Hailey-Hailey disease also called as familial benign chronic pemphigus is a rare chronic autosomal dominant disorder of skin. The name comes from the Hailey brothers who first described in 1939. It is characterized by recurrent blistering, crusting and erosion in the skin fold areas like groin, neck folds, chest, under the breasts and axillary areas.
Haily-Haily disease is most common on late twenties to forties but may occur at any age. The main cause is unknown but its is thought to be due to genetic defect in calcium pump protein. Besides genetic connection there are other contributing factors that might exacerbate it. Some factors that might exacerbate the disease are heat, sweating, friction, physical trauma , stress and infection. Most people often find their symptoms worsening during the summer.
Initially burning and itching followed by red, scaly or small fluid filled blister may appear on area where most friction occurs. The blisters may then rupture with malodorous in case of secondary infection. The lesion may be localized in certain area or sometimes generalized. These lesion comes and heals frequently without leaving scars. Patients with Hailey Hailey disease may often present with nail manifestation which looks like longitudinal white bands in the fingernails but are not brittle or fragile. Some females may also find their symptoms worsen during premenstrual periods. This suggest that Hailey-Hailey disease may have some connection with sex hormones.
Your doctor may diagnosis this Disease by its appearance and family history and confirm it with skin biopsy. This disease can sometimes be mistaken for fungal infection, heat rash, bacterial infection, eczema and other blistering conditions, so skin biopsy is highly recommended in case of suspicion.
There is no cure for Hailey-Hailey disease but its not a life threatening disease. The aim of treatment is to avoid aggravating factors like sweating, friction, stress and infection. You should avoid tight clothes, wear light loose clothes, avoid excessive sweating , keep the body folds cool and dry. Sunscreens might help to some extend. There are various treatments options that have been tried. Local lesions may be treated with topical corticosteroids and topical antibiotics. For widespread cases topical treatment with oral antibiotics may be given. Several oral medications like retinoids, dapsone, ciclosporin and methotrexate have been tried with limited results. Study suggest Calcipotriol cream may be useful in some cases.
In some severe cases Carbon dioxide laser ablation, pulsed dye lasers and dermabrasion have also been tried with some good results. Few research showed that Photodynamic therapy has no success in the treatment of Hailey-Hailey disease. Radiotherapy has also been tried for localized lesions with limited success in few patients. If the skin is severely affected, skin can be removed with surgery followed by skin grafting.
If you have any questions regarding Hailey-Hailey disease ask us at our Dermatalk Skin Care Advice Forum.
Brenda, my experience sounds very similar to yours. I’m not sure if it was a secondary infection but the pain was nothing I’d experienced before. When I went to the toilet I nearly screamed in pain and after when I’d get off the toilet I was unable to walk because of the stinging involved. My charming dermatologist (whom I had seen a week prior) because of the HHD, had done a swab and showed I also had thrush, looked at my lower regions briefly and said “you have thrush” end of discussion!!!! The fact I couldn’t sit down in her office and that I was in tears you would think maybe there was also something else involved. Alas, I got no treatment. I wasn’t able to put anything on my skin anyway but surely there should have been something!! This all lasted about 2 weeks, two weeks of hell. I have only had three bad outbreaks in 30 years and each one of them I had the same secondary infection. I too wear loose clothes which does nothing for ones self esteem. I don’t wear underwear anymore as the friction does not help the HHD. I suppose I should be pleased I don’t have this disease year round as so many sufferers do. Take care all.
