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danavs
@danavs

Posts: 25
Joined: Oct 04, 2016

Hailey-Hailey Disease

Posted by @danavs, Oct 31, 2016

I have a painful and itchy hereditary skin disease known as Hailey-Hailey. I am 78 years old and it has gotten much worse as I age. Before I fell twice in the last few months I was seeing my dermatologist every two months; now I talk to him on the phone or leave a message about my condition and prescription needs. The worst place on my body is between my legs and behind, causing horrible pain when I sit or try to get in and out of a car. I have the blisters, whelps, crusting, raw skin, and bleeding over much of my body and everything (clothing, bedding) hurts my body. Have had the disease in between legs/behind for 18 months; it gets a little better, then worse again and when urine touches it, I sometimes cry or almost scream. Do any of you or someone you know have HH?

REPLY

Hello @danavs,

Hailey-Hailey disease (HHD) is such a rare skin disease, and it can be so painful; I’m very sorry. However, I’m glad you came to Connect to seek some answers. I’d like to introduce you to a few members; they don’t have Hailey-Hailey, but hidradenitis suppurative, another rare skin condition, similar to HHD, and I hope they can provide some insight. Please meet @autumnleaves, @amy75, and @sandig.
I also hope @midnight, @mbrittain, @drew1991, @msdodo987 will return to give us an update on how things are going.

@danavs, have your doctors ever suggested a laser treatment? What do they recommend?

@kanaazpereira

Hello @danavs,

Hailey-Hailey disease (HHD) is such a rare skin disease, and it can be so painful; I’m very sorry. However, I’m glad you came to Connect to seek some answers. I’d like to introduce you to a few members; they don’t have Hailey-Hailey, but hidradenitis suppurative, another rare skin condition, similar to HHD, and I hope they can provide some insight. Please meet @autumnleaves, @amy75, and @sandig.
I also hope @midnight, @mbrittain, @drew1991, @msdodo987 will return to give us an update on how things are going.

@danavs, have your doctors ever suggested a laser treatment? What do they recommend?

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kanaazpereira, Connect Moderator. Thanks. HH is because a missing gene causes the skin cells to become unstuck, so laser surgery is out of the question. I refuse any surgery because it messes up the skin so bad. Even the pads they put on chest and under breast to monitor everything for a colonoscopy leaves my whole chest messed up forever. Last time they used juvenile pads and it wasn’t quite as bad. Skin on skin causes flare-ups, so I pat skin with corn starch on much of body every day. I have been using corticosteroids for years which is the only thing that helps to keep it from spreading and lessons the pain, but it also causes the skin to become thin. Anti-fungals helps some with the itching. Heat outside and heating in winter inside makes condition worse. I trust my dermatologist with treatment and he also has wonderful, caring bedside manners – which we all need.

Thank you for the information, @danavs; it must be exhausting to be so careful!

Have you heard about the drug afamelanotide? Here’s a paper that was published about its efficacy in treating Hailey-Hailey disease:
http://bit.ly/2fpuxmQ and http://bit.ly/2em4U5u

Although Bullous pemphigoid is different, but since Hailey-Hailey disease belongs to the ‘pemphigus’ autoimmune disease family, I would also like to introduce you to @earljohnson5030, and @profkatg as they have discussed their experiences with Bullous pemphigoid. Or you can view the discussion within the Autoimmune Diseases group here:
http://mayocl.in/2ema9lV

@danavs, has your dermatologist spoken to you about afamelanotide?

@kanaazpereira

Thank you for the information, @danavs; it must be exhausting to be so careful!

Have you heard about the drug afamelanotide? Here’s a paper that was published about its efficacy in treating Hailey-Hailey disease:
http://bit.ly/2fpuxmQ and http://bit.ly/2em4U5u

Although Bullous pemphigoid is different, but since Hailey-Hailey disease belongs to the ‘pemphigus’ autoimmune disease family, I would also like to introduce you to @earljohnson5030, and @profkatg as they have discussed their experiences with Bullous pemphigoid. Or you can view the discussion within the Autoimmune Diseases group here:
http://mayocl.in/2ema9lV

@danavs, has your dermatologist spoken to you about afamelanotide?

