Henoch-Schonlein purpura (HSP) is a vasculitis of unknown etiology that is characterized by inflammation of small blood vessels. It is the most common systemic vasculitis in childhood, occurring primarily in children ages 3-15. The hallmark of HSP is a palpable purpuric rash caused by small vessel inflammation in the skin. HSP is typically self-limiting and resolves within 3-4 weeks, though rash symptoms may persist for up to a year. Treatment focuses on supportive care, with corticosteroids sometimes used for gastrointestinal or renal involvement. Most children recover fully, though renal involvement carries a risk of long-term complications like hypertension if not properly

1. HSP
HENOCH SHOENLEIN
PURPURA
PRESENTED BY:
KOOROSH TAYARANI

2. ETIOLOGY
• HENOCH-SCHÖNLEIN PURPURA (HSP) IS A VASCULITIS OF UNKNOWN ETIOLOGY
CHARACTERIZED BY INFLAMMATION OF SMALL BLOOD VESSELS WITH
LEUKOCYTIC INFILTRATION OF TISSUE, HEMORRHAGE, AND ISCHEMIA. THE
IMMUNE COMPLEXES ASSOCIATED WITH HSP ARE PREDOMINANTLY COMPOSED
OF IMMUNOGLOBULIN A (IGA), SUGGESTING A HYPERSENSITIVITY PROCESS.

3. EPIDEMIOLOGY
• HSP IS THE MOST COMMON SYSTEMIC VASCULITIS OF CHILDHOOD AND CAUSE
OF NONTHROMBOCYTOPENIC PURPURA, WITH AN INCIDENCE OF 13 PER
100,000 CHILDREN. IT OCCURS PRIMARILY IN CHILDREN 3-15 YEARS OF AGE,
ALTHOUGH IT HAS BEEN DESCRIBED IN ADULTS. HSP IS SLIGHTLY MORE
COMMON IN BOYS THAN GIRLS AND OCCURS MORE FREQUENTLY IN THE WINTER
THAN IN THE SUMMER MONTHS.

4. CLINICAL MANIFESTATIONS
• RED URINE AND HEMATURIA
• PROTEINURIA
• SCROTAL PAIN
• FEVER AND RASH
• PETECHIAE/PURPURA

5. CLINICAL MANIFESTATIONS
• HSP IS CHARACTERIZED BY RASH, ARTHRITIS, AND LESS FREQUENTLY
GASTROINTESTINAL OR RENAL VASCULITIS. THE HALLMARK OF HSP IS PALPABLE
PURPURA , CAUSED BY SMALL VESSEL INFLAMMATION IN THE SKIN, LEADING TO
EXTRAVASATION OF BLOOD INTO THE SURROUNDING TISSUES, FREQUENTLY WITH IGA
DEPOSITION.
• BELOW THE WAIST, ON THE BUTTOCKS, AND LOWER EXTREMITIES RASHES
• URTICARIAL – PURPURA – ECCHYMOSIS-
• EDEMA, PARTICULARLY OF THE CALVES AND DORSUM OF THE FEET, SCALP, AND
SCROTUM OR LABIA
• ARTHRITIS OCCURS IN 80% OF PATIENTS WITH HSP AND IS MOST COMMON IN THE

6. CLINICAL MANIFESTATIONS
• GASTROINTESTINAL: MILD TO MODERATE CRAMPY ABDOMINAL PAIN, THOUGHT TO
BE DUE TO SMALL VESSEL INVOLVEMENT OF THE GASTROINTESTINAL TRACT LEADING
TO ISCHEMIA.
• LESS COMMONLY:SIGNIFICANT ABDOMINAL DISTENTION, BLOODY DIARRHEA,
INTUSSUSCEPTION, OR ABDOMINAL PERFORATION.
• RENAL INVOLVEMENT:ONE THIRD OF CHILDREN WITH HSP DEVELOP RENAL
INVOLVEMENT, WHICH CAN BE ACUTE OR CHRONIC. MILD IN MOST CASES.
GLOMERULONEPHRITIS-HEMANTURIA – HTN – ACUTE RENAL FAILURE.
• HSP OCCASIONALLY IS ASSOCIATED WITH:
• ENCEPHALOPATHY,
• PANCREATITIS,
• ORCHITIS.

7. LABORATORY AND IMAGING STUDIES
• ESR-CRP-WBC ARE ELEVATED IN HSP.
• NONTHROMBOCYTOPENIC PURPURA. NORMAL OR EVEN HIGH PLATELETS.
• HEMATURIA IN URINE ANALYSIS
• SERUM BUN AND CREATBUNININE SHOULD BE OBTAINED TO EVALUATE RENAL
FUNCTION.
• MAYBE BLOOD IN STOOL TESTS SUGGESTING OF UNDERLYING ISCHEMIA
• ANY QUESTION OF GUT PERFORATION REQUIRES RADIOLOGICAL INVESTIGATION.

