Case 21: 6yo - Henoch Scholein purpura

Question 1

diffdx for bruising and leg pain

• cause:
• presentation:
• history:
• bruising v. not bruising

Answer 1

BRUISING
COAGULATION D/O
- cause: bleeding d/o (hemophilias = hemarthroses), (vWF = easy bruising after minor trauma)
- presentation: easy bruising in deep tissues, hemarthrosis, petechiae, superficial bruising
- history: FHx, PMH of bleeding after trauma, immunizations, circumcision, dental procedures

HENOCH-SCHONLEIN PURPURA (HSP)

• cause: self-limited, IgA-mediated, small vessel vasculitis; post-URI
• presentation: skin, GI tract, joints, kidneys; bruising & arthritis

IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)

• cause: post-URI –> binding of anti-plt Ab to plt surface –> removal and destruction of plts in spleen andliver
• presentation: asymptomatic petechiae

LEUKEMIA

• cause: infiltration of bone marrow –> thrombocytopenia
• presentation: constitutional (fever, malaise, weight loss); BONE PAIN; petechiae

VIRAL INFECTION (viral exanthem)

• cause: enteroviruses
• presentation: low-grade fever; petechiae, +/- constitutional sxs
• history: +/- prior coughing and/or vomiting (esp. w/ petechiae above nipple line)

NOT BRUISING
REYE SYNDROME
- cause: Influenza + aspirin exposure
- presentation: + hepatomegaly, fever, rash, vomiting + encephalitis, increased ICP, cerebral edema

BACTERIAL ENDOCARDITIS

• cause: Step pyogenes
• presentation: low-grade fever, fatigue, weight loss, petechiae (NOT bruising)
• history: +/- prior hx of Step pyogenes pharyngitis

MENINGOCOCCAL SEPTICEMIA
- cause: Coxsackie, Staph Group B (young kid);
H. flu (unimmunized); Listeria (infant)
- presentation: petechiae and purpura (NOT bruising), at which point kid is TOXIC

ROCKY MOUNTAIN SPOTTED FEVER

• cause: R. Ricketseii
• presentation: FEVER, petechiae, extremities –> trunk

SLE

• cause: autoimmune
• presentation: variable rash; constitutional (fever, malaise)
• history: older children, girls (non-Caucasian)

Question 2

mechanisms of petechiae and purpura

Answer 2

• trauma
• platelet deficiency or dysfunction (ITP, BM infiltration/suppression, malignancy)
• coagulation abnormalities (hereditary/acquired clotting disorders)
• vascular fragility (immune-mediated vasculitis)
• infections causing coagulation abnormalities
• vascular fragility
• platelet consumption

Question 3

evaluationfo skin lesion

Answer 3

• type (shape, size, consistence, color, secondary features)
• arrangement (symmetric, scattered, clustered linear, confluent, discrete)
• location (scalp, trunk, extremities, +/- palms, soles)
• distribution (flexural, sun-exposed, dependent areas)
• change over time (to progressive distribution, progressive type/secondary)

Question 4

Hepatomegaly

• pathophysiology:
• causes:
• presentation:
• ?normal in infants:

Answer 4

• pathophysiology:
• causes: pulmonary disease (–> downward displacement), inflammation (- viral hepatitis), infiltration (-leukemia/lymphoma), accumulated storage products (-glycogen-storage dz), congestion (-CHF), obstruction (-biliary atresia)
• presentation: lower dge of liver below costal margin
• normal liver edge palpable :
  3. 5cm (infants); 1-2cm (older kids)

Question 5

Henoch Scholein purpura (HSP)

• epidemiology:
• pathophysiology:
• causes:
• presentation:
• location
• complications:
• tx and follow up:
• prognosis:

