Supplementary Material for: DIAGNOSIS, MANAGEMENT, AND OUTCOME OF BART’S SYNDROME OBSERVED IN A SUB-SAHARAN AFRICAN COUNTRY (SENEGAL, DAKAR): 2 CASES REPORTS

Bart's syndrome is an uncommon inherited congenital disorder associated with congenital cutaneous aplasia of the extremities, epidermolysis bullosa, and nail deformity. Bilateral and symmetrical involvement of the limbs is exceptionally described on black skin. In most cases the diagnosis is clinical, however, the management remains very difficult and the extended forms are a real therapeutic challenge. We report 02 cases of Bart's syndrome observed in a Sub-Saharan African country (Senegal, Dakar). It was about 2 premature female and male newborns. On physical examination: the girl presented with a total absence of skin on the limbs, associated with cutaneous detachment of the trunk representing a detached and detachable skin surface of 46%; the boy underwent a total absence of skin of more than 50% of the skin surface. The diagnosis of Bart's syndrome was set based on the typical clinical aspect. The blood count and CRP were normal for the girl whereas it revealed some disorders for the boy. The 2 newborns were urgently admitted to an incubator, and the intensive care was started with hyperhydration, anti-staphylococcal prophylaxis, and daily dermatological care with antiseptic baths and fatty dressings. Bart's syndrome is an uncommon genodermatosis characterized by a clinical triad associating: congenital cutaneous aplasia of the extremities, inherited epidermolysis bullosa suspected in the presence of bubbles, and areas of cutaneous fragility and nail deformity. All types of which can be associated with this syndrome. The easy clinical diagnosis but the difficult management encumber the vital prognosis of our cases.Bart综合征（Bart's syndrome）是一种罕见的遗传性先天性疾病，与四肢先天性皮肤缺损、大疱性表皮松解症（epidermolysis bullosa）以及指甲畸形相关。在黑皮肤人群中，双侧对称的肢体受累病例极为罕见报道。多数情况下，该病依靠临床诊断，但治疗难度极大，重型病例更是临床治疗的重大挑战。本文报告了撒哈拉以南非洲国家塞内加尔达喀尔地区收治的2例Bart综合征病例。患者为1名早产女婴与1名早产男婴。体格检查显示：女婴四肢皮肤完全缺失，伴躯干皮肤剥脱，剥脱面积占体表总面积的46%；男婴体表超过50%的皮肤完全缺失。结合典型临床表现，确诊为Bart综合征。女婴的血常规及C反应蛋白（CRP）水平均正常，而男婴则存在相关指标异常。两名新生儿均被紧急收入保温箱，并接受了强化治疗：包括水化补液（hyperhydration）、抗葡萄球菌感染预防，以及每日采用抗菌药浴与油性敷料进行皮肤护理。Bart综合征是一种罕见的遗传性皮肤病（genodermatosis），其临床特征为三联征：四肢先天性皮肤缺损、出现水疱时疑似遗传性大疱性表皮松解症（epidermolysis bullosa），以及皮肤脆性区域与指甲畸形。所有上述表型均可伴随该综合征出现。尽管该病临床诊断相对简便，但治疗难度颇高，这对本次报告的两例患者的生存预后造成了不利影响。