‘I have had Fox-Fordyce for many years and it is DRIVING me crazy.’

‘The problem is it’s also very itchy on my testicles because of this. The itching creates wounds and there is no cure for this.’

‘I have had it for a while, it is so itchy, I have no clue what to do.’

Fox-Fordyce disease, also known as apocrine miliaria, is a rare, debilitating skin condition. If someone has it, they can have itchy, dome-shaped, inflamed areas in their armpit, on their groin, and around their breasts. Although rare, Fox-Fordyce disease can have a significant impact on the quality of life, often leading to physical disability and affecting someone’s mental health and social life. This makes it incredibly important to increase awareness of the disease to promote better ways of treatment and disease management.  

Overview of Fox-Fordyce disease

Fox-Fordyce disease typically affects people assigned female at birth (AFAB) between the ages of 13 and 35 years, often linked to hormonal fluctuations during puberty, pregnancy, contraception-taking and pre-menopause. Most people have symptoms, although a few can remain asymptomatic.¹ It is not well-known or understood, which can lead to missed diagnoses and misdiagnoses. According to the National Organisation for Rare Diseases (NORD), the exact causes of Fox-Fordyce disease are unknown. However, scientists suggest that apocrine ducts, a narrow tube of the apocrine gland, need to be clogged with keratin for the mechanisms that cause the symptoms of this disease to develop. While AFAB are commonly diagnosed with this disease, people assigned male at birth (AMAB) may also have it, albeit much rarer. Several studies concluded the ratio of AFAB to AMAB is 9 to 1.² Getting an accurate diagnosis is challenging, and it can often be mistaken for other conditions with similar symptoms, such as miliaria (heat rash), hidradenitis suppurativa, keratosis follicularis, and Hailey-Hailey disease.

Living with this skin condition has multiple effects on the quality of life.

The effects of Fox-Fordyce disease on the quality of life

Physical effects 

Fox-Fordyce disease has debilitating effects on someone’s physical well-being. As it is chronic, its continuous itching and inflammation lead to severe discomfort, which lasts throughout the day and is associated with challenges in falling asleep. The inflammation is caused by white blood cells accumulating in the apocrine glands, which can sometimes generate white pus to leak out. This makes it harder to manage, and the patients may have to give up activities that were once enjoyable to prevent infection or more pain. The person affected by this condition may have to or already has given up on physical activities that cause friction, which could range from running to putting on clothes. Sometimes it may reach the point where everything feels painful, especially if all the apocrine glands in the body are inflamed.³

Psychological and emotional effects

Whilst this disease has mainly physical symptoms, the emotional and psychological impact of Fox-Fordyce disease should not be overlooked. Many people with this disease are going through hormonal transitions as well as adjusting to their new bodies and societal norms around them.⁴ The added pressure of the physical changes brought about by Fox-Fordyce disease may cause low self-esteem, which affects the way they interact and communicate with others. This condition is highly linked to developing depression and anxiety, which negatively affect a person’s quality of life.⁵ 

Social impact of Fox-Fordyce disease

The social impact of Fox-Fordyce disease is closely linked to the psychological impacts. Some individuals who have Fox-Fordyce disease may be withdrawn from intimate or sexual relationships due to physical discomfort and/or anxiety they feel about the inflammation around their intimate areas.³ They might also worsen their condition due to long, uncomfortable hours at work, which may increase friction, making them hyperaware of the inflammation, and contributing to reduced confidence.⁵ 

Diagnostic challenges and their impact 

The main challenge in diagnosing Fox-Fordyce disease is that it is so rare that many healthcare professionals and people around you don’t know about it. There are no proper diagnostic tools available, and often the diagnosis is made only when it is obvious to your clinician through examination that you have Fox-Fordyce disease. However, many diseases are similar, so misdiagnosis is very common. It leads to frustration due to inefficient treatment and management, as misdiagnosis may result in treating the symptoms rather than the actual problem. Since very few people have this condition, there is also a stigma surrounding it, mainly due to a lack of knowledge and education around it. 

Treatment challenges and their effects

The problem with treatment strategies is that, due to the lack of solid knowledge or treatment trials about the disease, treatments rely on a trial-and-error basis. Current therapies are based on successful case reports and are presented below.

Topical therapies

Topical steroids relieve itching, however, they can cause thinning of the skin, another debilitating side effect. There is also a risk of topical steroid withdrawal syndrome, a skin condition caused by steroid use. It can happen even if someone uses the steroid cream for a few weeks and takes a break for a few weeks before restarting the treatment, like doctors recommend. Topical retinoids can also be used, but they risk skin irritation.⁶ On the bright side, topical antibiotics have been successful in some patients.

Oral treatments

There are not many viable treatments, with the main options being the estrogen-containing contraceptive pill, antihistamines and oral tretinoin. The pill has been proven to be effective in balancing hormones. However, if someone stops taking it, there is a high chance of recurrence. Similarly, whilst oral tretinoin improves symptoms, it is advised to use it with caution in childbearing age due to its severe birth defect effects in babies. Conversely, antihistamines can be taken to combat itching, but they may cause drowsinesssomeone.⁶

Surgical treatments

 Patients can use botulinum toxin/Botox injections to relieve the itching or stop the sweating around affected regions. This comes with its own risks and side effects.¹

All in all, the treatments aren’t effective enough and may lead to more negative consequences, either physically, psychosocially, or both. 

Coping strategies

While the research and development field is sparse with robust treatment and management systems, there are personal measures that can be taken to improve your health and wellbeing whilst living with Fox-ordyce disease.

• Try to control the environment around your body in hot and humid spaces, like eearing looser, more comfortable clothing and avoiding places prone to making you sweat
• Use gentle-to-skin products for your skincare
• Try to reduce stress, as it can trigger your flare-ups 
• Avoid scratching as much as you can, as this may complicate your inflammation and may likely cause infection
• Join a support group (a list of regional and national support groups can be found here)

FAQs

Is Fox-Fordyce disease a sexually transmitted disease (STI)?

No, it is not an STI. It is a non-contagious, rare skin disease that affects apocrine sweat glands, found on the groin, armpits, and breasts. STIs are usually caused by a virus or bacteria. Conversely, Fox-Fordyce disease is suggested to be caused by hormonal changes.

What causes Fox-Fordyce disease?

There are four main causes of Fox-Fordyce disease. You could be genetically susceptible to developing it due to inheriting it from a family member. Another reason, which is suggested to be the main reason, is hormonal fluctuations that happen in people assigned female at birth between the ages they start puberty and their experience of menopause. It could also flare up during pregnancy and with the use of contraceptives. Other factors that typically trigger a normal itch, such as stress, excitement and sweating due to exercise, can trigger the itch experienced by people with this condition. Lastly, recent findings have shown that laser hair removal can also lead to this condition due to the damage and subsequent irritation that lasers cause.

Summary

Fox-Fordyce disease, also known as apocrine miliaria, is a rare, non-contagious skin condition that is extremely under-researched, resulting in inadequate diagnostic measures and treatments.  This negatively affects patients’ quality of life, often leading to social isolation, low self-esteem, depression and anxiety. This effect, combined with the physical discomfort and disability caused by the itching, irritation, and inflammation in regions where the apocrine sweat glands are located, such as the groin, armpits, and around the breasts. The lack of knowledge and medical education around it has contributed to the stigma associated with having the disease. The most reliable remedies include avoiding triggers such as humidity, heat, and stress, as well as joining support groups to help with resilience, especially when trying different therapeutic treatments for a cure.