anyone else with Hairy leukoplakia and odd bubble blisters
HI I posted awhile ago about my 9 y/o daughter Sammi who has been diagnosed by a subspecialty research immunologist with a primary immune dysfunction,(a signaling disorder where she does not signal her immune system to fight bacterial or yeast infections )and her immunologist has told us she is developing a secondary autoimmune disorder as I understand because of the primary immune issue. Anyway she had an ANA of 1:1,280, a sed rate of 30 and multiple migratory aches pains, hips, knees, ankles, headaches, dizzy spells, hairy leukoplakia, apthous oral and nasal ulcers and an odd vasculitic like rash below her elbows. As the remainder of the blood work for all the fancy lupus markers were negative and kidneys not yet apparently effected, no butterfly rash etc..her rheumatologist has called it an "autoimmune process we need ot allow to evolve". The immuno did research lab blood work and said her interferon alpha test results are consistent with those seen in lupus patients, the hairy leukoplakia, GI issues with esophagitis, gastritis, duodenitis, colitis and other issues all show inflammation and are she feels signs of atypical lupus. She has started her on low dose Kineret injections a short acting immune suppressant. That we just started two days ago. In tha last two months I have seen her have a new sort of "ulcer" form in her mouth that looks like a fluid filled odd blister near her jaw /gum on her cheek mucosa. The day after they appeared they "popped" and became mean red irregular looking ulcers, Has anyone here ever seen this? Has anyone else used Kineret injections? Any suggestions how to reduce the pain of the shots? We are using Elecon and EMLA but she gets very red, warm and bruises at the injection site as soon as I give it and cries for about 20 minutes, makes me feel like a heel.. Just hoping to find someone with similar experiences and support.. Thanks.
Lee Anne, mom of four, all with some chronic health issues:
Lauren, 23, college senior, classical clarinet performance and music ed major with one semester to go! who has; asthma, enivro allergies, IBS, GERD, visual impairment,( 5 eye surgeries) otosclerosis with hearing loss, recovering post late stage neurological lyme infection
Amanda, 13, budding young artist, sweet 7th grader who has;hypothyroidism, peripheral neuropathy, psoriasis,enthesitis, psoriatic arthritis, chondromalacia patellae,metabolic bone disorder with spondylolisthesis, hyper mobile joints, osteoporosis, hx multiple fractures, one year of IV Pamidronate.z score up from -3.0 to -1.0, GERD, constipation, EE, asthma, IEP "other health impaired" accommodations in school, Synthroid 75 mcg, FloVent, Prilosec, Zantac, reglan, Indocin, prednisone, methotrexate weekly sub Q injections, Citracal, Elavil, Zyrtec, xoponex .
Matthew, 11, naturalist, animal lover, math wizard and book worm who has: multiple anaphylactic food allergies, antibiotic allergies, environmental allergies, severe asthma, EG, EC, GERD, metabolic bone disorder, osteoporosis, hyper mobile joints, mild scoliosis, migraine, simple partial seizures..Prevacid, reglan, AdVair 500/50, albuterol MDI, Zyrtec 10, Singulair 10, FloNase, Keppra, Elavil, TOBI 300 for asthma flares, Xoponex and Atrovent..for asthma flares. No dairy, no legumes(peas, chick peas, kidney beans etc...), no peanuts, no tree nuts, no cantaloupe, IEP home tutored
Samantha,9 y/o, bright, loving, independent, wise and spiritual little girl, who has severe GERD, intestinal dysmotility/ pseudo obstruction, GJ tube for J feeding, small bowel bacterial overgrowth syndrome, asthma, mild allergies, metabolic bone disorder, osteoporosis, hyper mobile joints, history of multiple fractures minimal trauma, atypical Lupus, allergic to Propofol and Versed. on reglan, Nexium, Zelnorm, Erythromycin for motility, Miralax, Zyrtec,Elavil, alternating abx for overgrowth, Pentasa, Keneret
Oral hairy leukoplakia (OHL) is an oral mucosal disease that is associated with Epstein-Barr virus (EBV) and occurs almost exclusively in patients who are immunocompromised (like Lupus patients). It is not dangerous in itself, However, it is a generally taken as a sign that the immune system is weakening and that the person may be at increased risk of other, more serious infections.
