Hailey-Hailey Disease Is Interesting.

Hi Everyone! This is Dr. Stahr and here is a picture of a patient I saw last week. I am posting to share some cool skin science! The eruption you see here is called Hailey-Hailey; it is present on this patient's skin because she has a mutation in her DNA that codes for an improperly functioning calcium channel pump on a cellular structure called the Golgi apparatus. So, while the root of the problem lies in a structure you can only see with an extremely powerful microscope, you can definitely see the consequences that this defective structure has on the skin. Usually, this disease is inherited from a parent, but about 30% of the Hailey-Hailey patients will develop this mutation without any parent passing this on.

Hailey-Hailey is a tough problem to treat because we don't really have medicines right now that will fix this problem originating in the DNA. We can help alleviate the symptoms though, so treatment focuses on preventing infection and addressing the inflammation. In the picture below, you will see how this responded to application of anti-inflammatory topical steroids for about two weeks. It is much smoother and far less painful.

That's it for now. I want to say thank you to the patient who graciously granted us permission to share her photographs. We are glad to have a nice opportunity to raise awareness and to do a bit of education on the nature of this unique disease process.

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