五、治疗

五、治疗

本病迁延不愈。随年龄增长,症状可逐渐缓解。成年时仅表现为全身轻度发红,少量鳞屑。即所谓的鱼鳞病样红皮病。

本病无特效疗法。重症者多死亡。轻型出生时应清除鼻孔内角质碎片以免引起堵塞窒息死亡。使用甲状腺素及VitA疗效不著,局部搽油脂以滋润皮肤,3%水杨酸软膏虽有角质分离作用,但久搽时应注意避免吸收太多引起刺激。对于先天性骨骼畸形者可施行矫形术。

参考文献

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[2]Rodríguez-Pazos L,Ginarte M,Vega A,et al.Autosomal recessive congenital ichthyosis[J].Actas Dermo-Sifiliográficas(English Edition),2013,104(4):270-284.(https://www.daowen.com)

[3]Sethuraman G,Marwaha R K.Vitamin D,bone health and congenital ichthyosis[J].Indian Journal of Dermatology,Venereology and Leprology,2016,82(3):249-251.

[4]Glick J B,Craiglow B G,Choate K A,et al.Improved management of harlequin ichthyosis with advances in neonatal intensive care[J].Pediatrics,2017,139(1):e20161003.

[5]Sugiura K,Akiyama M.Update on autosomal recessive congenital ichthyosis:MRNA analysis using hair samples is a powerful tool for genetic diagnosis[J].Journal of Dermatological Science,2015,79(1):4-9.

(段宁 王文梅)