Earlier, the presence of OHL suggested a diagnosis of HIV, but recently, reports have described OHL in immunosuppressed persons with disorders other than HIV, including solid organ and hematopoietic cell transplantation, systemic lupus erythematosus, Behçet's disease, and immune suppression secondary to high-dose oral corticosteroids.
Oral hairy leukoplakia causes irregular white patches on the side of the tongue and occasionally elsewhere on the tongue or in the mouth. The patches look corrugated or folded in appearance with tiny hair-like protrusions running along the folds; these can be seen best when the tongue is stuck out and stretched to one side.
The patches look quite similar to candida (thrush), except hairy leukoplakia lesions cannot be moved or dislodged. Hairy leukoplakia usually responds to high doses (3 grams/day) of antiviral treatments.
Interleukin-1 (IL-1) is a cytokine that is secreted by macrophages (Macrophages play an important role in killing of some bacteria, protozoa and tumour cells, release substances that stimulate other cells of the immune system and are involved in antigen presentation. May further differentiate within chronic inflammatory lesions to epithelioid cells or may fuse to form foreign body giant cells or Langhans giant cells), monocytes and dendritic cells. It is an important part of the inflammatory response of the body against infection. It increases the expression of adhesion factors on endothelial cells to enable transmigration of leukocytes, the cells that fight pathogens, to sites of infection. It also re-sets the hypothalamus thermoregulatory center, leading to an increased body temperature which expresses itself as fever. It is therefore called an endogenous pyrogen. The increased body temperature helps the body's immune system to fight infection.
Anakinra (Kineret) is an IL-1 receptor antagonist. Kineret blocks the biologic activity of naturally occuring IL-1, including inflammation and cartilage degradation associated with rheumatoid arthritis and Lupus, by c inhibiting the binding of IL-1 to the Interleukin-1 type receptor, which is expressed in many tissues and organs. IL-1 is produced in response to inflammatory stimuli and lessens various physiologic responses, including inflammatory and immunologic reactions.
I hope that this has been helpful
Saysusie; OHL, Kineret etc..
Thank you for your response! It seems my dear Samantha is putting her cart before her horse and giving us all sorts of clues but does not meet her rheumatologists "clinical diagnosis" criteria for SLE as yet. Her research immunologist has explained, as best we can understand it, that Sammi has a immune dysfunction/deficiency and that it is triggering the autoimmune disease. She has told us if we wait until the inflammation and damage have caused enough damage to qualify for a clinical diagnosis of SLE in her experience those patients who have primary immune dysfunction who go on to manifest full diagnostic symptoms of autoimmune disease or SLE are more difficult to manage and that it is wisest to treat the inflammation sooner. I have a feeling we have found an extremely bright and most likely cutting edge doctor to treat our daughter,I just wish, as I often do, that things could be a bit clearer. I am afriad it never seems to work that way.
Has anyone else here dealt with oral hairy leukoplakia? Are there any other parents on the list with pre teen children who have SLE or atypical SLE? The Kineret seems to be a good choice to treat Sammi as it seems to be addressing the inflammation at its source. Has anyone here used it? I am finding it hurts like blazes. My Sammi has been through a lot and lives with a GJ tube for tube feeding, has had a port a cath for almost a year in 2002 and was on IV nutrition (TPN) and has had several procedures, she is not normally a whiney or teary kid, and complains little, but the Kineret almost instantly causes the injection site to be red and hot to touch and she tells us feels like she is being stabbed even with EMLA and Elecon cream. Sure would like a trick to make it hurt less.
Thanks again for your response,
LEE ANN, YOU HAVE YOUR HANDS FULL!
The fact that all of your kids have such significant health problems certainly suggests some genetic or environmental factor at work. Have you ever consulted a genetics specialist or been screened for exposure to heavy metals or other environmental toxins?
If your daughter has a primary immune system dysfunction, is your doctor worried that giving her immuno-suppresants such as Kineret will make her even more vulnerable to infections? Since your daughter is school age, you might want to talk to her teachers about making sure she isn't exposed to chickenpox or measles, which could be life threatening for her. Has your doctor ever discussed possibility of IVIG infusions to increase her immunity to infections?
