Alopecia universalis

The exact cause of AU is unknown. AU is an advanced form of alopecia areata (AA), a condition that leads to round patches of hair loss. The most widely accepted hypothesis is that AA is an autoimmune condition in which a person's immune system mistakenly attacks the hair follicles.

While genetic studies have found that AA and AU are associated with several immune-related genes, they are likely ultimately caused by the interaction of multiple genetic and environmental factors. This means that even if someone inherits a genetic predisposition to the condition (susceptibility), they may not develop the condition unless something in the environment triggers its onset. However, the exact role of environmental factors is yet to be determined. Factors that may trigger the onset or recurrence of hair loss may include a viral infection, trauma, hormonal changes, and emotional or physical stress.

A diagnosis of AU is usually based on the signs and symptoms present. In rare cases, a scalp biopsy may be needed to confirm the diagnosis.

AU is believed to be a multifactorial condition, which means it is likely caused by a combination of environmental triggers and genetic predisposition (susceptibility). While a predisposition can be inherited and some people with AU have a family history, the condition itself is not thought to be inherited.

The course of AU is highly unpredictable, and this uncertainty is one of the most difficult and frustrating aspects of the disease. People with AU may continue to lose hair, or hair loss may stop. Hair that has already been lost may or may not grow back. It has been estimated that only about 10% of patients experience full recovery.

According to Orphanet's Report Series on the prevalence and incidence of rare diseases from 2017, the estimated prevalence of alopecia universalis, based on European data, is 25/100,000 people (which is approximately 1 in 4000 people). We are not aware of statistics specific to the United States.

To our knowledge, estimates of the number of people with alopecia areata who eventually develop alopecia unversalis or totalis range from 7% to 25%.

No therapy has been found to work for everyone who has alopecia universalis (AU) which makes managing AU challenging. Although multiple treatments have been explored, no therapy is currently approved by the United States Food and Drug Administration (FDA). Some promising therapies include:

• diphenylcyclopropenone (DPCP)
• squaric acid dibutylester (SADBE)
• photodynamic therapy
• steroids
• cyclosporine in combination with methylprednisolone (a steroid)

There are several recent studies showing that a class of medication known as JAK inhibitors, which includes Tofacitinib and Ruxolitinib, are effective in alopecia areata, including AU. However, JAK inhibitors have not yet been approved by the FDA for use in skin conditions.

In some people with AU, hair regrowth occurs without treatment, sometimes after many years.

There are steps that can be taken to decrease the chance of getting too much sun and minimize other discomforts related to having no hair. These may include:

• Using sunscreen on the scalp, face, and all areas of the skin exposed to the sun
• Wearing eyeglasses or sunglasses to protect the eyes from too much sun and from dust and debris when eyebrows or eyelashes are missing
• Wearing wigs, caps, or scarves to protect the scalp from the sun and keep the head warm
• Applying an ointment inside the nostrils to keep them moisturized and help to protect against organisms invading the nose when nostril hair is missing

Many other treatments have been reported to have variable response rates in small studies in alopecia areata. These include latanoprost, nitrogen mustard, massage and relaxation, isoprinosine, acupuncture, and aromatherapy, among others.