PREFACE

Henoch–Schönlein purpura is the most common systemic vasculitis of

childhood presenting with a tetrad of purpura, arthritis or arthralgia, abdominal pain
and renal disease. While the presence of purpura is a compulsory criterion for the
diagnosis of Henoch–Schönlein purpura, other signs and symptoms are more
variably present.
Abnormal glycosylation of immunoglobulin A1 molecules predisposes
patients with Henoch–Schönlein purpura to formation of large immune complexes.
Clearance of these large molecules is impaired, they deposit in small vessel walls
of the affected organs and trigger immune response leading to inflammatory
reaction presenting as clinical signs and symptoms.
The long-term morbidity of Henoch–Schönlein purpura is related to nephritis.
Based on the current evidence, early immunosuppressive treatment of children with
Henoch–Schönlein purpura should be reserved for those presenting with severe
kidney involvement (rapidly progressive glomerulonephritis, nephrotic syndrome).
There might be a role for immunosuppression in patients with ongoing nephritis
(persistent/increasing proteinuria), but this approach will have to be tested in large
prospective studies before it can be widely accepted in clinical practice.

1
 CHAPTER I
CASE ILLUSTRATION

A. IDENTITY
a. Patient
 Name : Child AMR
 Date of birth : 22nd May 2009
 Sex : Female
 Address : Batu Ampar, Kramat Jati, East Jakarta
 Tribe : Javanese
 Religion : Islam
 Education : Primary school
b. Parents
Father
 Name : Mr. MK
 Date of birth : 3rd April 1981
 Sex : Male
 Address : Batu Ampar, Kramat Jati, East Jakarta
 Tribe : Javanese
 Religion : Islam
 Education : S1
 Occupation : Employee
Mother
 Name : Mrs. C
 Date of birth : 14th March 1985
 Sex : Female
 Address : Batu Ampar, Kramat Jati, East Jakarta
 Tribe : Javanese
 Religion : Islam
 Education : D3
 Occupation : Housewife

2
B. HISTORY
History was taken from the patient (autoanamnesis) at Flamboyan
Bhayangkara Hospital R. Said Sukanto, East-Jakarta on 20 July 2018.
a. Chief Complaint
Abdominal pain since 1 week before admission to hospital.
b. Additional Complaint
Rashes on the legs to the buttocks and arms, nausea, and vomiting.
c. History of Present Illness
Since 1 week before patient entered the hospital, the patient complained
of abdominal pain, the pain is diffuse but frequently hurts in the upper
middle to around the navel, abdominal pain (colic) appears erratic, the
stomach hurts when pressed, abdominal pain increasing after meals. There
is no history of trauma to the stomach.
Since 1 week before patient entered the hospital, the patient also
complained of nausea and vomiting, vomiting only 1x/day, vomit contains
liquid and food, volume of vomiting as much as 1/4 cup of aqua, vomiting
without blood, vomiting not blackish, not green, and vomiting doesn't spray.
Since 1 week before patient entered the hospital, the patient complained
of rashes appeared on the legs to the buttocks and arms. The rashes is
palpable, the red color of the rash does not disappear when touched, the
rashes does not itch and does not hurt. The rashes appeared suddenly and
there is no previous trauma history.

d. History of Past Illness

Disease Age
Upper Respiratory Infection (e.g. + (8 years old)
Pharyngitis/Tonsilitis)
Diarrhea -
Otitis -
Pneumonia -
Tuberculosis -
Seizure -

3
 Heart -
Blood -
Diphtheria -
Measles -
Mumps -
Dengue fever -
Typhoid fever -
Worms infection -
Allergy -
Accident -

e. Allergy history
 Food allergy: denied
 Drugs allergy: denied
 Asthma bronchial: denied

f. Dietary history
Age Breast/formula Fruit/ Milk Steam Rice & Side
(years) milk biscuit porridge Rice dishes
0-6 ASI ad libitum - - - -
months
6 months- ASI ad libitum - Porridge - -
1 year 3x/day
1-2 years ASI ad libitum - Porridge - -
and formula 3x/day
milk (SGM)
2-5 years Formula milk Fruit/ - Steam
(SGM) biscuit rice
5-9 years - Fruit/ - - Rice & Side
biscuit dishes 2-
3x/day

4
g. Growth and Development history
First dentition : 6 months old
Gross motor
Head up : 1 month old
Prone : 4 months old
Sit : 6 months old
Crawl : 8 months old
Stand : 9 months old
Walk : 11 months old
Run : 13 months old
Fine motor
Reaches for objects : 3 months old
Puts objects in mouth : 6 months old
Throw objects : 8 months old
Scribbling paper : 14 months old
Social skills
Smile : 1 month old
Hugs and kisses : 11 months old
Initiates play : 13 months old
Imitates adult activities : 15 months old
Language skills
Vocalized : 1 months old
Laughs : 2 months old
Squeals : 2 months old
Jabbers : 6 months old
Speaks : 1 year old
Development disorder : None
Mental/emotion : Stable

