过 敏 性 紫 癜
Anaphylactoid Purpura
许兰 -亨诺血管炎 (综合征 )
Schonlein-Henoch vasculitis(syndrome)
儿科医院 邬惊雷
概 貌 Outline
是一种小血管炎
A kind of vasculitis of small vessels
以皮肤紫癜、关节痛、积液、腹痛、便血、血
尿和蛋白尿为主要临床特征
Characterized clinically by purpura,arthralgia,articular
effusion,abdominal pain,hemafecia,hematuria
and proteinuria
学龄前和学龄期儿童多见
More frequent in preschoolers and school age children
男孩发病率高于女孩
Boys are affected more frequently than girls
估计人群总体发病率约为 9/10万
The overall incidence estimated to be 9/100,000
population
病 因 Etiology
病因不清楚，可能与下列因素有关,
The etiology is unknown,maybe related with the follows:
食物过敏：海鲜、蛋、牛奶
Food allergy,sea food,eggs,milk
感染：细菌、病毒、寄生虫
Infection,bacteria,virus,parasites
病 因 Etiology
药物：抗生素、阿司匹林
Drugs,antibiotics,aspirin
其它：虫咬、预防接种
Others,insect bite,immunization
病理改变 Histopathological findings
白细胞碎裂性血管炎
Leukocytoclastic vasculitis
IgA为主的免疫复合物沉积
Deposition of immune complexes of
IgA(in the small vessels and glomeruli)
病理改变 Histopathological findings
肾脏病变：微小、局灶、新月体形成等
箭头所指处为新月体
临床表现 Clinical manifestations
常随上呼吸道感染后
Usaually follows an upper respiratory tract infection
可有低热、纳差、乏力
Low-grade fever,losing appetite and fatigue may occur
皮肤紫癜 Purpura
高出皮面，初期压之退色
Palpable purpura that initially blanch on pressure
反复出现，呈紫红色
Appear at intervals and evolve from red to purple
对称分布于腰以下
Occur symmetrically below the waist
尚可伴有血管神经性水肿
May have angioedema
1 岁半女孩患过敏性紫癜：
注意：局限于半下身，明显的、边缘清楚分布的皮疹！
5 岁男孩患过敏性紫癜：典型的臀部皮疹
5 岁男孩患过敏性紫癜,
典型的皮疹和双踝关节肿胀
4 岁女孩患过敏性紫癜：
下肢伸侧显著、部分融合的皮疹
8 岁半男孩患过敏性紫癜：
皮肤出血几乎仅限于双足背和
大足趾；关节轻度肿胀
消化道症状 Symptoms of gastrointestinal tract
间歇性腹痛，位于脐周或下腹部
Intermittent abdominal pain localized to periomphalic
area and lower abdomen
呕吐、便血
Vomiting,hemafecia
可有肠套叠、肠梗阻和肠穿孔
Intussusception,obstruction and bower perforation may
occur
关节症状
主要累及膝、踝关节
Mainly affects the knees and ankles
伴有肿胀和积液
Concomitant with edema and effusion
能在一定时间内消退，不留畸形
May resolve after a few days without residual
deformity
4 岁男孩患紫癜性风
湿病：
所见：
双关节明显肿胀
（左 > 右 )
肾脏症状 Renal involvement
约 25～ 50％病例累及肾脏
Renal involvement occurs in 25-50% cases
多数在起病一个月内出现
Most will develop renal symptoms within 1 month
表现为血尿、尿蛋白和高血压
Presented with hematuria,proteinuria and hypertension
少数呈肾病综合征表现
Few may appear as nephrotic syndrome
其他表现 Other clinical findings
颅内出血：惊厥、瘫痪、失语、昏迷等
Intracranial hemorrhage,convulsion,paralysis(paresis)、
aphasia,coma et al.
可有胰腺炎、睾丸出血、肺出血等。
May have pancreatitis,testicular or pulmonary hemorrhage
实验室检查 Laboratory findings
无敏感和特异诊断指标
Routine tests are neither specific nor diagnostic
可有血小板、白细胞升高,血沉增快
May have thrombocytosis and leukocytosis,The
erythrocyte sedimentation rate may be elevated.