8. DIAGNOSIS AND DIFFRENTIAL DIAGNOSIS
• THE DIAGNOSIS OF HSP IS BASED ON
THE PRESENCE OF TWO OF FOUR
CRITERIA ( TABLE 87.1 ), WHICH
PROVIDES 87.1% SENSITIVITY AND
87.7% SPECIFICITY FOR THE DISEASE.
THE DIFFERENTIAL DIAGNOSIS
INCLUDES OTHER SYSTEMIC
VASCULITIDES AND DISEASES
ASSOCIATED WITH
THROMBOCYTOPENIC PURPURA,
SUCH AS IDIOPATHIC
THROMBOCYTOPENIC PURPURA AND
LEUKEMIA.

10. TREATMENT
• THERAPY FOR HSP IS SUPPORTIVE. A SHORT-TERM COURSE OF NSAID DRUGS CAN
BE ADMINISTERED FOR THE ACUTE ARTHRITIS.
• SYSTEMIC CORTICOSTEROIDS USUALLY ARE RESERVED FOR CHILDREN WITH
GASTROINTESTINAL DISEASE AND PROVIDE SIGNIFICANT RELIEF OF ABDOMINAL
PAIN. A TYPICAL DOSING REGIMEN IS PREDNISONE, 1 MG/KG/DAY FOR 1-2 WEEKS,
FOLLOWED BY A TAPER SCHEDULE.
• RECURRENCE OF ABDOMINAL PAIN AS CORTICOSTEROIDS ARE WEANED MAY
NECESSITATE A LONGER COURSE OF TREATMENT.
• ACUTE NEPHRITIS TYPICALLY IS TREATED WITH CORTICOSTEROIDS BUT MAY
REQUIRE MORE AGGRESSIVE IMMUNOSUPPRESSIVE THERAPY.

11. COMPLICATIONS
• MOST CASES OF HSP ARE MONOPHASIC, LASTING 3-4 WEEKS AND RESOLVING
COMPLETELY.
• THE RASH CAN WAX AND WANE, HOWEVER, FOR 1 YEAR AFTER THE INITIAL EPISODE
OF HSP. PARENTS SHOULD BE WARNED REGARDING POSSIBLE RECURRENCES.
• THE ARTHRITIS OF HSP DOES NOT LEAVE ANY PERMANENT JOINT DAMAGE; IT DOES
NOT TYPICALLY RECUR.
• GASTROINTESTINAL INVOLVEMENT CAN LEAD TO TEMPORARY ABNORMAL
PERISTALSIS THAT POSES A RISK OF INTUSSUSCEPTION, WHICH MAY BE FOLLOWED
BY COMPLETE OBSTRUCTION OR INFARCTION WITH BOWEL PERFORATION. ANY
CHILD WITH A RECENT HISTORY OF HSP WHO PRESENTS WITH ACUTE ABDOMINAL
PAIN, OBSTIPATION, OR DIARRHEA SHOULD BE EVALUATED FOR INTUSSUSCEPTION.
• RENAL INVOLVEMENT RARELY MAY LEAD TO RENAL FAILURE.

12. PROGNOSIS
• THE PROGNOSIS OF HSP IS EXCELLENT. MOST CHILDREN HAVE COMPLETE
RESOLUTION OF THE ILLNESS WITHOUT ANY SIGNIFICANT SEQUELAE.
• HSP PATIENTS WITH RENAL DISEASE (ELEVATED BLOOD UREA NITROGEN,
PERSISTENT HIGH-GRADE PROTEINURIA) ARE AT HIGHEST RISK FOR LONG-
TERM COMPLICATIONS SUCH AS HYPERTENSION OR RENAL INSUFFICIENCY,
PARTICULARLY IF THE INITIAL COURSE WAS MARKED BY SIGNIFICANT
NEPHRITIS.THERE IS A LONG-TERM RISK OF PROGRESSION TO END-STAGE
RENAL DISEASE IN LESS THAN 1% OF CHILDREN WITH HSP
• THE RARE PATIENTS WHO DEVELOP END-STAGE RENAL DISEASE MAY REQUIRE
RENAL TRANSPLANTATION. HSP MAY RECUR IN THE TRANSPLANTED KIDNEY.

13. THANK YOU
.REFRENCES:
1.NELSON ESSENTIAL OF PEDIATRICS - EIGHTH EDITION
2.WWW.MEDICOWESOME.COM
3. WWW.PINTEREST. COM