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]

    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:
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.

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.
    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

Figure 2b
Neonatal lupus erythematosus.
Close-up of an annular lesion on the forehead demonstrates central hyperpigmentation and atrophy.

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.
    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).

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.

Neonatal lupus erythematosus.

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.

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.
    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.

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.

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

发新话题 回复该主题