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.
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.
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.
The symptoms start to appear during the early 20s, and their severity varies from person to person. The typical symptoms include:
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.
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:
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:
Lifestyle changes are also key for an improved life with Hailey-Hailey, and some of those changes may include:
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.
Star Health offers extensive coverage for critical diseases like Hailey-Hailey under our Star Critical Illness Multipay Insurance Policy. We also provide hassle-free claim settlements with reduced turnaround time across over 14,000 network hospitals in India.
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