Fox-Fordyce disease (apocrine miliaria)
- Jami L Miller, MD
Jami L Miller, MD
- Assistant Professor of Medicine
- Vanderbilt University
Apocrine miliaria, more commonly referred to as Fox-Fordyce disease (FFD), is an uncommon, pruritic skin condition that is postulated to occur when apocrine or apoeccrine sweat ducts become occluded and inflamed (picture 1A-E). FFD was originally described in 1902 by George Henry Fox and John Addison Fordyce as pruritic papules in the axillae . Since then, FFD has been recognized in other body sites containing apocrine glands, including the areolae, anogenital skin, and other areas.
Treatment of FFD is difficult, but often necessary because of associated pruritus. Local therapies, such as topical corticosteroids, topical clindamycin, and topical calcineurin inhibitors, are typically used as initial treatments, with oral and procedural therapies primarily reserved for refractory disease. Limited data prevents conclusions on the relative efficacy of interventions. Recurrence is common after discontinuation of treatment.
The clinical features, diagnosis, and management of FFD will be reviewed here. Eccrine miliaria are reviewed separately. (See "Miliaria".)
The majority of cases of FFD occur in young women. The female to male ratio is 9:1 and the disease most often begins between the ages of 13 and 35 years . There are occasional reports of the development of FFD in prepubertal children and postmenopausal women [3-5]. There is no racial predilection.
The pathogenesis of FFD is not fully understood, but appears to involve occlusion of sweat glands. There are three types of sweat glands in humans: apocrine, apoeccrine, and eccrine glands . Apocrine glands are the largest sweat glands and are found on the axillae, eyelids, areola, nipple, external auditory meatus, and periumbilical and anogenital skin. The secretory ducts of apocrine glands open onto the infundibulum of hair follicles. Apoeccrine glands are of intermediate size, are primarily found in the axillae, and have secretory ducts that open directly onto the skin surface. Eccrine glands, the smallest and most widely distributed sweat glands, do not play a role in FFD.
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- CLINICAL FEATURES
- DIFFERENTIAL DIAGNOSIS
- CLINICAL COURSE
- First-line therapy
- - Topical corticosteroids
- - Topical clindamycin
- - Topical calcineurin inhibitors
- Second-line therapy
- - Topical retinoids
- - Intralesional corticosteroids
- Refractory disease
- SUMMARY AND RECOMMENDATIONS