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kanaazpereira, thanks for the info on afamelanotide. I will ask my dermatologist about it.

I’m so sorry to hear you are having such an awful time. I have HH, and know all to well what you’re going through. I hope you can find some relief!

@christy5348

I’m so sorry to hear you are having such an awful time. I have HH, and know all to well what you’re going through. I hope you can find some relief!

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Hi @christy5348. Thanks for letting me know I’m not the only one on Connect with HH. Can you tell me how the disease affects you. I’ll remember you in my prayers and send you a hug!

@christy5348

I’m so sorry to hear you are having such an awful time. I have HH, and know all to well what you’re going through. I hope you can find some relief!

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Hello @christy5348,

I’d like to welcome you to Connect, and sincerely apologize for the delay in responding. We are so glad to have you here.

I also wanted to encourage you and @danavs to view this article about carbon dioxide laser treatment for Hailey-Hailey disease, as results show that resistant cases that fail topical treatment have responded very well to this form of therapy:
http://bit.ly/2mQVAfI

@christy5348, @danavs, have you considered this minimally invasive procedure as an option?

Complicated disease with no easy answers. Have recently tried low dose naltrexone as per the Archives of Dermatology August issue (2017). Robinul can be useful. Blue light therapy (as you would do for pre-cancers) has helped. In other words, treatment is empiric-trial and error.

Hi, @dsl1, and welcome to Connect. Thanks for your input for this discussion on Hailey-Hailey Disease.

How are you feeling these days with some of these treatments? Are you still experiencing a lot of symptoms?

What a relief to find others with whom I can talk about this! After scratching my head (and a few other places) for about 12 years, a dermatologist finally diagnosed me with HH a year ago. @danavs , have you tried tacrolimus ointment? It isn't exactly working miracles for me but it seems to be helping. Additionally, I take 4-5 mg of glycopyrrolate to prevent sweating, as the dampness from skin-to-skin definitely aggravates it. Like I said, I'm not all better, but I'm much more comfortable than I was a year ago.
FYI I'm a 41y/o female and have the HH on my outer labia.

@huitzilin76 and anyone with HH- My dermatologist had me try a compound Naltrexone 3mg caps because my HH had gotten so bad. This drug in 30mg is for some other problem, but scientists discovered it helped HH get well as long as you continue taking the compound. The first thing it did was stop the itching, then the pain and every thing except between the legs & behind was gone. The HH between legs & behind was shrinking. Then I ended up in the hospital where they stopped the drug to see if it was interfering with blood pressure meds. The HH returned with a vengeance! So I called dermatologist to have doctor put me back on the compound, which he did. Now I'm just waiting and praying it will start working again. Just 2 days without compound made such a difference. If your dermatologist will try this one on you, don't let anybody take you off it. It may take a month or more to start working but be patient and I hope it will work for you. It was miraculous how it worked and I intend to keep taking it now and praying it will work again.

@danavs

@huitzilin76 and anyone with HH- My dermatologist had me try a compound Naltrexone 3mg caps because my HH had gotten so bad. This drug in 30mg is for some other problem, but scientists discovered it helped HH get well as long as you continue taking the compound. The first thing it did was stop the itching, then the pain and every thing except between the legs & behind was gone. The HH between legs & behind was shrinking. Then I ended up in the hospital where they stopped the drug to see if it was interfering with blood pressure meds. The HH returned with a vengeance! So I called dermatologist to have doctor put me back on the compound, which he did. Now I'm just waiting and praying it will start working again. Just 2 days without compound made such a difference. If your dermatologist will try this one on you, don't let anybody take you off it. It may take a month or more to start working but be patient and I hope it will work for you. It was miraculous how it worked and I intend to keep taking it now and praying it will work again.

