Fox-Fordyce Disease
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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]
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]
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.
No racial predilection is evident for Fox-Fordyce disease.
A distinct predilection for women exists for Fox-Fordyce disease; the female-to-male ratio is 9:1.
Fox-Fordyce disease is most common in women aged 13-35 years; it is rare before or after this age.
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.
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. 1956 Jan. 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. 1996 Jul-Aug. 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. 2003 Mar. 48(3):453-5. [Medline].
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 Jan 15. [Medline].
Alés-Fernández M, Ortega-Martínez de Victoria L, García-Fernández de Villalta MJ. Lesions in the axilla after hair removal using intense pulsed light. Fox-Fordyce disease. Actas Dermosifiliogr. 2015 Jan-Feb. 106 (1):61-2. [Medline].
Bernad I, Gil P, Lera JM, Giménez de Azcárate A, Irarrazaval I, Idoate MÁ. Fox Fordyce disease as a secondary effect of laser hair removal. J Cosmet Laser Ther. 2014 Jun. 16 (3):141-3. [Medline].
Sammour R, Nasser S, Debahy N, El Habr C. Fox-Fordyce Disease: An under-diagnosed adverse event of laser hair removal?. J Eur Acad Dermatol Venereol. 2016 Sep. 30 (9):1578-82. [Medline].
Stashower ME, Krivda SJ, Turiansky GW. Fox-Fordyce disease: diagnosis with transverse histologic sections. J Am Acad Dermatol. 2000 Jan. 42 (1 Pt 1):89-91. [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. 2008 Aug. 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. 2009 Jun. 31(4):393-7. [Medline].
Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol. 1995 Oct. 131(10):1112-3. [Medline].
George A, Bhatia A, Thomas E. Fox-Fordyce disease: a report of 2 cases responding to topical clindamycin. Indian J Dermatol Venereol Leprol. 2015 Jan-Feb. 81 (1):87-8. [Medline].
Chae KM, Marschall MA, Marschall SF. Axillary Fox-Fordyce disease treated with liposuction-assisted curettage. Arch Dermatol. 2002 Apr. 138 (4):452-4. [Medline].
Uzuncakmak TK, Karadag AS, Ozlu E, Akdeniz N, Cobanoglu Simsek B. Effective treatment of Fox-Fordyce disease with pulsed dye laser. Photodermatol Photoimmunol Photomed. 2016 Sep. 32 (5-6):311-313. [Medline].
Pock L, Svrckova M, Machackova R, Hercogova J. Pimecrolimus is effective in Fox-Fordyce disease. Int J Dermatol. 2006 Sep. 45(9):1134-5. [Medline].
Kaya Erdoğan H, Bulur I, Kaya Z. Clinical Effects of Topical Tacrolimus on Fox-Fordyce Disease. Case Rep Dermatol Med. 2015. 2015:205418. [Medline].
Christopher R Gorman, MD Avenues Dermatology, Private Practice
Christopher R Gorman, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.
Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society
Disclosure: Nothing to disclose.
William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine
William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.
Mary Farley, MD Dermatologic Surgeon/Mohs Surgeon, Anne Arundel Surgery Center
Disclosure: Nothing to disclose.
Fox-Fordyce Disease
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