5
h. Marital History
Mother’s Pregnancy History
The mother routinely checked her pregnancy to midwife. She denied any
problem noted during her pregnancy. She took vitamins routinely given.
Child’s Birth History
Maternity care : Midwife
Mode of delivery : Normal, spontaneous, no complication
Gestational age : 39 weeks
Child status :
 Weight of birth : 3200 gr
 Length of birth : 51 cm
 Head circumference : 34 cm
 Congenital anomaly :-
According to the mother, the baby started to cry and the baby's skin is red.

i. Immunization history
Immunization Frequency Time
BCG 1 time 1 month old
Hepatitis B 3 times 0, 1, 6 months old
DPT 3 times 2, 4, 6 months old
Polio 4 times 0, 2, 4, 6 months old
Hib 3 times 2, 4, 6 months old
Measles 2 times 9 months old, 6 years old
Varicella 1 times 7 years old

j. Family history
 Reproduction pattern
No Age Sex Alive Stillbirth Abor Death Health
tion (cause) status
1 9 yo Female V - - - Patient
2 7 yo Female V - - - Healthy

6
  Patient’s both parents were married when they were 27 years old and 24
years old, and this is their first marriage.
 There are not any significant illnesses or chronic illnesses in the family
declared.

k. History of Disease in Other Family Members / Around the House

There is no one living around their home known for having the same
condition as the patient.

C. PHYSICAL EXAMINATION
Physical examination was held on July 20th 2018 at Flamboyan ward
Bhayangkara Hospital, R. Said Sukanto Jakarta
a. General Examination
General condition : Mild ill
Awareness : Composmentis
Vital sign :
 Heart rate : 105 bpm
 Respiratory rate : 25 times/min
 Temperature : 36,6C
Anthropometry :
 Body weight : 27 kg
 Body height : 134 cm
b. Nutritional Status
Nutritional status measured based on National Center for Health Statistics
(2000):
 WFA : 27/29 x100 = 93% (Good)
 HFA : 134/133 x 100 = 101% (Good)
 WFH : 27/30 x 100 = 90% (Good)

7
 Conclusion: Nutrition status of the patient is good

c. Head to Toe Examination

Head :
 Measurement : Normocephalic
 Hair and scalp : Black, normal distribution, strong
 Eyes : Pale conjunctiva -/-, icteric sclera -/-, pupil isokor
3/3 mm, direct and indirect light response +/+ and +/+
 Ears : Normotia, secret/cerumen -/-, hyperemia -/-
 Nose : Septum deviation (-), nostril breathing (-), edema
conca -/-, secret (-)
 Mouth
- Lips : Moist

8
 - Teeth : Caries dentist (-)
- Tongue : Clean, tremor (-)
- Tonsil : T1/T1, detritus (-), wide crypt (-)
- Pharynx : Hyperemia (-)
Neck : No enlargement of lymph node
Thorax :
 Chest wall : Retraction (-)
 Pulmo :
- Inspection : Symmetric when static and dynamic
- Palpation : Vocal fremitus +/+
- Percussion : Sonor +/+
- Auscultation : Vesicular +/+, rhonchi -/-, wheezing -/-
 Cor :
- Inspection : Ictus cordis can’t be seen
- Palpation : Ictus cordis felt in ICS V Linea MCS without thrill
- Percussion :
Margin of right heart : ICS IV Linea PSD
Margin of left heart : ICS V Linea MCS
Margin of waist heart : ICS III Linea PSS
- Auscultation : S1S2 regular, murmur (-), gallop (-)
 Abdomen:
- Inspection : Even, there is no a widening of the veins, no spider
nevi
- Auscultation : Bowel sound (+) normal
- Palpation : Hepar and lien not palpable, epigastric and
umbilical tenderness (+)
- Percussion : Tympani
- Other : Ballotement (-)
 Anal and rectum : anal exist, diaper rash (-), no abnormalities
 Genital:
Pubic hair :-
Mons pubis : Tanner’s stage 1

9
  Extremities : Warm +/+/+/+, edema -/-/-/-, CRT < 2s, normal
ROM
 Skin : Cyanosis (-), icteric (-), palpable purpura in legs to
buttocks and hands (+)
 Vertebrae : Deformity (-), gibbus (-), kyphosis (-), scoliosis (-),
lordosis (-)
d. Neurologic examination
 Physiologic reflex:
- Brachioradialis : +2/+2
- Biceps : +2/+2
- Triceps : +2/+2
- Patella : +2/+2
- Achilles : +2/+2
 Pathologic reflex :-
 Motoric :5555 5555
5555 5555
 Meningeal sign :-

10
 Fig. Patient’s condition on the 1st day in hospital

D. LABORATORIUM EXAMINATION
a. Routine blood count (20/072018)
Results Normal range
Haemoglobin 13,7 g/dl 12-14 g/dl
Leucocyte 13.100 u/l 5.000-10.000 u/l
Haematocrit 41% 37-43 %
Thrombocyte 630.000 /ul 150.000-400.000 /ul

b. Biochemical profile (21/07/2018)

Results Normal range
Ureum 10 mg/dl 10-50 mg/dl
Creatinine 0,5 mg/dl 0,5-1,3 mg/dl
GFR estimated (CKD- -  90 ml/min/1,73m2
EPI)

c. Urine (21/07/2018)
Results Normal range
Color Yellow
Clarity Clear
Reaction/ Ph 7,5 5-8,5
Density 1.015 1.000-1.030