Answer 5

• epidemiology: most common vasculitis in kids (50%)
• pathophysiology: self-limited, IgA-mediated small vessel vasculitis
• causes: post-URI = auto-immune
• presentation: non-thrombocytopenic NON-BLANCHING purpura, renal involvement (hematuria, esp in >2yo); arthritis/arthralgia (knees and ankles); colicky abd pain (+/- intussusception intestinal bleeding)
  ==> no joint effusion
  ==> no hx of easy bruising
• location: over legs, buttocks (dependent areas); symmetrical. non-confluent
• complications: hospitalization for management of severe abd pain, GI bleeding, intussusception, renal involvement), chronic renal failure; ESRD
• tx: early corticosteroids (reduced GI problems - but does NOT prevent renal); acetaminophen for arthralgia
• f/up q1w for 3w (then q1mo until recover): repeat UA, BP –> monitor kidney inflammation
• hospitalization for: sudden/severe stomach pain +/- vomiting and dehydration, hematochezia, generalized edema
• prognosis:self-limiting (lasts for 1mo), even without treatment; 30% recurrence after weeks / months (+ more likely renal involvement)

Question 6

HSP

difference in kids <2yo and >2yo

Answer 6

> 2yo = more likely to have renal involvement

Question 7

MOST COMMON CAUSE OF ISOLATED THROMBOCYTOPENIA IN OTHERWISE HEATLHY CHILDREN

Answer 7

IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)

Question 8

Intussusception:

• epidemiology:
• pathophysiology:
• presentation
• • findings on AXR:

Answer 8

• epidemiology: 0.1-0.4%; mostly in kids <2yo, boys > girls
• causes = hypertrophied intestinal lymphoid tissue (concurrent viral infection); pathological leading point (Meckel’s)
• pathophysiology: proximal segment of bowel invaginates into distal segment [esp ileocecal] ==> entrapped mesentery –> vascular compression, ischemia
• presentation = paroxysms of severe abd pain + inconsolable crying; “sausage-shaped” mass in R abd; LATER “currant jelly” stool with blood and mucus +/- non/bilious vomiting, lethargy, toxic
• findings on AXR: Central ring of hypoattenuation corresponds to mesenteric fat in the intussusceptum
• dx and tx = 1) US, 2) air/barium enema ==> reduced by air/hydrostatic pressure

OR surgical reduction

Question 9

most common form of bowel obstruction in children between 6mo-6y

Answer 9

intussusception:

Question 10

how does intussusception in HSP differ from idiopathic?

Answer 10

• usually ileal-ieal
• begins at points of intestinal edema/submucosal hemorrhage

b/c of location, cannot be reduced by air enema

dx = US
tx = surgical reduction

Question 11

determine which FINDINGS are urgent v. emergent and why

A		Fever	
B		Altered mental status (lethargy, agitation, confusion, poor eye contact or lack of age-appropriate response to parents or medical providers)	
C		Mottled skin	
D		Respiratory distress	
E		Respiratory depression	
F		Cyanosis	
G		Pallor	
H		Obvious pain

Answer 11

EMERGENT –> early/impending respiratory / circulatory failure

• altered mental status == inadequate cerebral perfusion –> can compromise ABCs
• respiratory distress (tachypnea, grunting, increased WOB); respiratory depression (slow/shallow breathing) == impaired oxygenation, circulation
• mottled skin / cyanosis == inadequate perfusion and O2 delivery to tissues

URGENT

• fever (+ bruising === concerns of infection)
• pallor = anemia
• pain

Question 12

patient centered medicine with Stewart model + Kleiman questions

Answer 12

• patient’s ideas about what is wrong
• impact of illness on functioning
• elicit of patient’s feelings (fears)
• patient’s expectations and what shoudl be done

Question 13

examination of lymph nodes in kids (what to look for)

Answer 13

SIZE, LOCATION = >2cm, outside of the cervical, axillary, inguinal regions
- supraclavicular ==> lymphoma

DISTRIBUTION
- infection == enlargement where it drains
- diffuse adenopathy == generalized infection, malignancy, storage diseases, and
chronic inflammatory disease

TEXTURE
- tenderness, warmth, fluctuance, overlying erythema or edema == lymphadenitis or local infection

MOBILITY
- hard, rubbery, matted togehter, affexed to skin / soft tissue == malignancy

Question 14

causes of splenomegaly

Answer 14

• infection = EBV, CMV, bacterial sepsis, endocarditis
• hemolysis = sickle cell dz
• cancer = leukemia, lymphoma
• storage dz = Gaucher
• inflammatory = SLE, JIA
• congestion= complication of portal HTN

Question 15

MOST common cause of splenomegaly in children?

