Supervisor : dr. Pulung Silalahi, Sp.

A
 BY : ANNISA YUNITA RANI
 1102014035
 DEPARTMENT OF PEDIATRIC
 RADEN SAID SUKANTO POLICE CENTER HOSPITAL
 DEFINITION
Henoch-Schönlein purpura is an autoimmune (IgA mediated)
disease of vasculitis hypersensitivity, most commonly found in
children. It is a clinical syndrome of inflammatory disorders of
generalized vasculitis of small blood vessels in the skin, joints,
gastrointestinal tract, and kidneys.
 EPIDEMIOLOGY

On average 14 cases/100,000 school-aged children;

the highest prevalence at age 2-11 years (75%); 27% of
cases are found in adults, rarely found in infants. More
in boys than girls (ratio 2: 1)
ANATOMY AND PHYSIOLOGY
 ETIOLOGY
Still not known for sure cause of HSP. Presumably several factors
play a role: IgA is thought to play an important
• genetic factors role, characterized by elevated
• upper respiratory tract infection serum IgA concentrations, immune
complexes, and IgA deposits in
• Food blood vessel walls and renal
• insect bites mesangium.
• exposure to cold
• immunization (varicella vaccine, rubella, rubeolla, hep.A)
• drugs (ampisillin,erythromycin, quinine, penicillin, quinidine,
quinine).
PATHOPHYSIOLOGY

(Renata,2012)
 MANIFESTATION
 (1/2 - 2/3 cases) ISPA 1-3 weeks earlier.
 Arthralgia and arthritis (68-75% of cases).
 Skin disorders are found in (95-100% of cases) in the form of macular rash,
petechiae and eczema, can be accompanied by itching, symmetry,
especially in the skin that is often exposed to pressure that is the back of
the legs, buttocks and sides of the ulna.
 Macular rash color change:
 abdominal dissorder (35-85% of cases) 1-4 weeks after onset. Abdominal
colic in periumbilikal, accompanied by nausea and vomiting. Bloody
diarrhea may accompany pruritic rash.
 MANIFESTATION
 (20-50% of cases) facial angioedema (eyelids, lips) and
extremities (back of hands and feet).
 Kidney disorders are found in 50% of cases high in children and
25% are found in children <2 years of age. <1% progresses to
renal failure.
 The risk of nephritis increases at the age of onset above 7
years, persistent purpura lesions, severe abdominal complaints
and decreased factor XIII.
 Scrotum abnormalities resemble testicular torsion; Scrotum
edema may occur early in the disease (2-35%).
 DIAGNOSIS

 Anamnesis (history, kidney involvement, recurrence, other signs of symptoms)

 Physical examination (inspection, palpation, percussion, auscultation)
 Supporting investigation
- H2TL: normal - ureum / creatinine
- serum: IgA ↑ - UL
- LED: ↑
- biopsy: granulocytes on arteriolar walls or venules
- Immunofluorescence: IgA deposit and complement on blood vessel walls.
 Immunofluorescence

deposit of
IgA and
complement
on the wall
blood vessel
 DIAGNOSIS
A. Kriteria American College of Rheumatology1990:
Bila memenuhi minimal 2 dari 4 gejala, yaitu:
1. Palpable purpura non trombositopenia 2. Onset gejala pertama < 20 tahun
3. Bowel angina 4. Pada biopsi ditemukan granulosit pada
dinding arteriol atau venula
B. Kriteria European League Against Rheumatism(EULAR) 2006 dan
Pediatric Rheumatology Society (PreS) 2006
1. Palpable purpura harus ada
2. Diikuti minimal satu gejala berikut: nyeri perut difus, deposisi IgA yang predominan
(pada biopsi kulit), artritis akut dan kelainan ginjal (hematuria dan atau proteinuria)
 Kriteria Definisi

Purpura non trombositopenia (palpable Lesi kulit hemoragik yang dapat diraba, terdapat
purpura) elevasi kulit, tidak berhubungan dengan
trombositopenia

Usia onset ≤ 20 tahun Onset gejala pertama ≤ 20 tahun

Gejala abdominal / gangguan saluran cerna Nyeri abdominal difus, memberat setelah makan
(Bowel angina) atau diagnosis iskemia usus, biasanya termasuk BAB
berdarah

Granulosit dinding pada biopsy Perubahan histologi menunjukkan granulosit pada

dinding arteriol atau venula

Tabel 1. Kriteria Diagnosis HSP

Buku Ajar Alergi-Imunologi Anak 2007
 Management
 Basically there is no specific treatment for HSP.
 Pain: ibuprofen or paracetamol 10 mg / kgBB.
 edema: elevation of the legs.
 vomiting and abdominal pain: soft diet
 corticosteroids in very severe conditions such as persistent nephrotic
syndrome, edema, gastrointestinal bleeding, severe abdominal pain,
central and pulmonary central nervous system involvement:
metilprednisolone 250-750 mg / day / iv for 3-7 days combined with
cyclophosphamide 100-200 mg / day
 Management

 for severe acute HSP phases; oral prednisone 100-200 mg daily and
cyclophosphamide 100-200 mg / day for 30-75 days before
cyclophosphamide is stopped directly and tapering off steroids up to 6
months. Patients with severe stomach pain, gastrointestinal bleeding or
decreased renal function, require hospitalization
 Prognosis

 Ad Bonam generally
 Ad malam : if there are complications of kidney failure
 REFERENCE
 Yuly, A. Purpura Henoch-Schönlein. Dalam Cermin Dunia Kedokteran Edisi 194 Volume 139 Nomor 6.
2012. Available at http://www.kalbe.co.id diakses tanggal 14 July 2014
 Matondang CS, Roma J. Purpura Henoch-Schonlein. Dalam: Akip AAP, Munazir Z, Kurniati N,
penyunting. Buku Ajar Alergi-Imunologi Anak. Edisi ke-2. Jakarta: Ikatan Dokter Anak Indonesia,
2007;373-7.
 Bossart P. Henoch-Schönlein Purpura. eMedicine, 2005. Diakses dari
www.emdecine.com/emerg/topic845.htm Diakses tanggal 14 July 2014
 Scheinfeld NS. Henoch-Schönlein Purpura. eMedicine, 2008. Diakses dari
www.emedicine.medscape.com/article/984105-overview Diakses tanggal 14 July 2014
 D’Alessandro DM. Is It Really Henoch-Schönlein Purpura. Pediatric Education, 2009. Diakses dari
http://www.pediatriceducation.org/2009/02/ Diakses tanggal 14 July 2014
 Kraft DM, McKee D, Scott C. Henoch-Schönlein Purpura: A Review. American Family Physician, 1998.
Diakses dari http://www.aafp.org/afp/980800ap/kraft.html Diakses tanggal 14 July 2014