Cherubino M, Maggiulli F, Dibartolo R, Valdatta L
Plastic and Reconstructive Unit, Ospedale di Circolo e Fondazione Macchi, Varese, Italy.
University of Insubria, Varese Italy.
J Wound Care. 2016 Dec 2;25(12):760-762. doi: 10.12968/jowc.2016.25.12.760.
Aplasia cutis congenita (ACC) is a rare congenital disease, characterised by absence of skin, that can affect different parts of the body. ACC is more frequent on the scalp, but can involve limbs, with partial-or total-thickness loss of skin, with a functional impairment of the joint affected. There is no clear indication for surgical or conservative treatment, therefore, it would be helpful to find scientific support for the optimal treatment strategies.
We present a case of a newborn female, with six wounds on the inferior limbs, treated with conservative therapy. To prevent infection and promote healing, the defects were kept moist and covered with non-adherent/antimicrobical dressings. Complete wound healing occurred in eight weeks. The duration of follow-up was three years.
In this kind of ACC with superficial partial-thickness wounds of the lower limbs, conservative treatment was successful. The same conservative treatment can be proposed for larger or deeper wounds of lower limbs with no involvement of underlying structures, with delayed scar excision after the complete growth of the patient. ACC scar excision could require complex tissue rearrangement, tissue expansion, or skin grafting.
In the evaluation for treatment of ACC of inferior limbs, even in cases of total-thickness skin loss, even on joints, the conservative approach could be considered as the first choice.
None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.
先天性皮肤发育不全（ACC）是一种罕见的先天性疾病，其特征为皮肤缺失，可累及身体不同部位。ACC在头皮更为常见，但也可累及四肢，导致皮肤部分或全层缺失，并伴有受累关节功能障碍。目前对于手术或保守治疗尚无明确指征，因此，为最佳治疗策略寻找科学依据将有所帮助。
我们报告一例新生儿女性病例，其下肢有6处伤口，接受了保守治疗。为预防感染并促进愈合，伤口保持湿润，覆盖非粘性/抗菌敷料。8周后伤口完全愈合。随访时间为3年。
对于这种下肢浅表部分厚度伤口的ACC，保守治疗取得成功。对于下肢较大或较深且未累及深层结构的伤口，同样可采用保守治疗，并在患者完全生长后延迟进行瘢痕切除。ACC瘢痕切除可能需要复杂的组织重排、组织扩张或皮肤移植。
在评估下肢ACC的治疗时，即使是全层皮肤缺失的情况，甚至累及关节，保守方法也可被视为首选。
本文作者均未在本手稿提及的任何产品、设备或药物中拥有经济利益。