Answer 15

infection

Question 16

normal palpation of spleen in children

Answer 16

<2cm in 30% neonates, 10% normal children, and 2% healthy adolescents

if >2cm in ANYONE == abnormal

Question 17

rash distribution in HSP

Answer 17

LOWER EXTREMITIES (dependent areas)
== vasculitis, arthritis, arthralgia

NO SPLENOMEGALY

Question 18

rash distribution in ITP

Answer 18

diffuse petechiae
+/- fever

NO SPLENOMEGALY

Question 19

rash distribution in leukemia

Answer 19

joint pain (d/t BM expansion)

+ SPLENOMEGALY, LYMPHADENOPATHY

Question 20

which of the following are associated with splenomegaly?
A. HSP
B. ITP
C. leukemia

how are these three things related?

Answer 20

leukemia ONLY

HSP is associated with a lot, but NOT SPLENOMEGALY

ITP = associated with splenectomy (NOT SPLENOMEGALY) == In people with ITP the immune system treats platelets as foreign and destroys them. The spleen is responsible for removing these damaged platelets and therefore removal of the spleen can help to keep more platelets circulating in the body.

1) palpable purpura == HSP
2) palpable purpura + low Plts == ITP
3) palpable purpura + low Plts + splenomegaly/lymphadenopathy == LEUKEMIA

Question 21

HSP evaluation

Answer 21

• CBC (esp Plt count) == HSP [nml plt] ITP, or leukemia [low plts]
• UA == renal involvement [hematuria, proteinuria]
• BUN, CR == extent of renal disease

Question 22

non-thrombocytopenic purpura

diffdx

Answer 22

HSP

Question 23

thrombocytopenic purpura

diffdx

Answer 23

ITP

Leukemia

Question 24

bruise patterns concerning for intentional injury

Answer 24

• over back, buttocks, face, ears

==> shape of whatever was used to inflict

• at various stages of healing
• inconsistent with hx / devleopmental abilities

Question 25

accidental bruise patterns

Answer 25

• over bony prominences = shins, elbows, forehead

Question 26

in an otherwise healthy 6yo with petechiae and/or purpura, which of the following are the top 5 diagnoses?

A 	Systemic lupus erythematosus
B 	Coagulation disorder
C 	Drug reaction
D 	Henoch-Schönlein purpura
E 	Idiopathic thrombocytopenic purpura
F 	Leukemia
G 	Meningococcal septicemia
H 	Rocky Mountain Spotted Fever
I 	Bacterial endocarditis
J 	Viral infection

Answer 26

coagulation d/o
HSP
ITP
leukemia
viral infection

the others would require other sxs, hx of taking meds, or the kid looking way more toxic

Question 27

Causes of arthritis to consider in a school-age child

Answer 27

Trauma
Septic arthritis
Transient synovitis
Reactive arthritis (e.g., post-streptococcal arthritis)
Lyme disease
Rheumatic fever
Juvenile idiopathic arthritis
Systemic lupus erythematosus
Henoch-Schönlein Purpura

Question 28

treatment for ITP

Answer 28

steroids
plt transfusion for <10-20K
IVIg

Question 29

A previously healthy 4-year-old girl is brought to her pediatrician because her parents have noticed that she has been less active than usual for the past three weeks. Her father explains that it is difficult to get his daughter out of bed in the mornings and that she no longer plays outside with her older brother. Physical examination is notable for a temperature of 38.4 C, heart rate of 125 bpm, pallor, truncal bruising, and diffuse lymphadenopathy. The remainder of the exam, including a thorough neurologic assessment, is unremarkable. Which of the following is the most likely diagnosis?

