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.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Fox GH, Fordyce JA. Two cases of a rare popular disease affecting the axillary region. J Cutan Genitourinary Dis 1902; 20:1.
- SHELLEY WB, LEVY EJ. Apocrine sweat retention in man. II. Fox-Fordyce disease (apocrine miliaria). AMA Arch Derm 1956; 73:38.
- Sandhu K, Gupta S, Kanwar AJ. Fox fordyce disease in a prepubertal girl. Pediatr Dermatol 2005; 22:89.
- Ranalletta M, Rositto A, Drut R. Fox-Fordyce disease in two prepubertal girls: histopathologic demonstration of eccrine sweat gland involvement. Pediatr Dermatol 1996; 13:294.
- Pasricha JS, Nayyar KC. Fox-Fordyce disease in the post-menopausal period treated successfully with electrocoagulation. Dermatologica 1973; 147:271.
- Kamada A, Saga K, Jimbow K. Apoeccrine sweat duct obstruction as a cause for Fox-Fordyce disease. J Am Acad Dermatol 2003; 48:453.
- Helou J, Maatouk I, Moutran R, Obeid G. Fox-Fordyce-like disease following laser hair removal appearing on all treated areas. Lasers Med Sci 2013; 28:1205.
- Yazganoğlu KD, Yazici S, Büyükbabani N, Ozkaya E. Axillary Fox-Fordyce-like disease induced by laser hair removal therapy. J Am Acad Dermatol 2012; 67:e139.
- Tetzlaff MT, Evans K, DeHoratius DM, et al. Fox-Fordyce disease following axillary laser hair removal. Arch Dermatol 2011; 147:573.
- CORNBLEET T. Pregnancy and apocrine gland diseases: hidradenitis, Fox-Fordyce disease. AMA Arch Derm Syphilol 1952; 65:12.
- Ebling FJ. Apocrine glands in health and disorder. Int J Dermatol 1989; 28:508.
- Ioffreda MD. Inflammatory diseases of hair follicles, sweat glands, and cartilage. In: Lever's Histopathology of the Skin, 10th ed, Elder DE, Elenitsas R, Johnson B Jr, et al. (Eds), Lippincott Williams & Wilkins, Philadelphia 2009. p.492.
- 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 2008; 144:1020.
- Stashower ME, Krivda SJ, Turiansky GW. Fox-Fordyce disease: diagnosis with transverse histologic sections. J Am Acad Dermatol 2000; 42:89.
- Sakiyama M, Maeda M, Fujimoto N, Satoh T. Eruptive syringoma localized in intertriginous areas. J Dtsch Dermatol Ges 2014; 12:72.
- George A, Bhatia A, Thomas E. Fox-Fordyce disease: a report of 2 cases responding to topical clindamycin. Indian J Dermatol Venereol Leprol 2015; 81:87.
- Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol 1995; 131:1112.
- Feldmann R, Masouyé I, Chavaz P, Saurat JH. Fox-Fordyce disease: successful treatment with topical clindamycin in alcoholic propylene glycol solution. Dermatology 1992; 184:310.
- Pock L, Svrcková M, Machácková R, Hercogová J. Pimecrolimus is effective in Fox-Fordyce disease. Int J Dermatol 2006; 45:1134.
- Milcic D, Nikolic M. Clinical effects of topical pimecrolimus in a patient with Fox-Fordyce disease. Australas J Dermatol 2012; 53:e34.
- Kaya Erdoğan H, Bulur I, Kaya Z. Clinical Effects of Topical Tacrolimus on Fox-Fordyce Disease. Case Rep Dermatol Med 2015; 2015:205418.
- Tkach JR. Tretinoin treatment for Fox-Fordyce disease. Arch Dermatol 1979; 115:1285.
- Giacobetti R, Caro WA, Roenigk HH Jr. Fox-Fordyce disease. Control with tretinoin cream. Arch Dermatol 1979; 115:1365.
- Kassuga LE, Medrado MM, Chevrand NS, et al. Fox-Fordyce disease: response to adapalene 0.1%. An Bras Dermatol 2012; 87:329.
- KRONTHAL HL, POMERANZ JR, SITOMER G. FOX-FORDYCE DISEASE: TREATMENT WITH AN ORAL CONTRACEPTIVE. Arch Dermatol 1965; 91:243.
- González-Ramos J, Alonso-Pacheco ML, Goiburú-Chenú B, et al. Successful treatment of refractory pruritic Fox-Fordyce disease with botulinum toxin type A. Br J Dermatol 2016; 174:458.
- Effendy I, Ossowski B, Happle R. Fox-Fordyce disease in a male patient--response to oral retinoid treatment. Clin Exp Dermatol 1994; 19:67.
- Chae KM, Marschall MA, Marschall SF. Axillary Fox-Fordyce disease treated with liposuction-assisted curettage. Arch Dermatol 2002; 138:452.
- Storino WD, Engel GH. Office surgical management of recalcitrant axillary lesions. Cutis 1978; 21:338.
- Ahmed Al-Qarqaz F, Al-Shannag R. Fox-Fordyce disease treatment with fractional CO2 laser. Int J Dermatol 2013; 52:1571.
- 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