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Fox-Fordyce disease
See also in: Anogenital
Other Resources UpToDate PubMed

Fox-Fordyce disease

See also in: Anogenital
Contributors: Vivian Wong MD, PhD, Mat Davey MD, Mary Gail Mercurio MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Fox-Fordyce disease is a rare inflammatory condition of apocrine gland-bearing regions caused by obliteration of the follicular infundibulum with keratin. The etiology is unknown, although an endocrine role has been postulated. The disease manifests as intensely pruritic, skin-colored or keratotic papules in the axillary, anogenital, umbilical, and periareolar areas. Heat, humidity, stress, and other external stimuli such as laser hair removal may worsen symptoms and lead to appearance of new papules.

It is most common in females between puberty and age 35. Less commonly, it can affect male patients, prepubescent girls, and postmenopausal women. It may flare perimenstrually and improve during pregnancy and after menopause.

Codes

ICD10CM:
L75.2 – Apocrine miliaria

SNOMEDCT:
65038009 – Fox-Fordyce disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:01/31/2021
Last Updated:02/01/2021
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Patient Information for Fox-Fordyce disease
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Contributors: Lindsey Ayanruoh, Chesahna Kindred MD

Overview

Fox-Fordyce disease is a chronic inflammatory condition of the apocrine gland characterized by itchy red, purplish, or brown bumps, commonly occurring around the hair follicles of the armpits, navel, genital area, around the anus, and around the areolae.

The cause is unclear; however, it is thought to involve blockage of the duct of the affected apocrine glands. As a result, ducts rupture and inflammation occurs.

Exercise, sexual activity, sweating, and overproduction of androgens may worsen the disease. It may improve or worsen with pregnancy.

Fox-Fordyce disease is very itchy and can interfere with quality of life. Chronic scratching can result in skin thickening as well as darkening of skin. 

This condition may worsen in summer months due to increased sweat production.

Who’s At Risk

Fox-Fordyce disease primarily affects women aged 13-35 years and improves after menopause.

Signs & Symptoms

Smooth, round, dome-shaped 2-3 mm bumps (papules) are found in a linear pattern in apocrine-rich gland areas of the body, including the armpits, areolae, anogenital region, and navel.

Self-Care Guidelines

  • Wear loose clothing.
  • Avoid hot environments.
  • Over-the-counter itch creams that contain menthol, phenol, or pramoxine can be helpful. Keep these in the refrigerator as the application of cool creams can provide even better relief of itch.
  • Oral antihistamines such as cetirizine or diphenhydramine can further help to treat the itch.

When to Seek Medical Care

See a doctor if you are menstruating and papules have appeared in your axillary, areolar, perianal, or genital regions.

Conditions that cause itch can be very distressing. If you are experiencing itching in any of these areas and it is causing stress or anxiety, see your dermatologist.

Treatments

  • Topical corticosteroid cream or lotion for the affected area.
  • Topical calcineurin inhibitors, such as tacrolimus.
  • Prescription oral antihistamines, eg, hydroxyzine.
  • Tretinoin 0.05% cream and hydrocortisone 1% cream applied every other night for six weeks.
  • Botulinum toxin, lasers, or surgery are sometimes used in severe cases that do not respond to the above treatments.
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Fox-Fordyce disease
See also in: Anogenital
A medical illustration showing key findings of Fox-Fordyce disease : Areola, Axilla, Female genital, Follicular configuration, Intertriginous distribution, Pruritus, Tiny papules
Clinical image of Fox-Fordyce disease - imageId=96390. Click to open in gallery.  caption: 'Numerous hyperpigmented, monomophic, follicular-based papules in the axilla.'
Numerous hyperpigmented, monomophic, follicular-based papules in the axilla.
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