Discuz!NT|BBS|论坛

注册

 

发新话题 回复该主题

病例漫谈4:新生儿面部环状病变 [复制链接]

1#
    McCauliffe DP在《美国皮肤病学会杂志》上发表了一篇“新生儿面部环状病变”的病例,病例分析及译文如下:
History
A 1-month-old baby girl was referred with a 5-day history of a facial rash. The child was otherwise well, breast-feeding successfully and gaining weight appropriately. The pregnancy had been uncomplicated. The child’s parents were both well with no significant past medical history.
病史
    一名1月大女婴面部患皮疹已有5天。该女婴其它体征良好,正常哺育,体重正常增长。母体妊娠过程中无异状,双亲身体健康,没有显著的既往病史。

Clinical features
The baby appeared well. There were several raised, annular hyperpigmented lesions on the face (Figures 2a, b). Similar lesions were also observed in the scalp. Discrete erythematous macules were present on the palms and soles. Atrophic hyperpigmented lesions were seen on the back. The heart rate was 120 beats per minute and regular.
临床特征
    该女婴面部有几处凸起环形色素沉着性病变(图2a ,b),头皮上也有类似病变。手脚掌发现有离散红斑,背部有萎缩性色素沉着性病变。心率规律,每分钟120次。


Figure 2a
Neonatal lupus erythematosus.
There are multiple annular,hyperpigmented lesions scattered over the face and scalp
图2a
新生儿红斑狼疮
面部及头皮可见多处离散环状色素沉着性病变。




Figure 2b
Neonatal lupus erythematosus.
Close-up of an annular lesion on the forehead demonstrates central hyperpigmentation and atrophy.
图2b
新生儿红斑狼疮
前额环状病变特写图,提示中央色素沉着及萎缩。



Investigations
Hb: 10.2 g/dl (11.5–15.5 g/dl), WCC: 6.9×109/l (4.0–11.0×109/l), plts: 106×109/l(150–450×109/l).
ECG: normal sinus rhythm.
ANA: negative.
ENA: Ro positive; La negative.
Skin histopathology: Biopsy of an annular lesion demonstrated mild hyperkeratosis,an interface dermatitis with basal vacuolation and lymphocyte exocytosis (Figure 2c).
Skin direct immunofluorescence: negative.
Mother’s ANA: 1 in 10.
Mother’s ENA: Ro positive.
检查
    血红蛋白:10.2 g/dl (11.5–15.5 g/dl),白细胞:6.9×109/l (4.0–11.0×109/l),血小板:106×109/l(150–450×109/l).
    心电图:窦性心律正常。
    抗核抗体:阴性。
    可提取性核抗原:Ro阳性,La阴性。
    皮肤组织病理学检查:环状病变活检显示,轻度角化、基底细胞空泡变及淋巴细胞胞吐相关接触性皮炎(图2c)。
    皮肤直接免疫荧光检验法:阴性。
    母体抗核抗体:1:10。
    母体可提取性核抗原:Ro阳性


Figure 2c
Neonatal lupus erythematosus.
Skin histopathology (H&E, medium power). There is an interface dermatitis with basal vacuolation and lymphocytic exocytosis. There is overlying hyperkeratosis.
图2c
新生儿红斑狼疮
皮肤组织病理学检查(H&E染色,中倍镜下观察)。
可见基底细胞空泡变及淋巴细胞胞吐相关接触性皮炎,伴有覆角化现象。



Diagnosis
Neonatal lupus erythematosus.
诊断
    新生儿红斑狼疮

Treatment and progress
The child’s cutaneous lesions cleared over the next 3 weeks using a mild topical corticosteroid. There was some post-inflammatory hyperpigmentation and minimal residual atrophic scarring. Six months after presentation a repeated ENA was negative, showing loss of the circulating Ro antibody. The mother was advised that her subsequent pregnancies should be closely monitored. Despite this recommendation, 6 years later she represented with a second baby girl born 2 months earlier following an unsupervised pregnancy. The new baby had, like her older sister, a number of inflammatory lesions on the skin of her face. Investigations revealed a positive Ro antibody. She was otherwise well, with no cardiac problems. The cutaneous lesions settled over the next few weeks with the use of a mild topical corticosteroid.
治疗和进展
    使用温和的外用皮质类固醇治疗3周后,该女婴皮肤病变清除。残留几处炎症后色素沉着和细微萎缩性疤痕。6个月后,ENA复查显阴性,提示循环Ro抗体缺失。
    建议该女婴母亲在下次妊娠期接受密切的监测。然而,6年后她第二次妊娠没有接受监测,结果女婴早产2个月。该女婴与姐姐一样,面部皮肤有炎性病变。经过检查,其Ro抗体显阳性,其它体征良好,无心脏病。使用温和的外用皮质类固醇治疗几周后,该女婴皮肤病变得到清除。

