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PURUPURA HENOCH SCHONLEIN TIPE BULA HEMORAGIK
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PURPURA HENOCH SCHONLEIN WITH HEMORRHAGIC BULLOUS SKIN LESIONMedina Athiah
Correction
Halaman 15 Jawaban konsultasi ke Subdivisi Nefrologi: Kesan: Nefritis PHS seharusnya PHS dengan proteinuria.
Halaman 16, 17, 19, 20 Masalah no. 2 Nefritis PHS seharusnya proteinuria
Halaman 18 proteinuria (-) nefritis PHS perbaikan seharusnya proteinuria (-) proteinuria selesai
Halaman 18 feses rutin darah samar (+) seharusnya darah samar (-)
Introduction
Henoch-Schonlein Purpura (HSP) or anaphylactoid purpura, is a vasculitis of the small blood vessels that
are often found in children.
PHS incidence ranged from 13.5 to 18 per 100,000 children.
The purpose of this case report presents a case about PHS with the form of hemorrhagic bullae which is quite
uncommon manifestations in children.
Identification
I/♂8 y.o 11 m.o.
Admitted Nov 30TH 2013
Mr. A31 y.o.
Junior High School
Ojek Driver
Mrs. S29 y.o
Junior High School
House wife
Patient MotherFather
CC Abdominal pain
Anamnesis
Rash on legsAC
Anamnesis
44 days b.a days b.a
22 day b.a day b.a
High feverNauseaNo appetiteBrought to the clinic Given 2 kinds of drugs (paracetamol and antibiotics)
Rash on the legs and buttocksJoint pain, especially in the knee and ankle and Could not walkFever.
Present Illness History
Anamnesis
3 days b.a3 days b.aMore rash on the legs Abdominal pain Fever Nausea Vomiting, 2 times/day, the contents of what is eaten, @ 1/4 glass of water Joint pain at the knee and ankleBrought to the clinic 24 hours given the 4 kinds of drugs (paracetamol, antibiotics, stomachache drug, and anti-allergy drugNo improvement ER of Moh Hoesin hospital
Present Illness History
Was treated at the Allergy Immunology, Moh Hoesin Hospital with a diagnosis of PHS in 2010
Anamnesis
Past I.H
•No history of same illnessFH
Anamnesis
Normal
Complete according age and oncourse
Good in quality and quantity
History of Pregnancy and labor
History of immunization
History of nutrition
Anamnesis
Normal
Middle low income
Good
DevelopmentalDevelopmental
Socio-economic Socio-economic
EnvironmentEnvironment
Physical Examination
Alert
Pulse 88 b/m (v&p equal)
Temperature 36,8o
Resp. Rate 22 t/m (regular)
Good Nutritional Status
General Findings
Physical Examination
Head: Normocephal, no anemia, no icteric, pupil: round, isocor, positive reflex, no nasal flare.Thoracic: symmetrical, no retraction.Cor: normal heart sound, no murmur.Pulmo: normal vesicular, no ronchi, no wheezing.
Abdominal: flat, tender, unpalpable liver, unpalpable spleen, epigastric tenderness, tymphani, normal bowel sound.Extremities: warm akral, CRT <2 seconds, multiple purpura, diameter of 1-2 cm scattered on the left and right cruris and dorsum pedis
Spesific Findings Spesific Findings
Normal Neurologic status
Initial Analysis
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Diagnosis
Henoch-SchonleinPurpura
14
Initial Problems
Purpura Henoch-Schonlein-Methyl prednisolone tablet 4 mg tablet 3x2 tablet-Diet 1800 kcal and 50 g protein per day-Monitoring for blood pressure, abdominal pain, bloody stool, rash
Problem(P)
P 1
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16
Progress Note (1-12-2013)
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Progress Note (3-12-2013)
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Progress Note (4-12-2013)
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Progress Note (5-12-2013)
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Progress Note (6-12-2013)
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Progress Note (7-12-2013)
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Progress Note (11-12-2013)
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Progress Note (12-12-2013)
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Literature Review
History
Henoch-Schonlein Purpura (PHS) (1801) vasculitis of small blood vessels, was first described by William and Heberden.
Lucas Schonlein (1832) describe abnormalities in the form of purpura and arthralgia.
Eduard Henoch (1874) find a relationship with symptoms of gastrointestinal and genitourinary systems.
Definition
PHS (anaphylactoid purpura / purpura nontrombositopenik) clinical syndrome caused by systemic small blood vessels vasculitis characterized by specific skin lesions such as purpura nontrombositopenik, arthritis or arthralgia, abdominal pain or gastrointestinal bleeding, and occasionally nephritis or hematuria
Epidemiology
PHS incidence from 13.5 to 18 per 100,000 children.
PHS occurs at the age of 6 months to adults, 50% of cases occur in children less than 5 years, 75% at age below 10 years
Male: Female = 1.5-2: 1
Etiology Upper respiratory tract infection (60-75%) Bacteria and viruses such as Streptococcus grup A,
Yersinia sp, Legionela sp, Mycoplasma, Epstein-Barr, Varicella, Parvovirus B19, Adenovirus dan Campylobacter enteritis.
