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Related Disorders

·         Darier disease

·         Hailey-Hailey disease

·         Palmar-Plantar Keratodermas

·         Pachyonychia Congenita

·         Extensive Epidermal Nevi

·         Erthrokeratodermas

Darier disease is a stubborn rash which usually runs in families. The rash often starts when one is a teenager or older. It most commonly affects the chest, neck, back, ears, forehead, and groin, but may involve other body areas. At times the rash will cause a bad odor.

Darier's is an autosomally dominant inherited mutation in the gene ATP2A2. Family members with confirmed identical ATP2A2 mutations can exhibit differences in the clinical severity of disease, suggesting that other genes or environmental factors affect the expression of Darier's disease. With the discovery of the ATP2A2 gene, performing genetic tests to confirm the diagnosis of Darier's disease is now possible.


 

 

Hailey-Hailey disease
The cause of the disease is a defect in the calcium pump gene which is located on chromosome 3.
This causes the cells of the skin to not adhere together properly, causing the blisters and rashes. There is no cure.

Treatment

Topical steroid preparations have some effect during outbreaks, but do not stop them. Drugs such as antibiotics, antifungals, corticosteroids, dapsone, methotrexate, thalidomide, etretinate, cyclosporine and, most recently, intramuscular alefacept may control the disease but are ineffective for severe chronic or relapsing forms of the disease. Injections of botulinum toxin to inhibit perspiration may help. Maintaining a healthy weight, avoiding heat and friction of affected areas, and keeping the area clean and dry work about as well.

Some have found relief in laser resurfacing that burns off the top layer of the epidermis, allowing healthy non-affected skin to re-grow in its place.

Secondary bacterial, fungal and/or viral infections are common and may exacerbate an outbreak. Some patients have found that outbreaks are triggered by certain foods, hormone cycles and stress.

Palmar-Plantar Keratodermas

Pachyonychia Congenita

Extensive Epidermal Nevi

Erthrokeratodermas

 

 

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