11
 Protein - Negative
Bilirubin - Negative
Glucose - Negative
Keton - Negative
Blood/ Hb - Negative
Nitrit - Negative
Urobilinogen 0,1 0,1-1,0 IU
Leucocyte - Negative
Sediment:
 Leucocyte 0-2 0-5
 Eritrocyte 0-2 1-3
 Epithel -
 Cilinder -
 Crystal -
Other -

E. SUMMARY
A 9 years old girl came to emergency room, Bhayangkari R. Said Sukanto’s
hospital with her parents because of abdominal pain 1 week before patient
entered the hospital. Complaints are associated with rashes on the legs to the
buttocks and arms, nausea, and vomiting. On physical examination patient
looked mild ill, abdomen: epigastric tenderness (+), and palpable purpura
appear in legs to buttocks and hands, from additional examination theres:
Hemoglobin 13,8 g/dl, leucocyte 13.100 u/l, haematocrit 41%, and
thrombocyte 630.000 /ul.

F. WORKING DIAGNOSIS
1. Henoch-schonlein purpura
2. Gastritis
3. Normal Growth Status
4. Good Nutritional Status

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 5. Complete Immunization Status

G. PROGNOSIS
Quo ad vitam: ad bonam
Quo ad functionam: ad bonam
Quo ad sanationam: dubia ad bonam

H. TREATMENT
 IVFD RL 1500 cc/24 hr (20 tpm macro)
 Cefotaxime 3x600 mg IV
 Dexamethasone 3x1,5 mg IV
 Rantin 2x50 mg IV
 Ondancentron 3x3 mg IV

13
FOLLOW UP
Day-2 (21-07-2018)
S : Abdominal pain (+), rash on legs and arm (+), nausea (+), vomiting (-)
O :
 General appearance: look well
 Vital sign:
- HR: 94 bpm
- RR: 20 x/min
- T: 36,7C
 Eye: pale conjunctiva -/-, icteric sclerae -/-, sunken -/-, pupil isokor 3/3
mm, direct and indirect light response +/+ and +/+
 Mouth: coated tongue (-), dry mucousa (-)
 ENT: hyperaemic pharynx (-), tonsil T1-T1
 Lungs: vesicular +/+, rhonki -/-, wheezing -/-
 Heart: S1S2 regular, murmur (-), gallop (-)
 Abdomen:
- Inspection: even
- Auscultation: normal bowel sound
- Palpation: hepar and lien not palpable, epigastric and umbilical
tenderness (+)
- Percussion: tympani
- Ballotement -/-
 Extremity: warm, CRT < 2 s, edema (-)
 Skin: Purpura in legs to buttocks and arm (+)
A : Henoch-schonlein purpura, gastritis
P :
 IVFD RL 1500 cc/24 hr (20 tpm macro)
 Cefotaxime 3x600 mg IV (H2)
 Methylprednisolone 3x8 mg PO (H1)
 Ondancentron 3x3 mg IV
 Omeprazole 2x20 mg IV
 Sucralfat 4x1 cth

14
Day-3 (22-07-18)
S : Abdominal pain decreased, rash on legs and arm (+), nausea (+)
O :
 General appearance: look well
 Vital sign:
- HR: 89 bpm
- RR: 20 x/min
- T: 36,4C
 Eye: pale conjunctiva -/-, icteric sclerae -/-, sunken -/-, pupil isokor 3/3
mm, direct and indirect light response +/+ and +/+
 Mouth: coated tongue (-), dry mucousa (-)
 ENT: hyperaemic pharynx (-), tonsil T1-T1
 Lungs: vesicular +/+, rhonki -/-, wheezing -/-
 Heart: S1S2 regular, murmur (-), gallop (-)
 Abdomen:
- Inspection: even
- Auscultation: Normal bowel sound
- Palpation: hepar and lien not palpable, epigastric and umbilical
tenderness (+)
- Percussion: tympani
- Ballotement -/-
 Extremity: warm, CRT < 2 s, edema (-)

15
  Skin: Purpura in legs to buttocks and arm (+)
A : Henoch-schonlein purpura, gastritis
P :
 IVFD RL 1500 cc/24 hr (20 tpm macro)
 Cefotaxime 3x600 mg IV (H3)
 Methylprednisolone 3x8 mg PO (H2)
 Ondancentron 3x3 mg IV
 Omeprazole 2x20 mg IV
 Sucralfat 4x1 cth

Day-4 (23-07-18)
S : Abdominal pain (-), rash on legs and hands decreased, nausea (-)
O :
 General appearance: look well
 Vital sign:
- HR: 92bpm
- RR: 22 x/min
- T: 36,6C
 Eye: pale conjunctiva -/-, icteric sclerae -/-, sunken -/-, pupil isokor 3/3
mm, direct and indirect light response +/+ and +/+
 Mouth: coated tongue (-), dry mucousa (-)
 ENT: hyperaemic pharynx (-), tonsil T1-T1
 Lungs: vesicular +/+, rhonki -/-, wheezing -/-
 Heart: S1S2 regular, murmur (-), gallop (-)

