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Fox Fordyce Disease, also known as Sweat Retention Disease, is a very uncommon acne-like skin condition, when inflamed red pimple-like spots or lesions appear on the armpits, pubis, and area around the nipples.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Apocrine Duct Occlusion
  • Fox-Fordyce Syndrome
  • Sweat Retention Disease

What is Fox-Fordyce Disease? (Definition/Background Information)

  • Fox-Fordyce Disease, also known as Sweat Retention Disease, is a very uncommon acne-like skin condition, when inflamed red pimple-like spots or lesions appear on the armpits, pubis, and area around the nipples. Fox Fordyce Disease is almost exclusively observed in young women
  • This condition occurs when tiny sweat glands (specifically the apocrine glands) become clogged around the hair follicle, trapping sweat inside. These rupture and inflame, causing severe itching. The skin turns dry, leathery, flaky on continuous scratching
  • The specific cause of the disease is unknown, but many contributory factors are thought to play a role. These include hormonal changes, emotional stress, weather conditions, and genetic influences

Who gets Fox-Fordyce Disease? (Age and Sex Distribution)

  • Fox Fordyce Disease is ordinarily seen in young women between 13-35 years of age. Females below or above this age group are rarely affected. Pregnant women are also least affected by this disease
  • Men may be affected by this skin condition, although it is infrequently noted. The male-female ratio of this disorder is around 1:9
  • Male children and young adult men, have been observed with this disorder; however, this is quite uncommon
  • Fox Fordyce Disease is not confined to any geographical region or ethnic group

What are the Risk Factors for Fox-Fordyce Disease? (Predisposing Factors)

Fox-Fordyce Disease is a relatively unknown condition. Most of the suggested risk factors are based on speculation, and have not been validated. These include:

  • Changes in the hormonal state, especially during puberty and menstruation
  • Genetic factors; individuals with a positive family medical history
  • Emotional stresses at work, at home
  • Hot humid conditions
  • Constant friction between the skin and the clothes one wear

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Fox-Fordyce Disease? (Etiology)

The specific causative factors that define the development of Fox Fordyce Disease are still being researched into. The disease shows a spontaneous onset.

  • The roots of the hair (called follicles) at the pubic region, the underarms, and those on the breast surrounding the nipples, are attached to a type of sweat glands, known as apocrine glands
  • These glands get clogged due to certain internal or external conditions, leading to a buildup of secretions and dead cells. These ultimately rupture around the hair follicles, causing itching and inflammation
  • The blocked hair follicles appear as a small, red, and inflamed, surface lumps or blisters that break and ooze. No bacterial (or any other microorganism-related) infection is observed
  • Hormonal changes, emotional mood-related triggers, or changes to the sweat constituents are supposedly key factors that initiate Fox-Fordyce Disease. These factors may have a worsening effect on someone already with the disorder
  • A tropical environment (hot-humid weather) can aggravate the skin condition

What are the Signs and Symptoms of Fox-Fordyce Disease?

The apocrine glands are mainly present on the breasts, the groin, and armpits (and some other areas). Hence, these form the primary locations for lesion formation in Fox-Fordyce Syndrome. The signs and symptoms include:

  • Red- or buff-colored (sometimes yellow-colored) tiny eruptions, which are smooth, spherical (dome-shaped) in appearance
  • Slowly the skin turns dry, coarse, scaly, or thick, due to intense itching caused by rupture of the tiny blisters. Nighttime itching (while sleeping) is frequent
  • Hair loss, reduced/absent sweating, is felt around the affected area
  • In some individuals, the itching is mild, or there may not be any symptoms

How is Fox-Fordyce Disease Diagnosed?

  • The physician or dermatologist may ask many questions related to the individual’s family medical history, age, stress factors, current medications, cosmetics, body lotions used, other medical conditions, infections present, etc.
  • However, Fox-Fordyce Disease can be diagnosed by a simple physical examination, and on performing a skin biopsy

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis

What are the possible Complications of Fox-Fordyce Disease?

Some complications of Fox-Fordyce Disease include:

  • Increased emotional stress affecting one’s performance at office or school
  • Scarring of the skin requiring cosmetic treatment
  • Side effects of the treatment medication used such as local applications (antiseptic, anti-inflammatory)
  • Possible development of bacterial (or other pathogenic) infections
  • Recurrence of the condition

How is Fox-Fordyce Disease Treated?

Several passive and active symptomatic treatment remedies that address Fox-Fordyce Disease exist. All such measures may or may not work; there is also no particular cure for the condition. The prescription is dependent upon the intensity of the condition. Long-term treatment therapy may be required in some cases.