I'm sorry the Kineret injections are so painful for her. Have you had someone check your injection technique to make sure you are giving meds sub-q and not intradermal, which is very painful? If you are rotating injections sites every time and using good injection techniques, your daughter may be sensitive to the preservatives or other components of the drug. Are you letting drug come completely to room temp before injecting? Also, use alcohol wipe and let dry completely before applying EMLA cream, and allowing plenty of time for EMLA to work? There is also a product called Numbee which is good for children. Putting ice on injection site immediately after may reduce pain. There is also a product called arnica, available in cream or gel in many drugstores, which reduces bruising and pain. Vitamin K cream also helps reduce pain.
Are you using pre-filled syringes, or drawing the meds up yourself? If there is any way to use a smaller gauge needle, it could reduce pain. You might also talk to your doc or pharmacist about whether this drug can be given with a mechanical injection device, which is often less painful. I would definitely talk to her doc to find out if there is a less painful alternative. Good luck - I'll be thinking of you and your family.
P.S. - mouth ulcers
are really common with lupus. There's a concoction called miracle mouthwash your doc can prescribe that helps. Also, plain OTC liquid mylanta antacid - apply to ulcers with a cotton swab and let dry. you want to stay away from mouthwashes or toothpastes which contain alcohol - it can make the ulcers worse. If your daughter likes yogurt, eating the kind with active yogurt cultures every day can reduce chances of getting oral thrush while taking kineret.
Thank you for your suggestions. The medication Kineret is known to be very irritating. Sammi's physician prescribed the EMLA and Elecon steroid cream because of that. As Sammi is not particularly chubby I am avoiding giving the medicine IM as it is not supposed to be given in the muscle, but I don't think I am giving it intra dermally. I am a nurse as well and think my injection technique is OK, though its not easy giving a painful injection to your child that's for sure! I have often wondered if my days of crushing medicine and inhaling the dust from them has had an impact on my kids but I doubt we'd ever figure that out. As for heavy metals aside from the standard testing for lead I cannot think of any testing but they have been screened for quiet a few disorders and our gene pool is unfortunately riddled with auto immune disorders on both sides so certainly that may be factoring into all of what we see. Yes, the immunologist is concernd about immune suppression with the immune dysfunction and we are to hold it if any sign of infection, she also has discussed IVIG as a possibility but said that she wanted to see how Samantha does with this and that Lupus patients tend not to respond as well to IVIG and that she would need higher doses to treat the auto immune issue and that will require some work to get insurance to cover. Samantha is home tutored along with her brother due to her multiple medical issues, intestinal pseudo obstruction, immune deficiency, metabolic bone disorder, fatigue (she typically sleeps until 11:30am and has to be woken to get up for school in our basement classroom) she does go into school for an hour and a half three times a week in the afternoon for specials and to see her in school classmates but does not go in if she is feeling under the weather or bugs are running rampant . She has used mylanta for the apthous ulcers in the past with some relief but these newer "ulcers" are rather odd in that they start as fluid filled blisters, and then pop and become rather nasty red, irregular shaped mucosal ulcers. The oral hairy leukoplakia does not seem to bother her, though I have been told it will make her more prone to thrush which we have had several times, she also tends to get yeast small bowel bacterial and even esophageal overgrowth and will at times eat some yogurt as well is on daily twice a day lactobaccilous plantarum that her GI prescribed..Thanks again for the suggestions will look into some of those options.
LEE ANN, IVIG WAS A LIFE-SAVER FOR ME
I have a secondary immune deficiency caused by hypocomplementia, and when I had to go on cytoxan and cellcept for cns lupus it became a real problem. I had several major infections including mrsa and vre. Since I have multiple antibiotic allergies, IVIG was a good step for me.
I don't use EMLA often because it takes so long to work - waiting an hour or longer for it to take effect is a pain. There is a skin freezing spray called Instant Ice that I use with my blood-thinner injections - it doesn't last as long as emla but works much quicker. Have you asked your doc about some of the stronger topical anesthetics like nuerocaine or TopiCal - the things dermatologists use before skin procedures?