16
  Abdomen:
o Inspection: even
o Auscultation: Normal bowel sound
o Palpation: hepar and lien not palpable, epigastric and umbilical
tenderness (-)
o Percussion: tympani
o Ballotement -/-
 Extremity: warm, CRT < 2 s, edema (-)
 Skin: Purpura in legs to buttocks and arm (+) but decreased
A : Henoch-schonlein purpura, acute gastritis
P :
 IVFD RL 1500 cc/24 hr (20 tpm macro)
 Cefotaxime 3x600 mg IV (H4)
 Methylprednisolone 3x8 mg PO (H3)
 Ondancentron 3x3 mg IV
 Omeprazole 2x20 mg IV
 Sucralfat 4x1 cth

17
 CHAPTER II
LITERATURE REVIEW

2. Henoch-Schönlein Purpura
2.1 Definition
Henoch Schonlein Purpura (HSP) is a non-thrombocytopenic systemic
vasculitis of childhood characterised by small-vessel leukocytoclastic vasculitis
with the deposition of immunoglobulin A (IgA)-containing immune complexes in
the walls of small vessels (arterioles, capillaries and venules) which can affect the
skin, joints, bowel, and kidneys. It is also known as IgA vasculitis (IgAV).
According to the recently endorsed European League Against Rheumatism,
Paediatric Rheumatology European Society and Paediatric Rheumatology
International Trials Organisation classification of childhood vasculitis, HSP
belongs to the group of non- granulomatous, predominantly small vessel
vasculitides (Table 1).1,2,3

18
2.2 Epidemiology
HSP is mainly a disease of children that occurs predominantly between the ages
of 3 and 15 years. About 50% of all cases occur at or before the age of 5 years. In
North America, the annual incidence in children is around 13.5 cases per 100,000
population, with white people having the highest incidence and black people having
the lowest incidence. Males are more often affected than females by a ratio of up to
2:1. In the UK, Asian people are more affected than white people, with black people
having the lowest incidence. In a survey published in the UK, results revealed that
the HSP incidence was higher than previously expected, at 22.1 cases per 100,000
population. The true prevalence may be underestimated because cases are often not
reported. HSP can follow a URI, and as a result it mainly occurs in the fall, winter,
and spring. Certain autoimmune risk factors, such as complement deficiencies, and
hereditary fever syndromes, such as Familial Mediterranean Fever syndrome, may
predispose a child to HSP.4,5

2.3 Etiology
The underlying cause of HSP remains unknown. It is an immune-mediated
vasculitis, with a variety of infectious and chemical triggers having been proposed
as a cause. Many cases of HSP occur after a URI, especially streptococcal
infections. In addition, a complex interaction of several factors including age, sex,
ethnic background, and environmental influences is probably involved. Genetic
factors also seem to be implicated in HSP, with genes encoding host defense
molecules probably being triggered by environmental agents. Some cases of HSP
may be drug-related (e.g., penicillin, cefaclor, minocycline, hydralazine, or
phenytoin); however, the relationship is unclear.4

19
 Table 2. Etiology associations with HSP6

2.4 Pathophysiology
Antigen and antibody complexes, mostly IgA, form as a result of bacterial and
viral infections, vaccinations, drugs, and autoimmune mechanisms. These antigen
antibody complexes deposit in the small vessel walls and activate the alternate
complement pathway which leads to neutrophil accumulation resulting in
inflammation and vasculitis without a granulomatous reaction. This can involve
multiple systems including skin, gastrointestinal tract, kidney, and joints but it can
involve any organ system. Vasculitis causes extravasation of blood and its
components into the interstitial spaces resulting in edema and hemorrhage.6
Upper respiratory tract infections precede a majority of HSP cases and multiple
case studies propose a correlation between practically all respiratory pathogens and
HSP. Streptococcus strains and Parain uenza virus are the most commonly
associated pathogens, and in children Human Parvovirus B19 is a frequent viral
trigger. The interaction between leukocytes and vascular endothelial cells
contributes to the pathogenesis of HSP. Endothelial damage, perivascular

20
leukocytic infiltrates, chemokines and cytokines are important factors in this
process. Vascular deposition of IgA1-containing immune complexes plays a
pathogenic role. Complement activation, cellular damaging and IgA deposition
suggest that HSP is an IgA-mediated dysregulated immune response to an antigen.
Through binding and activation of complement factors, IgA cross-reacts with
endothelial cells and damages the cells. Advances in technology have allowed
updated data on the function of the human immune system and what happens when
it fails. In HSP, the dysregulated immune response may result in inflammation and
vasculitis without a granulomatous reaction. Several antibodies, cytokines,
chemokines, receptors, and transmembrane proteins have been found to be
involved. Amongst these are cytokines, such as tumour necrosis factor alpha (TNF-
alpha), interleukin (IL)-6, and IL-8 (38). Studies showed that Toll-like receptors
TLR-2 and TLR-4 were upregulated in children with HSP. These proteins are
mainly expressed, regulated and produced by cells of the immune system, including
macrophages and lymphocytes. They may also arise from non-immune cells, such
as epidermal cells, broblasts, kidney podocytes and mesangial cells. One study
stated that plasma levels of IgA anti-beta2-glycoprotein I antibodies are increased
in childhood HSP. They are thought to have a strong association with heavy
proteinuria and joint manifestations.7
GENETICS
Genetic predisposition may contribute to the development of HSP. An Israeli
study demonstrated that 10% of patients with HSP were homozygous for mutations
of the gene encoding MEFV (the gene defective in familial Mediterranean fever),
and additionally 17% had heterozygous defects. In comparison, in a randomly
selected cohort in the general Israeli population, only 1–2% carried 2 mutant alleles.
MEFV encodes the protein pyrin/marenostrin, which regulates caspase-1-activation
and IL-1B production. Human leukocyte antigen haplotypes may also play a role in
susceptibility to HSP. A study on children with HSP showed an increased risk for
the development of HSP in children carrying human leukocyte antigen A2, A11 and
B35 antigens, and a reduced risk in the carriers of HLA A1, B49 and B50 antigens.7

