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Benjamin Dowse, MD/PGY3

08/01/2014
3 year old previously healthy white female referred to the emergency
department from her PCP secondary to concern for a rash, which
began 4 days ago. She has also had intermittent fever since Saturday
(Tmax 39.1 per parents, was afebrile in clinic). Has had NB/NB emesis
today and yesterday.
ROS: No diarrhea, no headache, fever improves with Tylenol per
parents. Otherwise negative ROS.
HPI
Immunizations: UTD
NKDA
No routine home meds
Previously healthy, no chronic illness, hospitalizations or surgeries.
FHx: Mom with ankylosing spondylitis.
Past Medical History
VS: T 36.3, HR 108, R 24, Sat 98% RA, BP 90/60
General: Well developed 3 year old. Awake, alert, appropriate, non-toxic, no acute
distress. Cooperative, playful, talkative. No pain or discomfort.
HEENT: Normocephalic, atraumatic, PERRL, no conjunctival injection, TMs clear, nares
patent, pharynx, mouth, tongue all without lesions, exudate or erythema, neck with no
swollen or tender lymph nodes, supple with full range of motion.
Respiratory: Lungs CTAB, no increased WOB.
Cardiovascular: Normal S1/S2, without any murmur, gallop, click, or rub. 2+ pulses to
upper and lower extremities. Capillary refill less than 2 seconds.
GI: Abdomen soft, non-tender. Normal bowel sounds, no organomegaly.
Musculoskeletal: No pain in extremities, normal range of motion without pain to
passive or active movement. Mild pedal edema.
SKIN: Notable for purpuric type lesions. Predominantly to the lower extremities,
but do extend to upper torso, both the front and the back. Also appear on her face,
neck and arms. These lesions do not blanch. Small, approximately 1.5 cm at
largest. Non-painful.
Neurologic: Symmetric use of extremities, no evidence of weakness. Lower extremity
reflexes symmetric, toes downgoing, no clonus, cranial nerves normal.
Physical Exam
3 yo previously healthy girl with fever, nausea/vomiting and diffuse
petechial/pupuric lesions.
Differential Diagnosis
Infectious
Meningococcal/pneumococcal meningitis
Bacterial endocarditis
Viral hemorrhagic fevers
AIDS thrombocytopenia/HIV produced
Disseminated CMV
Ehrlichiosis
Measles (rubeola)
Rickettsial disease
Rocky mountain spotted fever
Babesiosis
Dengue Hemorrhagic fever
Colorado Tick Fever
Leptospirosis (Weils disease)
Rheum
ITP
HSP
Neoplastic
AML/ALL
Lymphoma
Congenital
Kasabach-Merritt syndrome
FEN
Scurvy (vit C deficiency)
Genetic
Von Willibrands disease
Absent radius/thrombocytopenia syndrome
Wiskott-Aldrich Syndrome
Heme
Disseminated Intravascular coagulopathy
Aplastic anemia
Renal
Hemolytic Uremic syndome
Other
Heat stroke
Drug induced thrombocytopenia
Radiation exposure
Differential Diagnosis
CBC:
WBC 10.3 (32% neut, 63% Lymph), HGB 12.4, Hct 35.3, PLTS 316
UA:
Normal color, appearance, glucose negative, hgb negative, protein trace, negative
nitrites and leuk esterase. Negative for bacteria, 3 WBC.
CMP:
Na 139, K 4.7, Cl 109, Bicarb 20, Glucose 73, BUN 13, Creatinine 0.35, Ca 9.5, Prot 6.5,
Albumin 3.5, Bili 0.3, Alk phos 237, ALT 10, AST 24
Labs
Most common form of systemic vasculitis in children.
Primarily between ages 3-15 years.
Highest incidence is 70 per 100,000 in children between four and six
years.
About half of cases are preceded by a known upper respiratory
infection.
Henoch Schonlein Purpura
(IgA Vasculitis)
Immune-mediated vasculitis associated with immunoglobulin A deposition.
Underlying cause remains unknown, although immunologic, genetic, and
environmental factors all seem to play a role.
Biopsies demonstrate involvement of small vessels (mostly post-capillary venules)

Pathophysiology
Immunofluorescence studies show IgA, C3, and fibrin deposition within vessel
walls. IgA, C3, fibrin, as well as IgG and IgM are deposited within the endothelial
and mesangial cells of the kidney.
Pathophysiology
Immunofluorescence microscopy showing mesangial
immunoglobulin A (IgA) deposits

Clinical Features
Presenting symptoms:
Purpura 74%
Arthritis 15%
Abdominal pain 12%

Classic Tetrad
Palpable purpura without thrombocytopenia and
coagulopathy (all patients) (?)
Often begins as urticarial wheals which
coalesce and evolve into palpable purpura,
mostly in dependent areas.
Arthritis/Arthralgia (50-75% of patients)
Usually transient or migratory, one to four joints,
more commonly lower extremity joints.
Abdominal pain (50% of patients)
Can develop intussusception, guaiac positive
stool, or just nausea. Endoscopy may
demonstrate purpuric lesions.
Renal disease (21-54% of patients)
Similar to IgA nephropathy. Watch for hematuria,
proteinuria. Occasionally can progress to serious
kidney disease.











This picture shows the classic skin manifestations of Henoch-Schnlein purpura (IgA
vasculitis), with clusters of typical ecchymoses, petechiae, and palpable lesions on the
legs in a typical distribution (gravity/pressure-dependent areas).

Skin manifestations of Henoch-Schnlein
purpura (IgA vasculitis)
Usually clinically diagnosed. If unusual, biopsy of an affected organ can be
confirmatory.


Diagnosis
Follow-up Renal involvement
90% with renal involvement manifest
within two months
96% within 6 months
Patients should be followed weekly
or biweekly for urinalysis and blood
pressure measurement for one to
two months after presentation
Then monthly for a year after initial
manifestation.
If persistent hypertension,
proteinuria, or renal insufficiency,
refer to nephrology.

Supportive care
Adequate rest, hydration,
symptomatic relief of pain
Hospitalization?
Unable to hydrate
Severe abdominal pain
Significant GI bleeding
Altered mental status
Renal insufficiency (elevated
creatinine), hypertension, and/or
nephrotic syndrome
Treatment
Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood. Incidence of Henoch-Schnlein purpura, Kawasaki disease, and rare vasculitides in
children of different ethnic origins. Lancet. 2002;360(9341):1197.
Rigante D, Castellazzi L, Bosco A, Esposito S. Is there a crossroad between infections, genetics, and Henoch-Schnlein purpura? Autoimmun
Rev. 2013 Aug;12(10):1016-21. Epub 2013 May 15.
Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M. Henoch Schonlein purpura in childhood: epidemiological and
clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005;35(3):143.
Trnka P. Henoch-Schnlein purpura in children. J Paediatr Child Health. 2013 Dec;49(12):995-1003. Epub 2013 Oct 18.
Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal
urinary findings: a systematic review. Arch Dis Child. 2005;90(9):916.
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References