Hailey-Hailey Disease
March 25, 2010 by: Deepak
Filed under: Skin Disorders
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.
It does scar.Especially noticable after getting out of a cool shower or swimming.Big purple outline of the once “flared up”
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?
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.
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!