Comment
Neonatal lupus erythematosus (NLE) is a lupus syndrome caused by autoantibodies that are passively acquired by the fetus from the maternal circulation. The majority of infants with NLE exhibit cutaneous and/or cardiac disease, although other manifestations have been described. Females seem to be affected more frequently than males, particularly by NLE skin disease. These skin lesions may be present at birth but usually develop days to weeks and sometimes months after delivery. Cutaneous NLE may be precipitated or exacerbated by UV light exposure and there are reported cases of cutaneous NLE being precipitated by phototherapy for hyper bilirubinaemia.
NLE skin lesions are both clinically and histopathologically similar to those of subacute cutaneous SLE, which is also characterized by a positive ENA, usually anti-Ro. Lesions, which are commonly found on the face, begin as erythematous macules, which enlarge into annular patches and plaques often with fine overlying scale. Spontaneous resolution within weeks is usual, with transient dyspigmentation, telangiectasis and epidermal atrophy. Histologically the lesions of NLE are characterized by vacuolar degeneration of the basal keratinocytes and a lymphocytic infiltrate in the upper dermis.
Although our patient had no demonstrable cardiac problems, complete heart block (CHB) occurs in approximately 50% of cases of NLE. A slow fetal heart rate noticed late in pregnancy provides the first clue of CHB. Fetal echocardiography confirms heart block by demonstrating slow ventricular contraction occurring independently of the atria.
Mortality rates in infants with CHB may be as high as 20%. Other manifestations of NLE include anaemia and transient thrombocytopenia, as in our patient. Hepatomegaly may occur, which is secondary either to extramedullary haematopoiesis or to congestive heart Failure.
评论
    新生儿红斑狼疮(NLE)是胎儿通过血液循环从母体中被动获得的一种自身抗体引起的狼疮综合征。红斑狼疮新生儿大多伴有皮肤病和/或心脏病,也会伴有其它症状。女婴往往比男婴更容易患病,新生儿红斑狼疮皮肤病居多。这些皮肤病变可能出现在婴儿出生时,但往往在分娩几天或几周有时几月后发生恶化。新生儿红斑狼疮皮肤病变在紫外线照射下可能发生色素沉着或者病情加重,有病例报道经彩超胆红素血症治疗后,新生儿红斑狼疮皮肤病变发生色素沉着。
    从临床特征和病理特征看,新生儿红斑狼疮皮肤病变与亚急性皮肤型红斑狼疮相似。亚急性皮肤型红斑狼疮ENA通常显阳性并抗Ro。病变通常发生在面部,开始是红斑,后来变大形成密集的环状斑块。几周内常见自行缓解,伴随瞬态色素沉着、毛细血管扩张、表皮萎缩。新生儿红斑狼疮的组织学病变特点为基底角质细胞的空泡变性,以及真皮上部的淋巴细胞浸润。
    虽然患者没有明显的心脏病,但是约有50%的新生儿红斑狼疮病例中出现完整性心脏传导阻滞(CHB)。妊娠晚期胎儿心率缓慢提示出现完整性心脏传导阻滞。通过胎儿超声心动图检测,显示出独立心房中出现缓慢心室收缩,证实发生心脏传导阻滞。
    完整性心脏传导阻滞婴儿的死亡率可高达20%。 其新生儿红斑狼疮特征还有贫血、瞬态血小板减少,与我们收治的患者表现一致。也可伴随肝肿大,对髓外造血或充血性心脏衰竭影响较小。

Learning points
1. The association of annular skin lesions and complete heart block in an infant is strongly suggestive of neonatal lupus erythematosus (NLE).
2. NLE is caused by Ro autoantibodies (occasionally La) transferred from the maternal circulation to the fetus.
3. The mother should be investigated for lupus erythematosus and be warned that NLE may develop in future pregnancies.
学习要点
    1、婴儿同时出现环状皮损和完整性心脏传导阻滞,提示新生儿红斑狼疮。
    2、新生儿红斑狼疮是由胎儿通过血液循环从母体中被动获得的Ro自身抗体(有时候是La)引起的。
    3、孕妇应接受红斑狼疮检查,并获知妊娠过程可能会引起新生儿红斑狼疮的发生。

Ro autoantibodies are present in approximately 80% of NLE patients and in 90% of their mothers. La autoantibodies are observed less frequently. Maternally derived autoantibodies appear to play a direct role in the pathogenesis of the NLE skin disease, an association supported by the simultaneous clearance of the dermatosis and maternally acquired antibodies at about 6 months of age.
    将近80%红斑狼疮新生儿及90%红斑狼疮新生儿母亲体内存在Ro自身抗体,La抗体则较少检测到。母体产生的自身抗体通常会直接影响新生儿红斑狼疮皮肤病的病理机制,妊娠6个月后,可清除皮肤病变,同时自身抗体也逐渐耗去。

Reference
McCauliffe DP. Neonatal lupus erythematosus: a transplacentally acquired autoimmune disorder.
Semin Dermatol 1995; 14:47–53.
参考文献
McCauliffe DP. Neonatal lupus erythematosus: a transplacentally acquired autoimmune disorder.
Semin Dermatol 1995; 14:47–53.

                                                                                                                          (编译:王凤霞 校对:王影影)
本主题由 管理员 admin 于 2013/6/21 16:21:56 执行 设置精华/取消 操作
分享 转发
TOP
2#

妊娠6个月后,可清除皮肤病变,同时自身抗体也逐渐耗去。,最后一句话说错了,应该是:新生儿6个月后皮肤病变可清除,同时自身抗体也逐渐耗去。
TOP
发新话题 回复该主题