OM goodness! I am so sorry you are all suffering! I too have suffered for years with this awful, awful disease. My first outbreak happened in 1984 when I was 21, but wasn’t diagnosed till ’87. I have tried almost everything over the years. Antibiotics (keflex seems to work the best these days), prednisone, anti fungal creams, antibiotic creams, steroid creams, dermabrasion (still hasn’t returned in that spot for over 10 years), botox (didn’t work), CO2 laser on a test spot on my labia to see if it would work (really didn’t hurt that much believe it or not!) The dermabrasion was uncomfortable but not super painful. This was done just at the top inside of the leg. After the CO2 test the doctor suggested I try taking Amitrityline, which is an anti depressant, but also acts as an anti-itch and a sleeping pill all in one. The rubbing because it’s itchy is half the battle for me. I used to take anti-histamine for the itching, but don’t need to anymore. For me, my worst spot is my groin/genitals/anus areas, but I also break out under my breasts, both underarms, and on my neck. I don’t cover my neck anymore. I’ve got to the point where I don’t care if people can see it, it is just too painful to wear a collar that rubs on the sores all day. Plus, it will never heal if clothes are rubbing on the sores all day. I wear a size bigger in clothes, tops never with a collar and a scoop neck so ther’s lots of room if I have sores. I wear skirts and also boxer shorts really help. I’ve had a break out in one elbow pit, but it didn’t amount to much and never came back again. I also had a small spot on one of my knee pits that didn’t amount to much (thank goodness). My worst years were from about 2000 until 2005? Can’t remember exactly, but it was a long stretch where I had sores on my neck, underarms, under breasts, on neck, in genital/groin area. It was absolute torture. I gained a bunch of weight because I was eating too much and not exercising because it was so painful just to make it to work and back I couldn’t even think about exercising. That was until in November 2007 I had the absolute worst brekaout ever. I thought it hurt all the times before, but it didn’t compare. My labia/vulva swelled up so bad I could hardly move. For a week and a half I couldn’t sit down, I couldn’t walk (well I could, but I looked like a crab walking.) Had to sleep and lay with my legs apart, because just to touch myself was so utterly painful! I got a bladder infection because I couldn’t wipe myself. I was prescribed predisone this time because of the severity. Was also given prednisolone eye drops which I tries to pat on the labia/vulva, but any contact at all was excruciatingly painful! I took 50mg of prednidone a day for 10 days before it started to work. My back went out as well from having to walk funny and lay funny. Since then I’ve only had 1 breakout in the labia/vulva area! Not sure why, I must be in some kind of remission! I haven’t had a bad breakout anywhere for a long while (knock on wood). I have 2 small spots on the back of my neck that I’m hoping won’t amount to much (fingers crossed). It was passed down from my Mom and 2 of 4 of us got it. I don’t have any children, but my sister, who also has HHD, passed it on to 1 of 2 kids. I had it the worst until last year when my sister was bed ridden for a month and got a blood infection. It was a month later before should could sit comfortably and where pants. Right now I am able to wear cotton sweat pants a size too big. Boxer shorts a size too big as well. Nothing rubs anything if I can help it. The only clothing item I wear that has a collar is a coat. I can’t have intercourse because the friction breaks me out.
Anyway, that’s my story. Hope you’re all feeling better soon.
I have had hailey hailey for 20 years. Right now I think is the worst I have had it. I don’t seem to get it much in the summer but the fall and winter it is extremely bad. Right now it is on the back of my knees , under my breasts , by my groin area and very large ones on my back. The back is new for me this year. It is so uncomfortable right now I cannot wear a bra or anything tight on me. I just dont know what to do anymore it hurts so bad. I am going to try to go gluten free to see if that helps me. Wish me luck.
I have had Hailey Hailey for 22 years. I get the blisters under my arm pits, under my breasts, in the groin area, the backs of both knees, my eyes, my neck, and in my private areas, front and back. When they are so bad, like they are now, behind my knees, it is like velcro ripping apart at every step I take. It is also like someone is burning me in all the spots with a continual iron. I have so much infection now that nothing seems to help. I have had LOTS of Prednisone, Cycolsporine, Amoxicillin, and cortisone shots. I have been in the hospital many times. I seem to keep getting worse. I rarely have a remission. It is has gotten so bad now, that the minute I put on clothes, the blisters start again. I live in a cotton caftan. I can’t really go anywhere, except to the doctor, becausse I pay with new blisters when I put on clothes. I have been this way since 1997. I truly get weary of the pain, as I am sure that you all do too.