21
 Fig. 1 Schematic diagram of HSP pathophysiology6

2.5 Clinical Manifestation

The most severely affected organs and locations are the skin, joints, digestive
organs, and kidneys. Although children are most commonly affected, HSP may also
occur in adults. It may be preceded by a headache, pharyngeal pain, and symptoms
of the common cold. Disseminated palpable purpura of several millimeters to 10
mm in diameter occur, mainly in the lower extremities and dorsum of the foot, but
sometimes on the thighs, upper extremities, and abdomen. Blisters, ulcers, and old
and new eruptions may be present together. In some cases there is transient edema
with slight pressure pain. Arthritic symptoms in the legs, knees, hands and elbows,
sharp pain in the abdomen, and gastrointestinal symptoms such as nausea, vomiting,
hematemesis, and melena are found. HSP may be accompanied by renal symptoms
including acute nephritis that progresses to nephrosis. Renal symptoms are closely
related to the prognosis.8
HSP is a systemic vasculitis with multiorgan involvement. The classic tetrad
of signs and symptoms includes: 1) palpable purpura, 2) arthritis or arthralgia, 3)
abdominal pain, and 4) renal disease.

22
 Purpura
Skin involvement is present in all children with HSP. Petechiae and palpable
purpura are the most common, but erythematous, macular, urticarial or even
bullous rashes have also been observed. Purpura is characteristically
distributed symmetrically over the extensor surfaces of the lower limbs,
buttocks and forearms with involvement of trunk and face described
occasionally in younger children. Recurrence of purpura, which might be
associated with more severe renal involvement, is observed in 25% of
children with HSP.

Fig. 2 Purpuric skin changes in a patient with HSP

 Arthritis/arthralgia
Arthritis/arthralgia is present in three quarters of children with HSP. Joint
involvement is usually oligoarticular with large joints of the lower
extremities (knee, ankle, hip) most commonly affected. There is usually
prominent periarticular swelling, tenderness and pain; erythema and joint
effusion are rare. Arthritis is non-deforming and heals without chronic
damage within a few weeks.
 Abdominal pain
Approximately two thirds of children with HSP develop abdominal pain,
usually diffuse, increasing after meals, and sometimes associated with
nausea and vomiting. These symptoms are caused by submucosal
haemorrhage and oedema of the bowel wall, predominantly affecting the
proximal small bowel. The most severe gastrointestinal complication is
intussusception, affecting 3–4% patients with HSP. In 60% of these cases,
it is limited to small bowel. Clinical presentation of intussusception is

23
 characterised by severe abdominal pain, often colicky in nature and
vomiting. Other significant, though less common gastrointestinal
complications are gangrene of the bowel, bowel perforation and massive
haemorrhage.
 Renal disease
Renal involvement is reported in 20–55% of children with HSP. The most
common finding is isolated microscopic haematuria, usually developing
within 4 weeks of the onset of the disease. Proteinuria of variable degree
might be present, and if severe can present as nephrotic syndrome.
Hypertension might develop at the onset or during recovery. Renal function
is usually normal but the occasional patient might present with a progressive
glomerulonephritis with significant renal impairment.
 Other less common clinical manifestations of HSP include cerebral
vasculitis, scrotal or testicular haemorrhage, and interstitial pulmonary
haemorrhage. Distal ureteric vasculitis resulting in ureteric stenosis,
presenting as renal colic has also been described.

Table 3 Clinical features associated with Henoch–Schönlein purpura

24
2.6 Diagnosis
HSP is usually diagnosed clinically, based on characteristic features in the
history and physical exam; laboratory tests are nonspecific. The classic tetrad of
rash, polyarthralgias, abdominal pain, and renal disease usually makes the diagnosis
straightforward.
However, while all patients with HSP will present with a characteristic rash, not
all patients present with the other classic symptoms. In patients with an unusual
presentation, such as pulmonary hemorrhage, headaches, or seizures followed by
the development of a rash, a biopsy of an affected organ such as skin or kidney will
show IgA deposition, which confirms the diagnosis of HSP. The American College
of Rheumatology has developed criteria for the diagnosis of HSP. The presence of
2 or more of the criteria (i.e., either palpable purpura, age at onset <20 years,
abdominal pain, or on biopsy showing granulocytes in the walls of small arterioles
or venules in superficial layers of skin) has a sensitivity of 87.1% and a specificity
of 87.7%. In 2010, the criteria for Henoch-Schonlein purpura were updated by the
European League Against Rheumatism (EULAR), the Paediatric Rheumatology
International Trials Organisation (PRINTO), and the Paediatric Rheumatology
European Society (PRES). Purpura or petechiae (mandatory) with lower limb
predominance and at least 1 of the 4 following criteria:
 Abdominal pain
 Histopathology: typical leucocytoclastic vasculitis with predominant IgA
deposits or proliferative glomerulonephritis with predominant IgA deposits
 Arthritis or arthralgia
 Renal involvement (proteinuria: > 0.3g/24 h or > 30mmol/mg of urine
albumin to creatinine ratio on a spot morning sample; and/or hematuria, red
blood cell casts: > 5 red cells per high power field or ≥2+ on dipstick or red
blood cell casts in the urinary sediment).4,9
A. History
History typically reveals the development of a rash, arthralgias, and abdominal
pain. Exploration of aggravating factors may reveal symptoms occurred following
a URI or drug ingestion (a full drug history should be taken). Uncommon symptoms
may include testicular swelling or pain, headaches, hemoptysis, or seizures.4

