Última actualización:
September 02, 2010
Años publicados: 1993, 2000, 2010
NORD gratefully acknowledges Timothy H. McCalmont, MD, Departments of Pathology and Dermatology, University of California, San Francisco, for assistance in the preparation of this report.
Fox-Fordyce disease is a rare skin disorder that primarily affects women. The disorder is characterized by intense itching especially in the underarm area, the pubic area and around the nipples. In Fox-Fordyce disease abnormalities affecting the apocrine sweat glands causes inflammation, and enlargement of the glands and the characteristic intense itching. Skin near an affected area may become darkened and dry and multiple, small, raised bumps (papules) may develop. Hair follicles in the affected area can become secondarily damaged, resulting in hair loss. The exact cause of Fox-Fordyce disease is unknown.
The symptoms of Fox-Fordyce disease may appear suddenly usually following conditions of heat, humidity or friction. The disease is characterized by an eruption of multiple, small, raised bumps on the skin near the apocrine glands.
The apocrine glands are specialized sweat glands that play a pheromonic role in animals; a similar role has been postulated in humans. Pheromones are chemicals secreted by animals that influence social or sexual behavior of other animals of that species. Apocrine glands respond to sex and stress stimuli. Apocrine glands become extremely active during puberty. Most apocrine glands are found in the armpits or the groin. They may also be found by the nipples, external ear canal, eyelids, and around the bellybutton.
The papules are usually skin-colored, but may be yellowish or reddish in color. They are usually dome-shaped and smooth. Affected areas usually have many small papules. Papules are most often found in the armpits (axillae). The affected areas are often extremely itchy (pruritus) and sweating in these areas may also be absent (anhidrosis). Itching may be mild or may be severe enough to disturb sleep. Hairs within follicles in the affected area may fall out.
The exact cause of Fox-Fordyce disease is unknown. Researchers have speculated that obstruction of the apocrine gland ducts is necessary for the development of Fox-Fordyce disease, but studies have not been able to definitely confirm this theory. Researchers speculate that blocked ducts rupture causing inflammation where the duct comes close to the hair follicle. The inflammatory reaction around hair follicles includes specialized leukocytes (white blood cells) that engulf extruded secretory debris.
Researchers have also speculated that additional factors such as hormonal or genetic ones may play a role in the development of Fox-Fordyce disease. However, research into the cause(s) of Fox-Fordyce disease has not yielded any definitive answers as yet. More research is necessary to determine the exact cause of Fox-Fordyce disease.
Fox-Fordyce disease occurs primarily in women between 13 and 35 years of age. However, in rare cases, it can affect men and children and women who are post-menopausal. Some reports place the ration of affected women to men at 9:1. The incidence of Fox-Fordyce disease is unknown. Heat, humidity, stress and exercise have been noted as exacerbating factors. In addition, Fox-Fordyce disease may be more severe during menstruation and tends to disappear (spontaneously resolve) during pregnancy. Fox-Fordyce disease was first described in the medical literature in 1902.
A diagnosis of Fox-Fordyce disease is made based upon identification of characteristic symptoms (i.e., papular eruptions on apocrine gland areas), a detailed patient history, and a thorough clinical evaluation. Surgical removal and microscopic evaluation (biopsy) of affected tissue may be useful in obtaining a diagnosis. An experienced dermatopathologist will be necessary to correctly diagnose the disease from a biopsy.
Treatment
The treatment of Fox-Fordyce disease is directed toward the specific symptoms that are apparent in each individual. Consultation with a dermatologist is recommended. Specific therapies that have been used include estrogen hormones, oral retinoids, steroid creams, and topical antibiotics. No therapy is universally effective in all patients.
Estrogen hormones, usually given as part of estrogen-based oral contraceptives, have been most effective in treating women with Fox-Fordyce disease. Less effective therapies include oral retinoids (such as tretinoin), corticosteroid creams and topical antibiotics (such as clindamycin) have been beneficial in some cases while ineffective in others. Some of these therapies may be associated with irritation, limiting their ability to be used a long-term therapy.
Some women with Fox-Fordyce disease see a significant improvement or resolution of the disease during pregnancy.
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TEXTBOOKS
James WD, Berger TG, Elston DM. Eds. Andrew’s Diseases of the Skin: Clinical Dermatology. 10th ed. Saunders. 2005:779-780.
Elgart ML. Fox-Fordyce Disease. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:114.
Sahn EE, ed. Dermatology Pearls. Adult and Pediatric. Handley & Belfus, Inc. Philadelphia, PA. 1999:222-223.
JOURNAL ARTICLES
Kao PH, Hsu CK, Lee JY. Clinicopatholgoical study of Fox-Fordyce disease. J Dermatol. 2009;36:485-490.
Kineston DP, Martin KO. Pruritic axillary papules. Fox-Fordyce disease. Am Fam Physician. 2008;77:1735-1736.
Helm TN, Chen PW. Fox-Fordyce disease. Cutis. 2002;69:335, 342.
Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol. 1995;131:1112-1113.
Effendy I, et al., Fox-Fordyce disease in a male patient–response to oral retinoid treatment. Clin Exp Dermatol. 1994;19:67-69.
Monk BE., Fordyce spots responding to isotretinoin therapy. Br J Dermatol. 1993;129:355.
Feldmann R, et al., Fox-Fordyce disease: successful treatment with topical clindamycin in alcohol propylene glycol solution. Dermatology. 1992;184:310-13.
FROM THE INTERNET
White SW, Gorman CR. Fox-Fordyce Disease. Emedicine Journal, October 30, 2009. Available at: https://emedicine.medscape.com/article/1070560-overview Accessed on: April 17, 2010.
Livet MO. Triose-phosphate isomerase deficiency. Orphanet encyclopedia, September 2003. Available at: https://www.orpha.net/data/patho/GB/uk-TPI.pdf Accessed on: April 4, 2007.
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