eMedicine Specialties > Dermatology > Diseases of the Adnexa

Fox-Fordyce Disease

Author: Stephen W White, MD, Clinical Assistant Professor, Department of Dermatology, George Washington University Hospital; Chief, Sub-section of Dermatology, Suburban Hospital
Coauthor(s): Christopher R Gorman, MD, Bethesda Dermatology, private practice
Contributor Information and Disclosures

Updated: Oct 30, 2009

Introduction

Background

Fox-Fordyce disease is an infrequently occurring chronic pruritic papular eruption that localizes to areas where apocrine glands are found. The etiology of Fox-Fordyce disease currently is unknown. The eponym is based on the 1902 report by G. Fox and J. Fordyce.1

Pathophysiology

Fox-Fordyce disease is a disease of the skin alone. In 1956, Shelley and Levy proposed apocrine miliaria as the cause.2 The observed pathophysiology is a keratin plug in the hair follicle infundibulum obstructing the apocrine acrosyringium and producing an apocrine anhidrosis. Histologically, a rupture of the apocrine excretory duct occurs, and spongiotic inflammation results. Extravasation of sweat and inflammation is postulated to cause the intense itching. Ranalletta et al found that the acrosyringium of the eccrine glands was similarly involved.3

In 2003, Kamada et al published a histopathologic analysis from which they concluded that the 2 types of this disease are (1) an apocrine (follicular) type and (2) an apocrine (nonfollicular) type.4

Frequency

United States

Fox-Fordyce disease is an infrequent condition. Geographic influence is not evident. Many case reports of Fox-Fordyce disease mention heat, humidity, and stress as exacerbating factors.

International

Reports of Fox-Fordyce disease from the United States are the most common; however, a geographic limitation is not evident.

Mortality/Morbidity

Fox-Fordyce disease has no risk of loss of life or limb. Patients often experience severe pruritus. Therefore, the patient's quality of life may be adversely affected.

Race

No racial predilection is evident for Fox-Fordyce disease.

Sex

A distinct predilection for women exists for Fox-Fordyce disease; the female-to-male ratio is 9:1.

Age

Fox-Fordyce disease is most common in women aged 13-35 years; it is rare before or after this age.

Clinical

History

  • Fox-Fordyce disease frequently appears under conditions of heat, humidity, and friction, often appearing suddenly.
  • Many patients present after decades of symptoms.
  • Few patients are asymptomatic.
  • Most patients relate pruritus that disturbs sleep.
  • Changing antiperspirants has not been reported to help.
  • Some patients report diminution of sweating after the onset of symptoms.

Physical

  • The apocrine glands are the site of Fox-Fordyce disease.
  • Lesions are most often found in the axillae, where they tend to be bilateral.
  • Lesions may also affect the periareolar, inframammary, and pubic areas.
  • The primary lesion is a flesh-colored to reddish, smooth, dome-shaped, discrete, and follicular or perifollicular papule.
  • Affected areas usually have many papules. The papules usually appear to affect every follicle in a given area.
  • Excoriations and lichenification may be seen as a consequence of scratching.
  • Sweating is often absent in the affected area.

Causes

  • The definite increased prevalence of Fox-Fordyce disease in women has led to an unproved theory of hormonal influences. Reports of cases of Fox-Fordyce disease in prepubertal girls are evidence against the hormonal theory.5 The exact pathophysiology is still unknown.
  • A number of factors, including (1) emotional and/or hormonal influences and (2) alterations in sweat components, have been implicated in Fox-Fordyce disease.

More on Fox-Fordyce Disease

Overview: Fox-Fordyce Disease
Differential Diagnoses & Workup: Fox-Fordyce Disease
Treatment & Medication: Fox-Fordyce Disease
Follow-up: Fox-Fordyce Disease
References
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References

  1. Fox G, Fordyce J. Two cases of a rare papular disease affecting the axillary region. J Cutan Genito-Urinary Dis. 1902;20:1-5.

  2. Shelley WB, Levy EJ. Apocrine sweat retention in man. II. Fox-Fordyce disease (apocrine miliaria). AMA Arch Derm. Jan 1956;73(1):38-49. [Medline].

  3. Ranalletta M, Rositto A, Drut R. Fox-Fordyce disease in two prepubertal girls: histopathologic demonstration of eccrine sweat gland involvement. Pediatr Dermatol. Jul-Aug 1996;13(4):294-7. [Medline].

  4. Kamada A, Saga K, Jimbow K. Apoeccrine sweat duct obstruction as a cause for Fox-Fordyce disease. J Am Acad Dermatol. Mar 2003;48(3):453-5. [Medline].

  5. Sandhu K, Gupta S, Kanwar AJ. Fox fordyce disease in a prepubertal girl. Pediatr Dermatol. Jan-Feb 2005;22(1):89-90. [Medline].

  6. Stashower ME, Krivda SJ, Turiansky GW. Fox-Fordyce disease: diagnosis with transverse histologic sections. J Am Acad Dermatol. Jan 2000;42(1 Pt 1):89-91. [Medline].

  7. Chae KM, Marschall MA, Marschall SF. Axillary Fox-Fordyce disease treated with liposuction-assisted curettage. Arch Dermatol. Apr 2002;138(4):452-4. [Medline].

  8. Bormate AB Jr, Leboit PE, McCalmont TH. Perifollicular xanthomatosis as the hallmark of axillary Fox-Fordyce disease: an evaluation of histopathologic features of 7 cases. Arch Dermatol. Aug 2008;144(8):1020-4. [Medline].

  9. Macarenco RS, Garces S JC. Dilation of apocrine glands. A forgotten but helpful histopathological clue to the diagnosis of axillary Fox-Fordyce disease. Am J Dermatopathol. Jun 2009;31(4):393-7. [Medline].

  10. Effendy I, Ossowski B, Happle R. Fox-Fordyce disease in a male patient--response to oral retinoid treatment. Clin Exp Dermatol. Jan 1994;19(1):67-9. [Medline].

  11. Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol. Oct 1995;131(10):1112-3. [Medline].

  12. Pock L, Svrckova M, Machackova R, Hercogova J. Pimecrolimus is effective in Fox-Fordyce disease. Int J Dermatol. Sep 2006;45(9):1134-5. [Medline].

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Further Reading

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Keywords

Fox-Fordyce disease, Fox-Fordyce syndrome, apocrine miliaria, chronic pruritic papular eruption, follicular infundibular occlusion

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Contributor Information and Disclosures

Author

Stephen W White, MD, Clinical Assistant Professor, Department of Dermatology, George Washington University Hospital; Chief, Sub-section of Dermatology, Suburban Hospital
Stephen W White, MD is a member of the following medical societies: American Academy of Dermatology, International Society of Dermatology, Society for Investigative Dermatology, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Christopher R Gorman, MD, Bethesda Dermatology, private practice
Christopher R Gorman, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Medical Editor

James Fulton Jr, MD, PhD, Center for Cosmetic Dermatology; Consultant, Vivant Pharmaceuticals, LLC
James Fulton Jr, MD, PhD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American Society for Laser Medicine and Surgery, Dermatology Foundation, International Society of Cosmetic and Laser Surgeons, and Skin Cancer Foundation
Disclosure: vivant pharmaceuticals Ownership interest Consulting

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Mary Farley, MD, Dermatologic Surgeon/Mohs Surgeon, Anne Arundel Surgery Center
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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