25
B. Physical examination
Skin lesions are characterized as palpable purpura and are typically
nonblanching. They can occur anywhere on the body, but are usually concentrated
on the lower extremities. Polyarthralgia can be present and is often associated with
edema as well as abdominal pain on examination. Scrotal pain and swelling may
occur in about 13% of boys with HSP. Neurologic examination will rarely reveal
focal deficits. Pulmonary hemorrhage is a rare presentation.4
C. Laboratory evaluation
There are no laboratory tests that are diagnostic for HSP; routine tests are not
specific. However, urinalysis should be done in all patients with suspected HSP to
assess the degree of renal involvement. Serum creatinine and electrolyte levels
should be considered in all patients with an abnormal urinalysis. Serum IgA levels
may be elevated, but this is not a specific test for HSP. Coagulation studies can
be considered to help exclude other causes. Laboratory evaluation including
antinuclear antibodies, antineutrophil cytoplasmic antibodies, and complement
levels are typically negative can help differentiate HSP from other vasculitides.4
Table 4 Recommended initial investigations for Henoch–Schönlein purpura10

D. Biopsy
Biopsy of the affected skin reveals leukocytoclastic vasculitis with deposition
of IgA-containing immune complexes, predominantly in small vessels in the
papillary dermis (primarily venules). Neutrophils undergo destruction
(leukocytoclasis) with destructive fragmentation of the nuclei of dying cells
(karyorrhexis) during apoptosis or necrosis. Deposits of IgA and C3 in the dermal
capillaries of purpuric lesions and uninvolved skin by immunefluorescent staining
are considered valid diagnostic criterion, with 100% specificity in combination with
leukocytoclastic vasculitis.
Kidney biopsy is usually performed in patients with uncertain diagnosis and in
those with more severe kidney involvement (rapidly progressive nephritis,

26
nephrotic syndrome). In general, there is a correlation between the severity of renal
manifestations and findings on kidney biopsy. Light microscopy findings can range
from mild mesangial proliferation to severe crescentic glomerulonephritis. Diffuse
mesangial IgA deposits seen on immunofluorescence are the hallmark of HSP
nephritis and co-deposition of C3 complement (75%) might also be present. The
absence of the classical complement pathway components (C1q and C4)
distinguishes HSP nephritis from other forms of immune-mediated
glomerulonephritis, such as lupus nephritis. Electron microscopy shows electron
dense deposits in the mesangial areas. The current classification of HSP nephritis
is based on the extent of proliferation and the presence of crescents on light
microscopy, but other histological findings, such as mesangial/subendothelial
deposits, the extent of tubulointerstitial damage or glomerular sclerosis might be
better predictors of the outcome.3

Fig. 3 Leukocytoclastic vasculitis of the skin in a child with Henoch-Schönlein

purpura. Superficial dermal vessels showing inflammatory infiltrate consisting
predominantly of neutrophils and eosinophils (arrows) [haematoxyillin/eosin;
magnification × 200].3

27
Fig. 4 Deposition of IgA immunoglobulin in Henoch–Schönlein purpura nephritis.
Immunohistological staining demonstrates granular deposition of IgA
immunoglobulin in the mesangium of the affected glomerulus [mag- nification ×
200].3
E. Imaging
Imaging by ultrasound is indicated for severe abdominal pain to evaluate for
intussusception or perforation. It may also be used to evaluate testicular pain and
swelling. Abdominal ultrasound is the technique of choice with the accuracy in
diagnosing intussusception approaching 100% in experienced hands. Concentric
rings of tissue representing components of bowel and mesenteric fat create a classic
‘target sign’. The classic ‘meniscus sign’ of intussusception on contrast enema,
where the apex of the intussusception projects into the contrast material, is not
present in cases of intussusception limited to small bowel.3,4

Fig. 5 Target sign on transverse ultrasound of an intussusception. The concentric

mass represents the tissue layers in the bowel wall of the intussusceptum and the
intussuscipiens. The curved, echogenic (bright) area is due to the trapped
mesenteric fat.3

