What is Hailey and Hailey Disease?

Exploring Hailey-Hailey Disease: A Comprehensive Overview

Hailey-Hailey disease is a rare inherited skin condition, also known as familial benign pemphigus. This genetic disorder primarily affects the skin folds, such as the armpits, neck, and groin area, and tends to worsen during the summer heat.

Even though this condition is not life-threatening, it can significantly disrupt the quality of life. It can make simple situations, like trying to hug a loved one or wearing your favorite dress, difficult. Keep reading to learn about Hailey-Hailey disease's causes, symptoms, diagnosis, and treatment.

Is Hailey and Hailey Disease Contagious?

Unlike most skin disorders, Hailey-Hailey is not contagious. It is an inherited genetic disorder, which is an autosomal dominant disorder. A single mutated gene from either parent is enough to cause the condition. You don't need to inherit two faulty genes; having one is enough to affect you.

What Causes The Hailey and Hailey Disease?

A variation or mutation in the ATP2C1 (ATPase calcium-transporting type 2C member 1) gene on chromosome 3q21-24 causes Hailey-Hailey. This gene contains instructions for making a protein that acts as a calcium and magnesium pump.

Calcium and magnesium ions are essential factors in skin cell structure. The gene mutation in Hailey-Hailey disrupts the production or pumping of calcium in cells. This malfunction does not let skin cells "stick" to one another, making the epidermis fragile. The result is visible blisters and erosions on the skin.

What are the Symptoms of Hailey and Hailey Disease?

The symptoms start to appear during the early 20s, and their severity varies from person to person. The typical symptoms include:

• A red, irritated, and erosive skin rash is in the skin folds. Areas such as the underarms, neck, groin region, and below the breast (for females) are most affected.
• Yellow, crusty blisters over the lesions.
• Cracked skin as a result of these lesions can be painful.
• Burning or stinging sensation on the rashes and blisters.
• Occasional secondary infections, especially bacterial or fungal, can occur on the lesions. That can cause unpleasant body odor.

The signs of Hailey-Hailey disorder are relapsing and remitting. Meaning they come and go. Symptoms can even worsen due to hot and humid weather, friction, sweating, or stress.

How is Hailey and Hailey Disease Diagnosed?

The symptoms of Hailey-Hailey disease mimic usual skin infections, such as eczema or bacterial or fungal infections, which is why it is frequently misdiagnosed. The diagnostic process typically involves:

• Physical exam
• Reviewing family history
• Performing a biopsy on a small part of the affected skin tissue. This procedure reveals abnormal keratin tissue.
   

What are the Treatments for Hailey and Hailey Disease?

Even though there is no cure for Hailey-Hailey disease, there are approaches to help manage it effectively. The treatment approach depends on the severity of the symptoms. It includes:

• Topical steroid or calcineurin inhibitors to reduce swelling or inflammation.
• Botox injections, popular for reducing wrinkles, are used to lessen sweating and friction.
• Oral antibiotics and creams for bacterial and yeast infections.

Lifestyle changes are also key for an improved life with Hailey-Hailey, and some of those changes may include:

• Avoiding the sun
• Carefully cleansing the skin folds twice daily with mild soap.
• Choose loose and comfortable clothing.
• Weight management or fat loss to minimize friction.

If you suspect you have Hailey-Hailey disease, consult a dermatologist immediately. Knowing the symptoms of rare diseases leads to early diagnosis, and more awareness will make managing this condition easier. It is also advisable to have health insurance to secure your health and finances during critical times.

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