Bonomi A, Gomirato G, Capalbo P, Crosato M
Divisione di Pediatria, Ospedale Martini, Torino, Italy.
Panminerva Med. 1990 Apr-Jun;32(2):96-9.
The authors report a case of "harlequin fetus" which was brought to their attention. The report was justified not only by the rarity of the disease, but above all because, after having examined the literature on the topic, this appears to be the third case treated with etretinate. The case in question was a female affected by congenital ichthyosis in its total form and by initial necrosis of the extremities. Etretinate treatment gave good results in terms of cutaneous modifications: reduction of hyperkeratosis, disappearance of ectropion and eclabium. Therefore, although the patient died when 16 days old due to sudden renal insufficiency, the case confirms the two previous reports on the value of this therapy.
作者报告了一例引起他们注意的“丑角样胎儿”病例。撰写该报告不仅是因为这种疾病罕见,更重要的是,在查阅了该主题的文献后发现,这似乎是第三例用阿维A酯治疗的病例。该病例为一名女性,患有完全型先天性鱼鳞病并伴有四肢初期坏死。就皮肤改善而言,阿维A酯治疗取得了良好效果:角化过度减轻,睑外翻和唇外翻消失。因此,尽管该患者在16日龄时因突发肾功能不全死亡,但该病例证实了之前两篇关于这种疗法价值的报告。