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2.7 Differential diagnosis
Children (less than 17 years of age) presenting with palpable purpura and
multisystem involvement (GI, kidney, and joints) without thrombocytopenia may
be diagnosed as HSP. The differential diagnosis of HSP includes conditions such
as Crohn’s disease, Wegener’s granulomatosis, infective endocarditis, IgA
nephropathy, and hemolytic uremic syndrome. Hypersensitivity vasculitis can
present as leukocytoclastic vasculitis involving the skin (palpable purpura) and
rarely, the gastrointestinal tract, but unlike HSP, IgA deposition is not seen. In
Crohn’s disease and IgA nephropathy, there is no palpable purpura.11
Table 5. Differential diagnosis for Henoch–Schönlein purpura11

2.8 Complication
Orchitis and scrotal swelling may occur in up to 35 percent of boys with
Henoch-Schönlein purpura. Severe scrotal edema may cause testicular torsion,
which requires emergent surgical exploration. Fewer than 10 percent of patients
with Henoch-Schönlein purpura experience myocardial infarction, pulmonary
hemorrhage, or central nervous system involvement with seizures and
haemorrhage.12

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 Table 6. Potential complications of Henoch-Schönlein purpura12

2.9 Treatment
Management of HSP includes supportive care, symptomatic therapy and, in
some cases, immunosuppressive treatment.
The basic principles of supportive care consist of maintenance of good
hydration, symptomatic pain relief and monitoring for the development of
complications. If adequate hydration cannot be maintained orally, intravenous
fluids should be considered. Parenteral nutrition is usually unnecessary, except in
cases with prolonged severe abdominal involvement precluding enteral feeding.
Patients with severe abdominal pain need prompt evaluation and investigations to
exclude intussusception. In cases with sudden change of mental status, intracranial
haemorrhage should be excluded with appropriate imaging. Arthritis/arthralgia
usually responds well to non-steroidal anti-inflammatory drugs (NSAIDs), but
occasionally requires opioids for adequate symptomatic relief. Treatment is usually
well tolerated and is not associated with an increased risk of gastrointestinal
bleeding. Patients with compromised renal function taking NSAIDs need close
monitoring of their fluid status, blood pressure and renal function.
The use of glucocorticosteroids (GCS) in HSP has been source of controversy
for many years. While the suggested benefits of early GCS treatment have included
shortened duration of abdominal pain, decreased risk of intussusception and

30
decreased risk of surgical intervention, the quality of evidence is generally poor,
having come from mostly from small studies or case reports. In clinical practice,
short courses of GCS are being used in patients with severe abdominal pain, usually
with rapid symptomatic improvement. This treatment cannot be recommended in
all patients with HSP since the majority will improve spontaneously. While some
reports have suggested that early treatment with GCS might prevent development
of nephritis and chronic kidney disease, a recent Cochrane review concluded that
there is no evidence from randomised controlled trials that the use of GCS prevents
kidney disease in children with HSP.
Immunosuppressive treatment of HSP nephritis is used in patients with severe
kidney involvement (nephrotic range proteinuria and/or progressive renal
impairment). In these cases, renal biopsy should be considered before treatment.
Mild renal involvement (microscopic haematuria or mild proteinuria) does not
require biopsy or immunosuppressive treatment, but these children need close
follow-up.
In patients with rapidly progressive glomerulonephritis or nephrotic syndrome
(usually accompanied by crescents on kidney biopsy), pulse intravenous
methylprednisolone followed by 3 to 6-month course of oral steroids is most
commonly used. A current KDIGO guideline suggests adding cyclophosphamide
to steroid treatment for crescentic glomerulonephritis even though the quality of
evidence is low with a lack of demonstrated improvement in renal outcome.
Plasmapheresis has also been used in children with rapidly progressive
glomerulonephritis, but it is difficult to assess its efficacy due to selection bias (used
in the most severe cases) and concurrent administration of other
immunosuppressive treatments. Recent studies in children with HSP nephritis and
nephrotic syndrome suggest a potential benefit of cyclosporine A (CsA) in
achieving remission of proteinuria and histological improve- ment of nephritis on
follow-up kidney biopsies. Other treatments used with some success in small
studies include intravenous immunoglobulin, combined therapy of immuno-
suppression and anti-clotting therapy (warfarin, dipyridamol and acetylsalicylic
acid), tonsillectomy, and B-cell depletion with rituximab and mycophenolate

31
mofetil. The efficacy of these treatments is yet to be tested in prospective clinical
trials.
Use of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin
receptor blockers (ARBs) has become an accepted treatment of HSP nephritis with
persistent proteinuria, with beneficial effects not only on reduction of proteinuria
but also on inhibition of renal fibrosis. Although there are no available studies on
the efficacy of ACEIs or ARBs in HSP nephritis, long-term data showing their
beneficial effect on renal survival and improvement of proteinuria in patients with
IgA nephropathy, a disease with the same pathophysiology, are encouraging.3
Table 8. Suggested management of various manifestations of Henoch-Schonlein
purpura13

2.10 Prognosis
In the majority of children, the outcome of HSP is excellent with spontaneous
resolution of symptoms and signs. HSP recurs in approximately one third of
patients, typically within 4 months of the initial presentation. Recurrent purpura can
be occasionally associated with joint complaints and episodes of gross haematuria
although each subsequent episode is generally milder and shorter. The long-term
morbidity of HSP is related to the degree of HSP nephritis.
In unselected cohorts of children, HSP nephritis is a mild disease, characterised
by microscopic haematuria and minimal proteinuria, with <1% risk of progression
to end-stage kidney disease (ESKD). Reports from tertiary centres indicating that
10–30% of children will develop ESKD are likely to overestimate the true risk of
ESKD due to the selection of cases with more severe renal impairment seen in these
centres. Children at risk are those with nephrotic or nephritic/nephrotic syndrome
or renal failure at presentation, and those with impaired kidney function and

