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福克斯-福代斯病(顶泌汗腺粟粒疹)

Author
Jami L Miller, MD
Section Editor
Mark V Dahl, MD
Deputy Editor
Abena O Ofori, MD
Translators
张国强, 主任医师，副教授

引言

顶泌汗腺粟粒疹更常称为福克斯-福代斯病(Fox-Fordyce disease, FFD)，是一种罕见的瘙痒性皮肤病，人们推测其是由顶泌汗腺管或顶外分泌汗腺管阻塞和炎症引起的(图片 1A-E)。FFD最早由George Henry Fox和John Addison Fordyce于1902年描述为一种腋窝处瘙痒性丘疹[1]。此后，在身体其他含顶泌汗腺的部位也发现了FFD，包括乳晕、肛门生殖器皮肤及其他部位。

FFD的治疗较困难，但由于伴瘙痒，经常需进行治疗。初始治疗通常为局部治疗，例如外用糖皮质激素、外用克林霉素、外用钙调磷酸酶抑制剂；口服药物治疗和手术治疗主要仅用于难治性疾病。由于数据有限，很难得出有关干预相对疗效的结论。停止治疗后经常复发。

本专题将总结FFD的临床特征、诊断和治疗。小汗腺粟粒疹的相关内容见其他专题。 (参见“Miliaria”)

流行病学

多数FFD病例为年轻女性。女性与男性之比为9:1，最常在13-35岁间发病[2]。偶有报道称青春期前儿童和绝经后女性可发生FFD[3-5]。FFD发病无种族倾向。

发病机制

目前尚未完全理解FFD的发病机制，但可能与汗腺阻塞有关。人类存在3种汗腺：顶泌汗腺、顶外分泌汗腺和小汗腺[6]。顶泌汗腺是最大的汗腺，分布于腋窝、眼睑、乳晕、乳头、外耳道、脐周及肛门生殖器处的皮肤。顶泌汗腺的分泌导管开口于毛囊的漏斗管。顶外分泌汗腺是中等大小的汗腺，主要分布于腋窝，其分泌导管直接开口于皮肤表面。小汗腺是最小但分布最广的汗腺，与FFD无关。

               

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Literature review current through: 2017-06 . | This topic last updated: 2015-12-21.
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