Other acantholytic disorders digital illustration

Other acantholytic disorders Save


ICD-10 code: L11

Chapter: Diseases of the skin and subcutaneous tissue

Other Acantholytic Disorders: Symptoms, Causes, and Treatment

Acantholytic disorders, also known as acantholysis, are a group of skin conditions that occur when the cells that hold the skin together break apart. These disorders can cause blisters, sores, and other skin abnormalities. While some acantholytic disorders are well-known, such as pemphigus vulgaris, there are many others that are less common but equally troubling. Here are some of the other acantholytic disorders you should know about:

  1. Hailey-Hailey Disease: This is a rare genetic disorder that causes painful blisters and sores on the skin. It typically affects the groin, neck, and underarms, but can occur anywhere on the body. Hailey-Hailey disease is caused by mutations in the ATP2C1 gene.
  2. Benign Familial Pemphigus: Also known as Hailey-Hailey-like pemphigus, this disorder causes blisters and sores on the skin, similar to Hailey-Hailey disease. However, it is not caused by mutations in the ATP2C1 gene and is not inherited in the same way.
  3. Pemphigus Foliaceus: This is a rare autoimmune disorder that causes blistering and sores on the skin, particularly on the face and scalp. It occurs when the immune system attacks the proteins that hold skin cells together.
  4. Paraneoplastic Pemphigus: This is a rare autoimmune disorder that is associated with certain types of cancer. It causes blisters and sores on the skin, as well as painful ulcers in the mouth and throat.
  5. Drug-Induced Pemphigus: Some medications can cause an acantholytic disorder known as drug-induced pemphigus. This disorder can cause blisters and sores on the skin, as well as painful ulcers in the mouth and throat.

Diagnosing acantholytic disorders can be challenging, as they can be mistaken for other skin conditions. A dermatologist will typically perform a skin biopsy to confirm the diagnosis. Treatment for acantholytic disorders typically involves medications to suppress the immune system and reduce inflammation. In some cases, surgery may be necessary to remove affected areas of skin.

If you suspect that you may have an acantholytic disorder, it is important to seek medical attention as soon as possible. Early diagnosis and treatment can help to prevent complications and improve your quality of life.