Post-vaccination, such as typhoid and paratyphoid A and B, Measles, Varicella, Rubella, and Hepatitis B.
Drugs, such as Penicillin, Ampicillin, Quinin, etc/ Foods, weather and insect bites. Genetic factors. Malignancies, leukemia and lymphoma.
Pathophysiology
Pathophysiology
Pathophysiology of hemorrhagic bullous at PHS remains unclear.
Studies Kobayashi et al. increase in matrix metalloproteinase-9 (MMP-9, gelatinase B) in liquid bullaewhich is examined by zymography
MMP-9 (secreted by PMN in dermoepidermal junction) migrate from intensive vasculitis lesions and form bullae by degrading basal membrane components such as collagen type VII
Histopathology
Typical picture of a vasculitis leucocytoclastic vasculitis with or without fibrinoid necrosis, until perivascular lymphocytes infiltrates with extravasation of erythrocytes
IgA deposits in the walls of blood vessels specific and sensitive markers of PHS
Figure 2: A. H & E staining of skin biopsy showed leukocytoclastic vasculitis with neutrophil infiltration B. Direct Imunofloresensi of
skin biopsies showed IgA deposition in blood vessels
Clinical Manifestations
Skin: palpable purpura, found on the skin buttocks and lower extremities, but can also be on the upper extremities, face and body.
Gastrointestinal tract: abdominal pain, vomiting, colicky abdominal bleeding, gastrointestinal bleeding, intestinal perforation and intussusception ileoileal or ileokolonal
Clinical Manifestations
Clinical Manifestations
Clinical Manifestations
Uncommon form of the lesions in HSP are vesicles containing blood that can develop into bullae containing blood (hemorrhagic)
Only occurs <2% of cases of hemorrhagic bullae in children
Clinical Manifestations
Clinical Manifestations
Joints: can not walk, arthralgia, joint swelling, redness and arthritis.
Kidney: hematuria, proteinuria, hypertension and renal failure.
Supportive Findings
There are no specific laboratory tests for HSP
Routine bloodUrinalysisRenal functionElectroliteIgA level in serumSkin and renal biopsy
Diagnostic
Based on specific clinical symptoms, laboratory tests, and histopathological.
There are two classification systems for HSP American College of Rheumatology (ACR) and the Chapel Hill Consensus Group
Henoch-Schonlein Purpura from ACR
Criteria Definitions
Purpura non trombositopenia (Palpable
purpura)
Hemorrhagic skin lesions that can be
touched, there is elevation of the skin, and
not associated with thrombocytopenia
Onset of age ≤ 20 y.o Onset of age ≤ 20 y.o
Abdominal symptomps (Bowel angina) Diffuse abdominal pain, become heavy
after a meal, or a diagnosis of bowel
ischemia, usually including bloody stool
Granulocytes in vessel walls in biopsy Showed histologic changes of granulocytes
in the walls of arterioles or venules
44
Management
Supportive and symptomatic, including maintenance of hydration, nutrition, electrolyte balance and relieve pain with analgesics.
Self-limiting disease within a few days-weeks.
Corticosteroids for severe symptoms, arthritis, severe abdominal pain, gastrointestinal bleeding, edema and persistent nephrotic syndrome.
Management
Management for hemorrhagic bullae on HSP can not be explained with certainty until today.
In general HSP with hemorrhagic bullae still be managed as a case of PHS in general.
The difference is its symptomatic treatment compress with 0.9% NaCl three times a day, topical antibiotics such as gentamicin or fusidic acid ointment
Case Analysis
Case
• Age: 8 years 11 months• Boy
Literature
47
• PHS occurs at the age of 6 months to adults, 50% of cases occur in children less than 5 years, 75% at age below 10 years
• Male: Female = 1.5-2: 1
Case Analysis
Case
• Palpable skin rash• Abdominal pain• Joint pain• Bloody stools
Literature
• Palpable purpura non trombocytopenia
• Bowel Angina (Diffuse abdominal pain, become heavy after a meal, or a diagnosis of bowel ischemia, usually including bloody stool)
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Case Analysis
Case
• Lab findings:• No trombocytopenia• Leucocytosis• Prolonged of ESR• Proteinuria• Occult blood (+)
Literature
• Lab findings:• No trombocytopenia• Leucocytosis• Prolonged of ESR and
CRP• Proteinuria• Hematuria• Decreased of renal
function in severe complications of renal.
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Case Analysis
Case
• Bed rest• Metil prednisolon 1-2
mg/kgBW/day full dose within 2 weeks
• Gentamicin cream• Monitoring of blood
pressure, urinalysis, and renal function
trum
Literature
• Bed rest• NSAID for arthritis and fever• Kortikosteroid for severe
symptoms• Severe Complications in
renal corticosteroid and immunosuppresants
• For bullae hemorrhagic lesions: compress with normal saline, topical antibiotic
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