Dereksson Kristjan, Kjartansson Sveinn, Hjartardóttir Hulda, Arngrimsson Reynir
Pediatrics Department, Skåne University Hospital, Malmö, Sweden.
BMJ Case Rep. 2012 Aug 27;2012:bcr0220125823. doi: 10.1136/bcr.02.2012.5823.
Ichthyosis prematurity syndrome (IPS) is a rare inherited skin disorder. Children are born prematurely with thick skin and have been found to develop neonatal asphyxia due to occlusions in the bronchial tree from debris in the amniotic fluid. At 31 weeks of gestation, separation of amniotic and chorionic membranes was identified as well as polyhydramnion. The child was born 2 weeks later, with thickened skin with a granular appearance and required immediate ventilation and intensive care. At 2 years of age, the patient has developed an atopic skin condition with severe itching, recurrent skin infections, food intolerance and periods of wheezing. Prenatal observation of separation of foetal membranes or dense amniotic fluid may be signs of IPS and severe complication immediately after birth.
鱼鳞病早产综合征（IPS）是一种罕见的遗传性皮肤病。患儿早产，皮肤增厚，且已发现因羊水碎屑阻塞支气管树而发生新生儿窒息。妊娠31周时，发现羊膜和绒毛膜分离以及羊水过多。患儿在2周后出生，皮肤增厚，呈颗粒状外观，需要立即通气和重症监护。患儿2岁时，出现了特应性皮肤病，伴有严重瘙痒、反复皮肤感染、食物不耐受和喘息期。产前观察到胎膜分离或羊水浓稠可能是IPS的迹象，且出生后会立即出现严重并发症。
