for that minority that the follow-up exists.
Two more things are worth keeping in mind. In about a third the illness returns, usually within the following months and milder than the first time; if the rash comes back, the urine test is repeated. And if the purpura involved the kidneys, mention it to the doctor when planning a pregnancy: blood pressure and urine are then checked more often while she is expecting.
Online consultation
A remote appointment here is not for making the diagnosis but for deciding where to go and how fast. The doctor will ask what the rash looks like and what came before it, ask to see the legs on camera and to do the glass test, judge from your answers whether there are warning signs from the tummy and the kidneys, and set a timescale: emergency, today, or routine. Later, as the illness runs on, it is a convenient way to go through urine results and blood pressure readings, to work out when the next check is due and to settle whether a particular painkiller is allowed. Examining the tummy, checking the scrotum and taking blood cannot be done at a distance, and a rash with high fever, confusion or severe tummy pain is not something to book a consultation for — that is a reason to call an ambulance.
This material is for information only and does not replace medical advice.
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