约 50％病例 IgA升高
About 50% of patients have elevated concentration of IgA
重症者可出现高凝状态
Hypercoagulable state may occur in severe cases
鉴别诊断 Differential diagnosis
突发性血小板减少性紫癜
Idiopathic thrombocytopenic purpura
外科急腹症 （急性阑尾炎、肠套叠）
Surgical abdomen(acute appendicitis,intussusception
细菌感染（败血症，感染性心内膜炎,流脑）
Bacterial infection(septicemia,IBE,meningococcal meningitis
风湿性关节炎 Rheumatic arthritis
治疗：对症治疗 Symptomatic treatment
去除诱因：感染、食品、药物
Excluding induction factors（ infections,foods,drugs)
对症治疗：
Symptomatic treatment
休息、饮食、抗组胺药物、钙剂、维生素 C、
非甾体类抗炎药
Rest,bland diet,antihistamine drugs,calcium,vitamine C
and NSAID
肾上腺皮质激素：能明显改善消化道症状和关节
痛，但不能阻止肾脏病变
Costicosteroids,Often associated with dramatic improvement
of both gastrointestinal symptoms and arthralgia.But,the
renal involvement can not be stoped
其它免疫抑制剂：严重病例可选用
Other immunosuppressive drugs,used in severe cases
肾脏病变的治疗 Management of renal involvement
与急性肾小球肾炎相同
The same as for acute glomerulonnephritis
抗凝治疗：用以肾脏病变为主要表现者
Anticosgulant therapy,used for the cases mainly with
renal damage
肝素 Heparin
阿司匹林 Aspirin
双嘧达莫（潘生丁） Dipyridamole
预后 Prognosis
多呈自限性，预后良好
Most cases have a self-limited course with an good overall
prognosis
部分有复发
Some may recur
<1％病例发展成持续肾脏病变
<1% patients develop persistent renal disease
Anaphylactoid Purpura
许兰 -亨诺血管炎 (综合征 )
Schonlein-Henoch vasculitis(syndrome)
儿科医院 邬惊雷
概 貌 Outline
是一种小血管炎
A kind of vasculitis of small vessels
以皮肤紫癜、关节痛、积液、腹痛、便血、血
尿和蛋白尿为主要临床特征
Characterized clinically by purpura,arthralgia,articular
effusion,abdominal pain,hemafecia,hematuria
and proteinuria
学龄前和学龄期儿童多见
More frequent in preschoolers and school age children
男孩发病率高于女孩
Boys are affected more frequently than girls
估计人群总体发病率约为 9/10万
The overall incidence estimated to be 9/100,000
population
病 因 Etiology
病因不清楚，可能与下列因素有关,
The etiology is unknown,maybe related with the follows:
食物过敏：海鲜、蛋、牛奶
Food allergy,sea food,eggs,milk
感染：细菌、病毒、寄生虫
Infection,bacteria,virus,parasites
病 因 Etiology
药物：抗生素、阿司匹林
Drugs,antibiotics,aspirin
其它：虫咬、预防接种
Others,insect bite,immunization
病理改变 Histopathological findings
白细胞碎裂性血管炎
Leukocytoclastic vasculitis
IgA为主的免疫复合物沉积
Deposition of immune complexes of
IgA(in the small vessels and glomeruli)
病理改变 Histopathological findings
肾脏病变：微小、局灶、新月体形成等
箭头所指处为新月体
临床表现 Clinical manifestations
常随上呼吸道感染后
Usaually follows an upper respiratory tract infection
可有低热、纳差、乏力
Low-grade fever,losing appetite and fatigue may occur
皮肤紫癜 Purpura
高出皮面，初期压之退色
Palpable purpura that initially blanch on pressure
反复出现，呈紫红色
Appear at intervals and evolve from red to purple
对称分布于腰以下
