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]
Epidemiology
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.
Fox G, Fordyce J. Two cases of a rare papular disease affecting the axillary region. J Cutan Genito-Urinary Dis. 1902;20:1-5.
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].
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].
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].
Sandhu K, Gupta S, Kanwar AJ. Fox fordyce disease in a prepubertal girl. Pediatr Dermatol. Jan-Feb 2005;22(1):89-90. [Medline].
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].
Chae KM, Marschall MA, Marschall SF. Axillary Fox-Fordyce disease treated with liposuction-assisted curettage. Arch Dermatol. Apr 2002;138(4):452-4. [Medline].
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].
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].
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].
Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol. Oct 1995;131(10):1112-3. [Medline].
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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