Fox-Fordyce disease

What is Fox-Fordyce disease?

Fox-Fordyce disease is a rare skin disorder that occurs mainly in women between the ages of 13 and 35 years. However, it sometimes affects males and children. The condition is also referred to as ‘apocrine duct occlusion’ and ‘sweat retention disease’.

The condition is characterised by the development of itchy bumps around the hair follicles of the underarm area, pubic region, and around the nipples. It results from inflammation of the apocrine sweat glands, which are found only in these areas.

What are the signs and symptoms of Fox-Fordyce disease?

Fox-Fordyce disease may result in very intense itch that disturbs sleep, but in some cases does not result in any symptoms. The condition frequently occurs under conditions of heat, humidity, friction and stress. Common features include:

• Dome-shaped flesh-coloured to small reddish papules affecting almost every hair follicle in the area
• Darkened, thickened and dry skin as a consequence of scratching (lichenification)
• Reduced or absent sweating in the affected area.

The condition may persist for many years. In some instances, it may clear up in pregnancy for unknown reasons. In others, it may resolve at the menopause (but it may also persist afterwards).

Fox-Fordyce disease

Fox-Fordyce disease

Fox-Fordyce disease

Fox-Fordyce disease

What causes Fox-Fordyce disease?

The cause of Fox-Fordyce disease is unknown. For some reason, apocrine sweat becomes trapped as a scaly plug forms in the hair follicle. The apocrine sweat ducts rupture, leak and become inflamed, resulting in intense itching.

Factors identified as playing a part in the development of the condition include:

• Emotional and hormonal influences
• Alterations in sweat components

How is the diagnosis made?

Diagnosis is made on patient history and clinical appearance of the rash.

What is the treatment for Fox-Fordyce disease?

There is no cure for Fox-Fordyce disease. Medical treatments that have been used with varying degrees of success include:

• Topical retinoids
• Topical steroids
• Oral antibiotics
• Clindamycin solution
• Antiandrogenic hormonal therapy

Other forms of treatment used are ultraviolet radiation (phototherapy), dermabrasion, liposuction and surgical excision.