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The naltrexone definitely sounds like something to ask the doctor about!

Welcome to Connect, @huitzilin76,

I really like your sense of humor, especially when you shared, "After scratching my head (and a few other places) for about 12 years…" Maintaining a positive attitude while struggling with your health takes effort, but it certainly helps!
I'm so glad you connected with @danavs – thanks so much for sharing your insights, @danavs – and I do hope @dsl1 @christy5348 and others will join in as well.

Naltrexone (brand name Revia or Vivitrol) is a medication that blocks the effects of opiates,and narcotics. Originally used to treat dependence on opiate drugs, it has also been approved by the U.S. Food and Drug Administration (FDA) as treatment for alcohol dependence, and now, as an anti-inflammatory treatment for chronic pain and Hailey-Hailey disease, https://www.ncbi.nlm.nih.gov/pubmed/28768313

@huitzilin76, how has the tacrolimus ointment helped? Does it relieve the pain?

@kanaazpereira

Welcome to Connect, @huitzilin76,

I really like your sense of humor, especially when you shared, "After scratching my head (and a few other places) for about 12 years…" Maintaining a positive attitude while struggling with your health takes effort, but it certainly helps!
I'm so glad you connected with @danavs – thanks so much for sharing your insights, @danavs – and I do hope @dsl1 @christy5348 and others will join in as well.

Naltrexone (brand name Revia or Vivitrol) is a medication that blocks the effects of opiates,and narcotics. Originally used to treat dependence on opiate drugs, it has also been approved by the U.S. Food and Drug Administration (FDA) as treatment for alcohol dependence, and now, as an anti-inflammatory treatment for chronic pain and Hailey-Hailey disease, https://www.ncbi.nlm.nih.gov/pubmed/28768313

@huitzilin76, how has the tacrolimus ointment helped? Does it relieve the pain?

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Thanks, @kanaazpereira! About the tacrolimus, I think it is mostly effective, but not so much against acute flare-ups. When that happens, I have a clobetasol ointment that helps to ease irritation, although I hesitate to use it regularly because of the subsequent thinning of the skin.

In my opinion, what has really helped me the most, especially in summer months, has been the oral glycopyrrolate. It really cuts down on sweating, and that makes a huge difference. Just two days without it lead to a dramatic worsening of HH.

I just ordered a tube of terrasil online, hoping that at least the anesthetic qualities will ease comfort as the current flare-up diminishes. It comes in tomorrow, so I'll update soon to let you all know if it's helpful at all.

Thank you all for this page. I have HHD also, dx with it 6yrs ago and I have not gotten much relief from with meds I've been put on. Not much time in between flare ups. Went to my primary care doc 2 weeks ago and got cortisone injection and pills plus antibiotics and antifungal, it worked for the duration of the treatment and about 4days after, but flare up came right back. Im so tired of the pain, itching and smell of this disease. I have it all over my body… between my legs, under my breasts, on my neck, under my armpits, sometime under my gluteal folds, and right now on my labia and groin. I need help. I just asked my pcp to refer me to a dermatologist. Im pretty new to the area and have not established a dermatologist yet. These flare ups are making it hard for me to work..I work in pain all the time. I hope I can get some relief soon….and my poor husband is suffering too due to lack of intimacy. I am frustrated. Please pray for me. I am using clobetasol mix with mupirocin, but it doesn't seem to be helping anymore and my skin is getting very thin. I can't even cover up the rash with any kind bandaid as removing the bandaid peels my healthy skin off so I'm left to only use foam bordered dressings to cover the areas so I work with alittle relief from the pain. The foam bordered dressings are so expensive. I buy them on Amazon because the medical supply stores are outrageous with their prices. I hope the dermatologist that I will be referred to knows about this disease. I am willing to try anything at this point. I have written down all the treatments that are suggested on this page. Thank you all. I am relieved that I'm not the only one who has this diagnosis.

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