Thick white build up on the tongue, sores on the corner of the mouth and issues with the voice box (larynx) have been reported in PC patients.
Approximately 84% of PCers experience buildup of a thick white film on the tongue and inside cheeks. At the 2005 Patient Support Meeting, Dr. Leonard Milstone explained that this is just excess keratin. It is white because it is wet/moist (like when you leave your fingers in water for a long time and the skin turns white). The medical term is oral leukokeratosis. It is not leukoplakia, which is a pre-cancerous condition. In babies, oral leukokeratosis can be misdiagnosed as Candida albicans and may cause difficulty in sucking.
Angular cheilitis (inflammation and fissuring sores at the corners of the mouth) which is sometimes secondarily infected.
Laryngeal involvement is a less common feature, reported primarily in young children. It might be symptomatic but usually presents as hoarseness, stridor and, occasionally may cause life-threatening respiratory distress requiring medical intervention.
CARING FOR MOUTH, TONGUE, & LARYNX
Oral Leukokeratosis normally doesn’t cause pain or any issues. Some people do nothing. Others use their teeth to scrape their tongue or brush it with a toothbrush or tongue scraper.
We have found out from surveying patients about Angular Cheilitis that “An antibiotic usually helps; even neosporin right away can help. Bactoban is the next level and does work well. However, there is a new product that we’ve had great reports on. It is called AltaBax in the USA. It is a more potent or higher level antibiotic and requires a prescrption. The 3 patients we know who have used it have had excellent results and the sore cleared very quickly. However, it is very expensive (like $60 for a small tube). A little goes a long way, and the physician was able to give some samples which helped.”
Laryngeal Leukokeratosis in PC
Dr. Eli Sprecher, “The most important thing to do in patients with laryngeal involvement in PC is to try to avoid any surgical intervention (which often leads to further aggravation), unless of course air way is compromised. When we reviewed previous cases, our impression was that every intervention just triggers further regrowth of the hyperkeratotic tissue.”
Dr. Edel O’Toole “Intervention with laser or debulking makes it worse. The laryngeal leukokeratosis usually gets better as the child gets older. There is no evidence that oral retinoids help substantially with the keratoderma of PC (although some patients do take them intermittently to lessen callus, but at the expense of skin fragility/blistering). I am not aware of oral retinoids being used in a child specifically for the larygeal leukokeratosis.”
Goldberg I, Mashiah J, Kutz A, Derowe A, Warshauer E, Schwartz ME, Smith F, Sprecher E, Hansen CD. Symptomatic Mucosal Involvement in Pachyonychia Congenita: Challenges in Infants and Young Children. Br J Dermatol. 2019 Nov. 182(3).708-713.
PATIENT TIPS & COMMENTS FOR MOUTH, TONGUE, & LARYNX
K6a patient: “I brush with a toothbrush to keep the white layer from getting too thick. It doesn’t ever take the whiteness away, but it does seem thinner and prevents excessive build-up.”
K6a patient: “I don’t do anything except bite off the inside of my cheeks when they grow too much.”
??? patient: “I brush my tongue as well as possible.”
K6a patient: “I chew the stuff off.”
K6a patient “Hi I just wondered if anyone had any advice for the mouth, my daughter is 6 and her tongue is so thick on the sides she’s crying it’s hurting. She’s brushed it with a toothbrush but it’s making it worse. Does anyone else have this? Thank you”
K16 patient “I have had troubles with my mouth my whole life and a few things that have helped is using an extra soft toothbrush and plain toothpaste for brushing. I like Colgate as it does not irritate my mouth like Crest and some of the other brands. Sometimes swishing with warm salt water helps. When it flares up with multiple sore spots, I skip brushing and just rinse daily with Peroxyl, which helps with healing. She is probably too young for that, but I think Orajel makes a sore mouth rinse for children. If you are unable to get relief for her at home, you could check with her dentist to see if he might have something that can help. My dentist once prescribed a steroid rinse to help me clear up a mouth full of sore spots. Another thing to watch for is irritation or sores at the corners of her mouth. A lot of us develop angular cheilitis which my doctor treats with a prescription of nystatin cream. It is awful to see a child suffer. I hope she feels better soon.”
K6a patient “I had a lot of trouble with the white build up when I was young, a few things helped, eating toast in the morning and scrubbing it with a towel, it’s quite gross to think of now but worked for me, also if I used mouthwash daily that seemed to help too. To help with the pain, use a teething gel like sm33 or bonjela, it’s got numbing agents in it.”
K6a patient “I started using a tongue scraper a couple of years ago, and I loveee it. I was nervous to try it bc I thought the friction might make things worse, but it’s made things better ! There are days, when my tongue looks pretty close to normal. I just do three pretty firm scrapes every morning. I would definitely choose a metal scraper. A friend of mine was getting her certification in Ayurvedic medicine … she told me waste is excreted from the tongue, which is what motivated me to try it … if we have extra tissue, I can see us holding on to more of the waste. Might be worth a try.”
K6a patient “I use a tongue scraper daily. It definitely helps.”
K6a patient “White tongue. Does anyone have a thought of what makes the tongue more or less white? The thickness of the white mucous membrane is varying a lot and we don’t know why. Does anyone know what we can use to make it thinner? If something she eats affects? Or if you know something medical to use.”
