Lim S Y, Ho N K, Tan K C, Giam Y C
Neonatal Medicine I, Kandang Kerbau Hospital, Singapore.
J Singapore Paediatr Soc. 1990;32(3-4):164-8.
Aplasia cutis congenita (ACC) including epidermolysis bullosa (EB) are rare congenital conditions. In ACC there is a localised absence of skin while in EB blistering occurs with a split at the epidermal or dermal level. In the past these 2 conditions have been reported to occur in the same patient. A case of a Gurkha baby girl illustrating just such an entity with severe erosive defects over the head, face, trunk and acral parts of the limbs presenting at birth is reported. There was also widespread blistering over the chest, abdomen and back. The baby died after 3 days from serious complications.
先天性皮肤发育不全(ACC)包括大疱性表皮松解症(EB)是罕见的先天性疾病。在先天性皮肤发育不全中,局部皮肤缺失,而在大疱性表皮松解症中,表皮或真皮层会出现分裂并形成水疱。过去曾有报道称这两种病症会出现在同一患者身上。本文报告了一例廓尔喀女婴的病例,该女婴出生时头部、面部、躯干和四肢末端出现严重糜烂性缺损,胸部、腹部和背部也有广泛水疱。该婴儿在3天后因严重并发症死亡。