Haily-Haily Disease was first described by the Haily brothers in 1939. It is also called benign chronic pemphigus. Pemphigus is a general term that is used to describe a broad group of rare autoimmune disorders. Autoimmune disorders are those that occur when the body's immune system attacks its body parts or tissues mistakenly. However, Haily-Haily disease is not an autoimmune disease. It is a genetic disorder and usually appears in early adulthood. The gene variant responsible for this disease is ATP2C1.
What Causes The Disease?
Haily-Haily disease is caused by a mutation in the ATP2C1 gene. The gene contains information for producing protein (hSPCA1) that appears to be particularly important for functioning keratinocytes. The hSPCA1 protein also acts as a calcium (Ca++) pump in the cell. Calcium ion helps cells to stick together. When the calcium pump does not act properly, the stickiness of cells gets broken and cells become unable to keep foreign invaders out of the body. As a result, affected cells cause the epidermis to become fragile and it can't tolerate minor trauma. The skin becomes damaged with blisters and erosions. The reason behind the mutation of the protein is still unclear. However, it is understood that the mutation of the protein causes limitation to store adequate calcium. The absence of calcium ions in the affected area is the reason behind the disease. Recent studies state that Haily-Haily disease and Grover's disease show effects in a similar structural system.
Who Is At The Risk?
Both men and women can be affected equally by Haily-Haily disease. About 1 out of 50,000 people are affected in the general population.
Treatment
Haily-Haily disease is a chronic disease with no cure. Current therapeutic strategies aim to settle disease flares, manage symptoms and improve patient's condition. When medication is required, a combination of systemic and topical medications is incorporated. Topical and systemic medications can be employed to help:
• Reduce inflammation and itching
• Fight against bacterial and fungal infections
• Reduce skin lesions
• Reduce flaming at the affected skin
Natural Treatments For Haily-Haily Disease
An important consideration about Haily-Haily disease is that there is no known cure for this disease. So natural treatment focuses on preventing further complications, managing symptoms and improving patient's condition. Some treatment ideas are given to experience physical improvement:
- Use Aloe vera Oil on the affected area. This will reduce your inflammation and irritation by giving you a soothing effect.
- Apply Coconut Oil on the affected skin. It has antibacterial and antifungal properties that will save you from infections. Besides, it works as a moisturizing agent that keeps your affected skin smooth.
- Take your bath mixing Magnesium Chloride/ Potassium Permanganate in water. Both have antiseptic properties that should protect your skin.
- Tea Tree Oil helps reduce redness of the skin.
- Manuka Honey is pretty useful for healing your wound and it has antimicrobial properties.
- Turmeric has antifungal and anti-inflammatory properties.
- Eat Protein-rich Food to repair wounded tissue.
- Keep Omega-3 Fatty Acids rich foods on your diet list. Omega-3 Fatty Acids help reduce inflammation.
- Mix baking soda with water to prepare a paste and rub it in the affected tissue. This may help you to reduce itching.
- Apply a cool compress to the affected area. This will give a soothing effect. Have a break at regular intervals to let your skin recover.
- Eat Probiotic Foods like yogurt and Fermented Foods(Tempeh, Kefir etc). These are helpful to soothe itching of the affected skin.
- Eat Vitamin C and Antioxidant Rich Foods. These can support your skin health.
- Flavonoid Rich Foods(Apple, Berries, Grapes) are helpful to fight against skin allergies.
- Eat Healthy Fats( Avocado, Fatty Fish, Nuts and Seeds) to retain moisture and skin elasticity.
- Eat Water Rich Fruits daily.
- Avoid heat and hot showers.
- Drink more water to avoid dehydration.
- Reduce alcohol drinking.
- Avoid tight clothing.
- Use talc-free powders.
- Avoid processed foods and sugary drinks.
- Have enough rest.
- Lose extra Fats.
Follow the doctor's advice to have more control over the disease.