The treatment measures include:

  • Use of antiseptic, antibiotic topical applications, anti-inflammatory gels, lotions and corticosteroid creams. Topical retinoids are used for removing the blocking adherent skin cells  from the openings of the hair follicles
  • Medications used include antibiotics, topical retinoids, hormones (testosterone, estrogen, oral contraceptives for women), and steroids
  • Phototherapy: Controlled exposure to controlled doses of ultraviolet light
  • Surgical procedures using laser
  • Taking care of oneself, being clean and hygienic, avoiding the urge to touch/break blisters, drinking lots of water, etc. may be helpful
  • However, some treatment chemicals, topical applications, and medications have caused more irritation to the patients than relief
  • Several alternative medicines, herbal topical ointments exist; but, these may not have medical acceptance due to a lack of reliable, verifiable and/or tested information

How can Fox-Fordyce Disease be Prevented?

  • Fox-Fordyce Disease is a rare skin anomaly that occurs due to various medical factors and external conditions that might occur together. It cannot be prevented, but the factors that can be controlled have to be considered
  • It is thought that certain factors contribute to the abnormality, or aggravate its state/condition. Nevertheless, Fox-Fordyce Disease is still not fully understood
  • Avoiding certain conditions (like hot-wet weather) or physical activities (sports, exercise) that cause excessive sweating may be advantageous. Individuals with the condition, seem to prefer swimming as an exercise

What is the Prognosis of Fox-Fordyce Disease? (Outcomes/Resolutions)

  • There is no cure for Fox Fordyce Disease. The attempted resolution is symptomatic treatment, and control of conditions that aggravate the skin disorder
  • Normally, the skin condition resolves itself after a certain period of time. However, this may not be true in all cases and long-term therapy may be needed
  • Individuals vary, and hence the treatment methods chosen should be based on one’s level of comfort and cost considerations

Additional and Relevant Useful Information for Fox-Fordyce Disease:

There is no evidence to implicate any food or food-related products, as having an adverse influence on Fox-Fordyce Disease.

What are some Useful Resources for Additional Information?

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue Danbury, CT 06810
Phone: (203) 744-0100
Toll-Free: (800) 999-6673
Fax: (203) 798-2291
Email: orphan@rarediseases.org
Website: http://www.rarediseases.org

References and Information Sources used for the Article:

http://www.dermnetnz.org/hair-nails-sweat/fox-fordyce.html (accessed on 11/24/12)

http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/941/printFullReport (accessed on 11/24/12)

Helpful Peer-Reviewed Medical Articles:

Brain, R. T. (1937). Fox-Fordyce Disease. Proceedings of the Royal Society of Medicine, 30(11), 1336.

Dowling, G. B. (1936). Fox-Fordyce Disease. Proceedings of the Royal Society of Medicine, 29(8), 919.

Duckworth, G. (1943). ? Fox-Fordyce Disease. Proceedings of the Royal Society of Medicine, 36(6), 292.

Ozcan, A., Senol, M., Aydin, N. E., Karaca, S., & Sener, S. (2003). Fox‐Fordyce disease. Journal of the European Academy of Dermatology and Venereology, 17(2), 244-245.

Sherry-Dottridge, F. G. (1944). Fox-Fordyce Disease. Proceedings of the Royal Society of Medicine, 37(11), 643.

Yost, J., Robinson, M., & Meehan, S. A. (2012). Fox-fordyce disease. Dermatology online journal, 18(12).

Blasco-Morente, G., Naranjo-Díaz, M. J., Pérez-López, I., Martínez-López, A., Ruiz-Villaverde, R., & Aneiros-Fernández, J. (2016). Fox-Fordyce Disease. Sultan Qaboos University Medical Journal [SQUMJ], 16(1), 119-120.

RILEY, G. M. Fox-Fordyce Disease.

Jaiswal, A. K., Vaishampayan, S., Verma, R., & Baveja, S. (1999). Fox-Fordyce Disease. Indian Journal of Dermatology, 44(2), 94.

Effendy, I., Ossowski, B., & Happle, R. (1994). Fox–Fordyce disease in a male patient–response to oral retinoid treatment. Clinical and experimental dermatology, 19(1), 67-69.

Miller, M. L., Harford, R. R., & Yeager, J. K. (1995). Fox-Fordyce disease treated with topical clindamycin solution. Archives of dermatology, 131(10), 1112-1113.

Feldmann, R., Masouye, I., Chavaz, P., & Saurat, J. H. (1992). Fox-Fordyce disease: successful treatment with topical clindamycin in alcoholic propylene glycol solution. Dermatology, 184(4), 310-313.