Occur symmetrically below the waist
尚可伴有血管神经性水肿
May have angioedema
1 岁半女孩患过敏性紫癜：
注意：局限于半下身，明显的、边缘清楚分布的皮疹！
5 岁男孩患过敏性紫癜：典型的臀部皮疹
5 岁男孩患过敏性紫癜,
典型的皮疹和双踝关节肿胀
4 岁女孩患过敏性紫癜：
下肢伸侧显著、部分融合的皮疹
8 岁半男孩患过敏性紫癜：
皮肤出血几乎仅限于双足背和
大足趾；关节轻度肿胀
消化道症状 Symptoms of gastrointestinal tract
间歇性腹痛，位于脐周或下腹部
Intermittent abdominal pain localized to periomphalic
area and lower abdomen
呕吐、便血
Vomiting,hemafecia
可有肠套叠、肠梗阻和肠穿孔
Intussusception,obstruction and bower perforation may
occur
关节症状
主要累及膝、踝关节
Mainly affects the knees and ankles
伴有肿胀和积液
Concomitant with edema and effusion
能在一定时间内消退，不留畸形
May resolve after a few days without residual
deformity
4 岁男孩患紫癜性风
湿病：
所见：
双关节明显肿胀
（左 > 右 )
肾脏症状 Renal involvement
约 25～ 50％病例累及肾脏
Renal involvement occurs in 25-50% cases
多数在起病一个月内出现
Most will develop renal symptoms within 1 month
表现为血尿、尿蛋白和高血压
Presented with hematuria,proteinuria and hypertension
少数呈肾病综合征表现
Few may appear as nephrotic syndrome
其他表现 Other clinical findings
颅内出血：惊厥、瘫痪、失语、昏迷等
Intracranial hemorrhage,convulsion,paralysis(paresis)、
aphasia,coma et al.
可有胰腺炎、睾丸出血、肺出血等。
May have pancreatitis,testicular or pulmonary hemorrhage
实验室检查 Laboratory findings
无敏感和特异诊断指标
Routine tests are neither specific nor diagnostic
可有血小板、白细胞升高,血沉增快
May have thrombocytosis and leukocytosis,The
erythrocyte sedimentation rate may be elevated.
约 50％病例 IgA升高
About 50% of patients have elevated concentration of IgA
重症者可出现高凝状态
Hypercoagulable state may occur in severe cases
鉴别诊断 Differential diagnosis
突发性血小板减少性紫癜
Idiopathic thrombocytopenic purpura
外科急腹症 （急性阑尾炎、肠套叠）
Surgical abdomen(acute appendicitis,intussusception
细菌感染（败血症，感染性心内膜炎,流脑）
Bacterial infection(septicemia,IBE,meningococcal meningitis
风湿性关节炎 Rheumatic arthritis
治疗：对症治疗 Symptomatic treatment
去除诱因：感染、食品、药物
Excluding induction factors（ infections,foods,drugs)
对症治疗：
Symptomatic treatment
休息、饮食、抗组胺药物、钙剂、维生素 C、
非甾体类抗炎药
Rest,bland diet,antihistamine drugs,calcium,vitamine C
and NSAID
肾上腺皮质激素：能明显改善消化道症状和关节
痛，但不能阻止肾脏病变
Costicosteroids,Often associated with dramatic improvement
of both gastrointestinal symptoms and arthralgia.But,the
renal involvement can not be stoped
其它免疫抑制剂：严重病例可选用
Other immunosuppressive drugs,used in severe cases
肾脏病变的治疗 Management of renal involvement
与急性肾小球肾炎相同
The same as for acute glomerulonnephritis
抗凝治疗：用以肾脏病变为主要表现者
Anticosgulant therapy,used for the cases mainly with
renal damage
肝素 Heparin
阿司匹林 Aspirin
双嘧达莫（潘生丁） Dipyridamole
预后 Prognosis
多呈自限性，预后良好
Most cases have a self-limited course with an good overall
prognosis
部分有复发
Some may recur
<1％病例发展成持续肾脏病变
<1% patients develop persistent renal disease