Did Sammi's mouth ulcerations coincide with any worsening of her bowel or digestive problems? I ask because my niece has ulcerative colitis and when she is in a flare she develops severe mouth ulcers and arthritic symptoms. Her ulcerations are very red, deep and extremely painful - almost like burns on the roof of her mouth. Sometimes she also gets them underneath her tongue.
EMLA, oral ulcers...
Just gave this evening Kineret and I must say the Elecon and EMLA just do not cut it..the shot/needle itself is not the issue its the darned medicine itself. She was OK with the needle going in but as soon as the medicine gets in she starts to cry. This is just mean stuff. Don't know what can numb deep enough for sub q injection?..
In all honesty the latest blisters/ulcers were when she was complaining less of aches pains and headaches and I was second guessing myself and the Kineret, if we should even start it. She had an endoscopy and colonoscopy June 20th which found esophagitis, gastritis, duodenitis and colitis, basically inflammation of all sites biopsied but not consistent with IBD: crohns or Ulcerative colitis. The research immunologist also told us that her blood work is actually the opposite of what one finds in UC or crohns patients. However from what little I can gather Lupus can cause inflammation pretty much anywhere including the intestinal mucosa if that is where inflamatory cells settle. I think this is why her immuo feels she is basically showing symptom of atypical lupus as well as the Overactive I believe interferon alpha test results she had with her blood work and the other inflammation elevated sed rate and high ANA. I have only seen the bubble blisters in her mouth twice in the past 6 weeks or so, she does also get nasal, very large and sore ulcers on the inner septum of her nose as well as in her mouth along the gum line, under her tongue, and occasionally on the roofof her mouth but those ulcers look like fairly round defined superficial craters. Her GI issues in general have worsened over the past year but as she has intestinal pseudo obstruction, has issues with small bowel bacterial overgrowth infections and spontaneous ileus or bowel shut down for no apparent reason and has dysmotile symptoms already it has been difficult to know whether her symptoms were related to pseudo flares, overgrowth or something new..as it turned out the scopes in June did show something new without a clear reason as to why this had developed. She also had obvious physical worsening of her large intestine with dilated overly stretched colon in spite of keeping her stools liquid for almost 3 yeards now and giving her large doses of laxatives and promotility medications daily. I know pseudo obstruction can be a complication of lupus and other autoimmune issues though it appears in Sammi's case pseudo came first..autoimmune second. I will be talking to the immuno doc assistant tomorrow and ask about any stronger topical analgesics for the kineret. Sammi is a trooper and cooperating but its not a good thing to see her in tears and pain nightly from something we are hoping will help...
I understand Lupus in and of itself causes immune deficiency and compliment levels can drop with active disease, it does make sense to me to use IVIG to protect against infection, I was not sure but it also sounded as if IVIG might in high doses also be used as a treatment FOR Lupus? Perhpas I misunderstood the immuno but I got that impression too..perhpas hse was telling us as a treatment for Lupus itself it is not been found to be very effective? Have you heard of its use to treat Lupus itself?
INFO ABOUT NUMBY - IT DEADENS DEEPER THAN EMLA
cOPIED FROM WEBSITE - PAINLESSHOTS.ORG
With its name, "Numby Stuff," there wont be any trouble selling this idea to most kids, but selling it to the doctors might be a bit harder. It takes up quite a bit of time, and the science fictiony look to the procedure might scare some kids (and adults) the first time it is used on a patient. However for deep procedures it is probably the most effective way of giving pain relief.
Numby Stuff is another use of lidocaine, and like EMLA it uses a patch, but Numby Stuff also uses a device called a Phoresor II Auto Iontophoretic Drug Delivery system to provide skin numbing up to a depth of 10 mm. It takes ten minutes to work. It is called iontophoresis for short. It uses a mild electric current from a small battery powered generator and two electrodes to push the lidocaine and epinephrine into the skin. The lidocaine numbs the skin and the epinephrine pushes the lidocaine to deeper levels and makes the numbing effect last longer. To most children it feels like a tingling or warm sensation under the patch.
After the procedure the skin may be blanched or red, but this reaction is normal and the normal skin color should return in a few hours.
It should be used only with caution in patients with liver disease because their body might not be able to process local anesthetics. It should also be used with caution with heart patients. It also cannot be used on damaged skin, or on temples or on the eyes.