K16 “I just brush harder, but my dentist doesn’t like that. I haven’t heard of any remedy
K6a patient “Drinking a lot of water and avoiding sugar helps me. Brushing my tongue when I brush my teeth also helps. I use baking soda toothpaste.”
K6a patient “I brush my tongue after brushing my teeth, especially in the morning. The reason the leukokeratosis is white is simply because it’s in the mouth and it’s always wet.”
K6a patient “This help alot. Use organic coconut oil as a mouth wash.”
Trouble with nursing/sucking
K6a patient: “Sucking for baby with PC was difficult and seemed painful. I used very soft bottle nipples with big holes. I needed to sometimes squirt the milk in the baby’s mouth at first, so I used bottles with soft plastic that could be squeezed, which worked well. Sometimes, the baby will cry at first then be hungry enough to just “go for it” and then get used to the sucking. Either the pain lessened as sucking went on or just got used to it. The big nipples and squishy bottles helped a lot. The pain of sucking seemed to subside after the first half year.”
K6a patient: “I didn’t feed for a long while and ended up staying in the hospital for a few months at the start of my life. These days you cant keep me away from food.”
K6a patient: “I was actually taken aback to see this listed as a possible manifestation of PC on the home page…”Intense pain lasting 15-25 seconds often when beginning to eat (this may be connected with salivary glands rather than ears and more research is needed on this finding).” When my son (who has PC) was born, he had horrible feeding difficulties. He would scream and cry when he attempted to suck, and it was absolutely chaotic for the first few days/weeks of his life. We tried breastfeeding, multiple styles of nipples, etc. At a few days old, I discovered blood in one of his spit-ups (Imagine the panic!). A general pediatrician had me give him Mylanta (ADULT STRENGTH) suspecting “indigestion.” Needless to say, we never returned to him. I noticed a blister on my son’s tongue and in the rear roof of his mouth. I was so incredibly petrified that he had EB (epidermolysis bullosa). Dad and I were misdiagnosed with EB until I was about 17 years old. As you may or may not know, EB can be very, very severe, where the mucosal tissues blister. We took my son to our dermatologist (AMAZING physician) who assured me that she did NOT believe my son had EB. As an aside, she also told me that he has the most beautiful eyes that no one would ever notice his nails. HAHA! Way to a new mom’s heart, eh? We finally ended up in the care of a pediatric GI specialist who basically, by trial with medication, diagnosed Cooper with reflux. Praise be to God — Zantac and Reglan (bad stuff) seemed to ease his suffering. I think we collectively believed that it was reflux complicated by PC or vice versa. We still had to find new styles of nipples periodically, but he grew to a happy, chunky little monkey in no time.”
K6a patient: “My son had a hard time feeding as a baby. It’s strange, but he also brought up blood when he was a couple of days old. That was the first of many, many hospital visits. My son could not suck as it was too painful. I breast fed for the first few days, but he was constantly crying. I thought he was not getting enough milk, so I changed to a bottle, but this never helped. I would sit for hours squeezing the milk into his mouth. Doctors changed his milk a few times, thinking he was allergic to various things. He was then put on to solids very early. We were also told he had gastric reflux, and my son was given all sorts of meds — he was always sick. Then, at 10 months, he was diagnosed with PC. We stopped all the meds and he seemed to get better very quickly. He doesn’t seem to have problems with his mouth now, except he doesn’t like things which are acidic. My son does get the ‘ear’ pain though, it is usually when starting a drink/meal. It’s quite intense, and he likes us to put pressure on his ears for a few seconds, then it seems to pass.”
See Miscellaneous Questions & Answers for Breastfeeding.
Trouble with Angular Chelitis
K17 patient “Can anyone who gets skin cysts at the corners of your mouth please give us some advice on what we can do to treat/prevent/help remedy? These just started – 14 yr old, K17. Not fun for middle school age girl, she is having hard time. Thanks in advance.”
K6a patient “I had this a few years ago and it managed to clear up by itself the only thing I did it was put Vaseline with aloe Vera on my lips to help the moisture and it’s cleared up now sometimes I’ll get a tiny tiny one appear but I have to leave it and not touch it. The doctor thought I had oral herpes, I tried explaining I have pc and it was that … Herpes was fairly offensive as I have a wife who I’ve been with 10 years 😁. Hope your daughter is okay”
K16 patient “My son gets these from time to time, I’ve always just thought they were cold sores, we put abrevia on them and they clear up. Never thought to connect it to pc.”
K16 patient “Looks like angular cheilitis (aka perleche) and yes, it is related to PC. I am K16 and have had this for as long as I can remember. The dermatologist can prescribe a cream to put on at night that helps clear it up and/or keep it under control. It is also helpful to make her dentist aware of her PC condition; mine uses vasoline or numbing cream when having dental work done because it can become quite painful.”
K16 patient “Great tips. If kept too moist, it can turn into a fungal infection. My doctor gives Nystatin cream for that.”
K6a patient “The moist, spongy tissue is a set-up for bacterial or fungal infection. I remember being prescribed nystatin lozenges as a teen. My 16 yo old is dealing with them right now. Bactraban seems to help. Bactraban is my magic juju cream – I swear it helps everything ! Naughty to say bc it’s an antibiotic, but a little goes a long way. I think my dad actually puts yogurt on his (anti-fungal). Also, my son had a dental appt this week. He’s got these in both corners right now. She recommended a multivitamin – worth a try.”