OMG…I have to tell everybody about this…I mean it is a miracle for me….A little about me…I have been battling Hailey-Hailey disease for the last 10 years and only knew it was Hailey-Hailey disease when my lesions were biopsied 5 years ago after finding out my father also had it and his father. I was devastated because the doctor told me that all we could really do was treat the symptoms and that the breakouts would stop in my 50s…well I am in my 30s, a young women, and from reading all of your posts it obviously doesn’t just stop in your 50s!….The lesions I would get were huge….10 cm long, purulent, sticking to my shirt….the whole nine yards….I would get them especially bad on both sides of my neck (couldn’t hide them) under my breasts, and in my axillae. I always had active lesions…sometimes they would start to heal but then get bad again and again, always starting out as what appears to be little blisters…then bam!….over the last 6 years it has been so bad…painful…like burns…been on everything…tacrolimus, vitamin D cream and supplements, dapsone, prednisone….etc, etc…nothing would stop them….Tried everything on this website….magnesium baths, bleach baths, whatever was new I tried….well I gave up on the Hailey-Hailey disease and decided I would forget about it and focus on my acne instead. Yep acne too….So I started reading the web and found alot of sites stating that if you want clear, acne free skin to go gluten free…then I started reading about other signs and symptoms you could get by eating gluten and realized that alot of those lesions looked like mine…now keep in mind my lesions were biopsoed several times and showed Hailey-Hailey disease with the acanthylosis (or however that is) etc…My dad was diagnosed with it when he was 30 and his dad while he was in Korea….Hmmm…I finally decided what the heck…I am miserable…I am going to go gluten free and see if it helps my skin at all….OMG…I cannot believe it..8 weeks ago I started this and I had two active lesions…one on my neck (4 cmX4cm) and a huge horrible, painful one under my breast that I have had active for about six months!!! Lots of acne….after day 2 of completely gluten free the itchiness was gone…and my lesions were already healing…I could not believe it and still cannot believe…My acne and Hailey-hailey lesions almost completely healed after 2 weeks….It is two months later and no sign of any Hailey-Hailey lesions….no itchiness, no blisters….no redness…and since going gluten free I have lost quite a few pounds….Going to the gym now…getting sweaty….Still no Lesions….I want you to understand that I always have had at least 2 active lesions at any given moment over the last 10 YEARS!!!! I wanted to wait longer to post this but I feel so good right now….I wanted to put it out there! I mean it costs nothing to try except your time to look at what you are eating carefully and cut out any possibility of gluten…I will post again in a few weeks to let you guys know how things are going…No I have not had one single smidge of gluten in the last 2 months!! not one! Please try it…what do you have to lose??? I mean maybe it is just a fluke in timing, but, I made a drastic change, and I am getting a drastic result!!! I mean I know we can get meds and everything…and we listen to what the docs are telling us but lets face it….Doctors do not know everything….Hailey-Hailsy disease is rare athough getting more common….please just try it and keep me posted on your success or no success…just do not cheat!
I too suffer from Hailey Hailey disease since my early 20’s. I have the same type of purple scaring after the lesions go away. If anyone knows of a good treatment for this I’d love to hear them! I have taken oral antibiotics and steroid creams but right now I’m suffering badly.
I suffered from Hailey-Hailey disease since November 2008 till now. What treatments other than the use of steroids? Whether the disease will be cured?
It does scar.Especially noticable after getting out of a cool shower or swimming.Big purple outline of the once “flared up”
[…] Finger nails change shows red and white longitudinal bands, longitudinal nail ridges and longitudinal splitting. Finger nails becomes fragile. Other additional symptoms and signs may be present in some cases. Atypical presentation and coexistence with other disease may be common. It can coexist with chronic familial pemphigus ( Hailey-Hailey Disease ) […]