Lidocaine and Numby Stuff should not be used on people who are allergic to lidocaine, epinephrine, sulfites, or people who are allergic to certain kinds of adhesive tape. It also should be used only cautiously with people who have peripheral vascular disease.
RE THE IVIG, I think it is used to treat primarily immune deficiencies, including those that occur with lupus. I have heard of IVIG being used in conjunction with plasmaphoresis to treat severe lupus, but it is horrendously expensive and not covered by most insurers. I have undergone plasmaphoresis by itself twice when my antibody levels and sed rate were through the roof (ANA 1:10240 at which point they stopped counting - sed rate over 100) - both times it gave me a temporary remission but didn't last.
Your're right that lupus can cause inflammation anywhere in the body. If it develops in the abdominal cavity , it is sometimes called serositis. It can cause a lot of problems with intestinal adhesions.
As a nurse, you know a lot more about this stuff than I do, but has your daughter been evaluated by a pediatric rheumatologist? If she does have an atypical form of lupus - a pediatric rheumy might be able to suggest more treatment options. Also, have any of her doctors ever mentioned the possibility of CREST syndrome, which is a form of scleraderma? It can cause severe GERD, esophageal and intestinal motility disorders, arthralgias, calcinosis, and other symptoms.
NUMBY STUFF! Thank you!
I will definitely ask about it...sounds much better then what she has at present.
Yes she has been seen by rheumatologist, in 2002 when she had sever pseudo flare and was inpatinet she was found to have an ANA of 1:160 and they considered dermatomyositis as well as IBD's. neither of which was diagnosed though at one point her sigmoid colon was scoped and found covered in apthous ulcers and the differential was Crohns. This spring she had horrid multiple times a day head pains she called headaches, stabbing, sudden and blessedly brief spells, joint aches and pains all over that migrated, a raised spider like rash on her elbow that has come and gone for 4 years but now persisted for months, oral and nasal ulcers and then teh oral hairy leukoplakia appeared, a repeat ANA of 1:1,280 and sed rate of 30. The rheumy did lupus, scleroderma, thyroid and Wegners ANCA, PANCA blood test...IgG was elevated sed rate still up repeat ANA 1:320 speckled with positive mitotics. Her GI thought a vasculits, suspected Behcets. however his scope biopsies did not pick up vasciulitis..may not have been deep enough? may not have been read by experienced pathologist??? we did find ONE giant cell and itis of entire intestines all biopsies were inflamed but not IBD. Rheumy told us..she has an "autoimmune process going on which we need to allow to evolve". The research immunologist agrees its an immune process, with an underlying primary imune dysfunction that is lab typical of Lupus patients and that her experience is these type of patients rarely express "typical autoimmune diseases"and that if treatment is not inititated to control the inflammation her experiences is they do go on to full blow autoimmune disorders and are much more difficult to manage then as we understand patients with autoimmune diseases who do not have coexisting primary immune dysfunction. Thhis doc is one of few in the country and is at a University medical center with a research lab, which is where she tested Sammi's blood. I expect she is not in the realm of "typical or necessarily accepted standards as much of her work is research based but she strikes me as extremely intelligent, very versed and knowledgable and her explanations from my nursing and medical perspective make sense. As we've been told once inflammatory damage is done you may have a clinical bonafide diagnosis BUT you also have irreversible damage. We have watched Sammi have increasing issues and increasng issues for five years now, you can imagine why we do not wish to wait and see more damage before we try to prevent more problems. We have lived with her and heard and seen the daily fatigue, limited energy, heard the pain and headache and dizziness complaints..seen the GI issues, read the lab tests, it just feels to us how many times do you need to be hit over the head with the sledge hammer before you get it... So a long answer to your short question yes have seen the rheumy, did not like the texxt book typical answer..come back with butterfly rash, fever and protein, blood in urine and I'll call it..or double stranded dna antibodies etc..as the immuno said the conversion to positive antibodies usually takes years, 30 % get a facial butterfly rash..The headpains and dizziness worry me enough, the arthralgias worry me enough. I'd have a hard time forgiving myself if I wait for obvious neuro lupus/seizures ad such or kidney damage and know I could have at least TRIED to spare my child some of that. Hope that makes sense..