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患婴,男,38天。因皮疹1个月,反复抽搐、发绀4～5小时,于1981年8月11日入院。患儿系第3胎足月顺产。生后第3天周身皮肤出现散在红色小丘疹,渐变水疱,化脓。发病第4天住入我院治疗,出院后皮疹反复出现,经常咳嗽,第二次入院前4～5小时,突然反复抽搐、发绀。母妊娠期体健,无服药史,第2胎男孩,生后4个月反复抽搐医治无效而死亡。体检:T36.5℃,鼻翼搧动,口唇发绀。右面颊部、背部皮肤可见数个表浅小溃疡。全身浅表淋巴结未触及。头颅对称,前囟2×2厘米,平坦。五官端正。气管稍左移,右胸较膨隆。右肺呼吸音明显减弱,未闻及罗音。心率160次/分,律齐,未闻及杂音。腹平软,肝肋下3厘米,剑突下3厘米,脾肋下1厘米。脊柱无畸形,右上臂稍肿胀,右上肢活动受限。神经系统未发现异常。实验室检查:血红蛋白8克％,白细胞4000,中性粒细胞81％,淋巴细胞19％。血钙9.4毫克％,无机磷3.4毫克％。血清蛋白电泳A66.09％,α_14.02％,α_210.92％,β8.62％,γ10.35％。硷性磷酸酶26单位。康氏反应阴性。骨髓检查:髓象左移。X线摄片:肋骨、锁骨及肱骨均见不规则斑点状透明区,右肱骨病理性骨折。腰椎、骨盆及股骨均见斑点状不规则溶骨性破坏,股骨轻度骨膜反应。颅骨侧位未见异常。右侧气胸,右肺压缩10％;两肺见片状边缘不规则緻密阴影。入院后经右胸尼龙管闭塞引流,抗菌素应用及其他对症处理无效,于住院第六天死亡。死后病理诊断:组织细胞增生症急性型(勒-雪氏病)。病变侵犯皮肤、双肺、双肾、肝、脾、胰、胸腔及腹腔淋巴结、肋骨髂骨和肱骨。勒-雪氏病多见于2岁以下6个月以上婴幼儿,国内潘素英等(1957)首先报告3例,以后有少数病例报道。本例发生于新生儿具有较典型的临床表现和X线象,并经病理检查证实,为罕见病例。该病目前无有效疗法,预后不良。
Infant, male, 38 days. 1 month due to rash, repeated convulsions, cyanosis 4 to 5 hours, admitted on August 11, 1981. Children with third-term full-term fetus. 3 days after birth, whole body skin scattered scattered red papules, blisters, purulent. The first 4 days admitted to our hospital for treatment, recurrent rash after discharge, often cough, 4 to 5 hours before the second admission, sudden convulsions, cyanosis. Pregnancy, physical health, no medication history, the first 2-boy, 4 months after birth, repeated convulsive treatment and died. Physical examination: T36.5 ℃, nose flap, lips cyanosis. The right cheek, the back of the skin can be seen a few superficial ulcers. Whole body superficial lymph nodes not touched. Head symmetrical, anterior fontanel 2 × 2 cm, flat. Good facial features. Trachea slightly left, right chest more bulging. Right lung breath sounds significantly weakened, did not smell and rales. Heart rate 160 beats / min, law Qi, no smell and noise. Abdomen soft, liver ribs 3 cm, 3 cm under the xiphoid, 1 cm under the spleen ribs. Spine without deformity, right upper arm slightly swollen, limited right upper limb movement. Nervous system found no abnormalities. Laboratory tests: 8 grams of hemoglobin, white blood cells 4000, 81% of neutrophils, lymphocytes 19%. 9.4 mg of calcium, 3.4 mg of inorganic phosphorus. Serum protein electrophoresis A66.09%, α_14.02%, α_210.92%, β8.62%, γ10.35%. Alkaline phosphatase 26 units. Kang’s reaction negative. Bone marrow examination: medullary left. X-ray: rib, clavicle and humerus are irregular spot-like transparent area, the right humeral pathological fracture. Lumbar vertebrae, pelvis and femur are spotted irregular osteolytic destruction, femoral mild periosteal reaction. Skull lateral no abnormalities. Right pneumothorax, right lung compression 10%; two lungs see flaky irregular irregular shadow. After admission through the right chest nylon tube occlusion drainage, antibiotics and other symptomatic treatment ineffective, died on the sixth day of hospitalization. Postmortem pathology diagnosis: Acute type of histiocytosis (Leuchet’s disease). Lesions invading the skin, lungs, kidneys, liver, spleen, pancreas, chest and abdominal lymph nodes, rib iliac and humerus. Leh-Schue disease more common in infants under 6 years of age more than 6 months, the domestic Pan Suying (1957) first reported 3 cases, a few cases later reported. This case occurred in neonates with more typical clinical manifestations and X-ray images, and confirmed by pathology, is a rare case. The disease is currently no effective therapy, the prognosis is poor.