Fox-Fordyce disease.
نویسندگان
چکیده
Fox-Fordyce disease (FFD) is a rare inflammatory disorder that affects the apocrine sweat glands. Clinically, lesions are equidistant, smooth, uniform, firm, folliculocentric papules, which can range in color from flesh-colored to red-brown to slightly yellow. Whereas the axillae are most commonly involved, FFD also can involve the anogenital and periareolar areas, lips, umbilicus, sternum, perineum, and upper medial aspects of the thighs. The underlying etiology of FFD remains unclear although epidemiologic data support a hormonal component because women are more commonly affected than men. Moreover, symptoms initially present after the onset of puberty, flare perimenstrually, and often resolve during pregnancy and after menopause. Histopathologic findings include the obstruction of the apocrine duct by a hyperkeratotic plug in the follicular infundibulum, which is believed to represent the primary pathophysiologic process; subsequent ductal rupture and resulting inflammatory response produce the typical clinical picture. Treatment of FFD is difficult because no one agent has proven particularly effective. Topical and interlesional glucocorticoids are often considered the first-line pharmacologic agents, although use is often limited by concerns for atrophy. Other agents that have shown some success include topical and systemic retinoids, topical clindamycin, topical pimecrolimus cream, benzoyl peroxide, and oral contraceptives. For medication-refractory cases, mechanical destruction or removal of the apocrine glands has been efficacious in small case series.
منابع مشابه
[Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease].
Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum...
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On examination, discrete 2to 3-mm perifollicular papules localized to the bilateral axillae ( fig. 1 ) and the pubic area were noted. The color of the papules ranged from flesh-colored to slightly hypopigmented. Additionally, her left axilla showed areas of superficial erosion, erythema and overlying honey-colored crust ( fig. 2 ). Of particular interest, the patient’s father was also examined ...
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5. Simón P, Sánchez-Yus E, Requena L. La glándula ecrina y la glándula apocrina de la piel humana normal. In: Requena L, editor. Neoplasias anexiales cutáneas. Madrid: Grupo Aula Médica; 2004. p. 3. 6. Zouboulis C, Tsatsou F. Trastorno de las glándulas sudoríparas apocrinas. In: Wolff, Goldsmith, Katz, Gilchrest, Paller, Leffell, editors. Fitzpatrick. Dermatología en Medicina General. 7.a ed. B...
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The Fox-Fordyce disease is a rare inflammatory dermatosis that affects mainly young women and is characterized by multiple follicular papules, skin color or brownish, very itchy, localized in areas rich in apocrine glands. Histopathology shows focal spongiosis of the upper infundibulum with fibrosis and perifollicular lymphohistiocytic infiltrate. The diagnosis is based on clinical and histopat...
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F ox-Fordyce disease (FFD) (also known as apocrine miliaria) is a rare dermatologic condition characterized by multiple skin-colored, equidistant, perifollicular papules distributed in areas rich in apocrine glands. These areas typically include the axillae, anogenital, and periareolar regions. This condition primarily affects young females between 15 and 35 years of age, and rarely presents be...
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ورودعنوان ژورنال:
- Dermatology online journal
دوره 18 12 شماره
صفحات -
تاریخ انتشار 2012