32
persistent proteinuria after several years of follow-up. Children with uncomplicated
HSP are usually managed in the primary care setting either by a GP or a
paediatrician. The aim of the initial follow-up is to identify patients with worsening
kidney involvement and is based on serial urinalyses, blood pressure measurement,
blood tests to assess kidney function and exclusion of other causes of
glomerulonephritis. A practical pathway for detection and referral of children with
HSP nephritis to a paediatric nephrologist during the first 6–12 months after
diagnosis has been developed. The involvement of a paediatric rheumatologist in
cases of severe arthritis/arthralgia might also be warranted.
Histological recurrence of HSP nephritis (IgA deposition) in transplanted
kidneys can be as high as 60% but is rarely associated with clinical recurrence.
Long-term outcomes of transplanted kidneys in patients with HSP nephritis are
com- parable to other primary diseases with ∼90% survival at 10 years.3
In general, most of the HSP cases are self-limited, with good prognosis and
five-year survival rates of 95%. One third of the patients have relapses, which are
milder and shorter in duration, usually within 4 months, and involving the same
organs. The prognosis depends on the age of onset, extent of renal involvement and
its course, extent of skin involvement, particularly above the waist line,
immunoglobulin imbalance, and neurological involvement. In adults, a 75%
survival rate seen at 14.8 years may not be solely related to intrinsic HSP, as there
may be other comorbid conditions.6
Henoch-Schönlein purpura might reccur in up to 33% of affected children.
Recurrences appear to be more common in older children (>8 years of age at onset)
and in children with renal involvement. Current research indicates placebo is as
good as prednisone in preventing HSP recurrence after 1 month, with 1 RCT finding
recurrence rates of 17% and 25% in the placebo and prednisone (1 mg/kg daily)
groups, respectively (P = .22). A second RCT, as well as a systematic review of
both prospective and retrospective trials, supported these findings, reporting no
statistically significant difference in recurrence rates after 1 month between the
placebo and prednisone groups, with considerable heterogeneity in the retrospective
observational trials.5

33
Table 9. Prognostic factors for Henoch-Schönlein purpura6

34
 CHAPTER III
CASE ANALYSIS

Theory Case
Etiology The underlying cause is unknown, but Unknown
has many trigger factor
Clinical - Skin: Palpable purpura - Palpable purpura
Manifestation - Arthritis/arthralgia - Abdominal pain,
- Abdominal pain, increasing after increasing after
meals, and sometimes associated meals, nausea and
with nausea and vomiting vomiting
- Renal disease: Hypertension,
glomerulonephritis, nephrotic
syndrome
- Other: cerebral vasculitis, scrotal or
testicular haemorrhage, and
interstitial pulmonary haemorrhage
Diagnosis History: the development of a rash, - Rashes appeared on
arthralgias, and abdominal pain the legs to the
buttocks and arms.
The rashes palpable,
the red color of the
rash does not
disappear when
touched, the rashes
does not itch and
does not hurt.
- Abdominal pain,
diffuse but
frequently hurts in
the upper middle to
around the navel,

35
 increasing after
meals
- Nausea and
vomiting
Physical examination: palpable Palpable purpura in
purpura on body, but usually legs to buttocks and
concentrated on the lower hands, epigastric and
extremities. Abdominal tenderness. umbilical tenderness
(+)
Supporting investigation: - Laboratory
- Laboratory evaluation: evaluation:
1. Hematology: anemia and/or 1. Hematology:
leucocytosis leucocytosis and
2. Biochemical profile: renal thrombocytosis
function may be altered with 2. Biochemical
raised creatinine profile: normal
3. Urine: haematuria, proteinuria (if ureum and
renal disease occurs) creatinine
- Biopsy: 3. Urine: normal
1. Skin: leukocytoclastic vasculitis Biopsy and imaging
with deposition of IgA- are not done.
containing immune complexes,
predominantly in small vessels
in the papillary dermis (primarily
venules)
2. Renal: diffuse mesangial IgA
deposits
- Imaging: target sign on transverse
ultrasound of an intussusception
Criteria The American College of - Palpable purpura
Rheumatology

36
2 or more of the criteria - Age at onset < 20
below: years (patient’s age
- Palpable purpura is 9 years old)
- Age at onset < 20 years - Abdominal pain
- Abdominal pain
- On biopsy showing
granulocytes in the walls
of small arterioles or
venules in superficial
layers of skin
EULAR/PRINTO/PRES - Palpable purpura,
Palpable purpura, not not
thrombocytopenic/petechiae thrombocytopenic
(mandatory) with lower with lower limb
limb predominance + ≥ 1 of predominance
the following: - Abdominal pain
- Abdominal pain
- Histopathology: typical
leucocytoclastic
vasculitis with
predominant IgA
deposits/proliferative
glomerulonephritis with
predominant IgA deposits
- Arthritis or arthralgia
- Renal involvement
(proteinuria and/or
hematuria)

37
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