Fox-Fordyce Disease

Updated: Aug 14, 2017
  • Author: Christopher R Gorman, MD; Chief Editor: William D James, MD  more...
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Overview

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]

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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]

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Epidemiology

Frequency

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

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.

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Prognosis

Management with topical retinoids and antibiotics has brought some hope to patients with Fox-Fordyce disease for decades. Long-term follow-up studies are not available; therapy may need to be prolonged for a very long time. Acceptable therapy should be safe and relatively inexpensive.

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.

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