A		Aseptic meningitis	
B		Kawasaki disease	
C		Non-accidental trauma	
D		Acute lymphoblastic leukemia	
E		Mononucleosis

Answer 29

D

fever, tachycardia
pallor
bruising
DIFFUSE lymphadenopathy = not Kawasaki, not mono

the child presents with evidence of anemia (fatigue, tachycardia, pallor) and thrombocytopenia (unexplained bruising). Failure of two or more hematologic cell lines should always raise suspicion for malignant invasion of the marrow. Furthermore, the child’s chief complaint, fatigue, is the most common presenting symptom of acute leukemia. Finally, the incidence of ALL peaks at age 4 years.

Question 30

Steven, a 5-year-old boy with no significant past medical history, was in his usual state of health until last night when he developed abdominal pain. This morning his mother noticed a red and blotchy rash on his buttocks and lower extremities and his abdominal pain has worsened. Otherwise, he has no other symptoms and except for an upper respiratory tract infection last week, he has been in good health recently. On exam, the presence of palpable purpura and petechiae over the buttocks is confirmed. Laboratory studies are normal and, after a clinical diagnosis is made, he is discharged home the same day and given instructions to return for follow-up. Which of the following is important to measure at the first follow-up visit?

A		BP and urinalysis	
B		Platelet count	
C		PT/PTT	
D		White blood cells and hemoglobin	
E		Signs of intracranial hemorrhage

Answer 30

A

prior URI

abd pain
rash on dependent areas

== HSP
f/up UA and BP for renal problems.

This patient likely has Henoch-Schonlein Purpura (HSP). The exact etiology of HSP is unknown, but it is believed to involve an IgA-mediated immune response to infection or other triggers. The incidence is 10 cases per 100,000 children with a peak at ages 4 to 6 years (range of 2 to 17 years). HSP is characterized by a rash consisting of petechiae and palpable purpura. Other findings include a colicky diffuse or periumbilical abdominal pain, arthritis or arthralgia, and renal disease. Given the incidence of renal disease, it is important to check the urine for signs of hematuria or proteinuria; sudden changes in blood pressure can potentially suggest a change in renal function. With abnormal findings, serum BUN and creatinine must be checked.

Question 31

A 5-year old previously healthy boy is brought to his pediatrician with complaints of intermittent abdominal pain, right ankle pain, and a purpuric rash over his buttocks and lower extremities. His mom says she thinks he may recently have recovered from an upper respiratory infection. Which of the following statements is true?

Single Choice Answer:
Please select one answer.
A CBC would likely reveal thrombocytopenia
B This disease is classified as a small vessel vasculitis
C Urinalysis is not warranted for this patient’s work-up
D Treatment options include IVIG
E This disease is equally common in girls and boys

Answer 31

B

abd pain
arthralgia
purpuric rash on dependent areas == small vessel
post-URI
more likely in boys

== HSP

HSP is classified as a small vessel vasculitis. The exact mechanism of HSP is unknown; however, it is thought to be an IgA-mediated immune response affecting small vessels (skin, GI tract, joints, kidneys). Approximately 50% of cases follow viral or bacterial URIs. Biopsy of affected organs shows leukocytoclastic vasculitis with IgA deposition.

Question 32

Alex is a 6-year-old boy who presents to the clinic with a chief complaint of acute onset of bruising. He is afebrile, and his mother reports that he recently had a URI. He was born at full-term and has never been hospitalized. He was circumcised at birth with no problems with bleeding. No one in his family has any chronic medical problems. There have been no serious childhood illnesses or deaths. No one has a history of easy bruising or bleeding. On exam you find that he has a purpuric rash on his buttocks and legs. His urinalysis reveals 15 to 20 RBCs/hpf. Which of the following additional findings would NOT be consistent with the likely diagnosis?

 Single Choice Answer:
Please select one answer.  
A		Elevated serum IgA	
B		Blood in the stool	
C		Colicky abdominal pain	
D		Pain in his knees and ankles	
E		Low platelets

Answer 32

E

bruising
afebrile
post-URI
no hx easy bleeding
hematuria

• elevated IgA = IgA vasculitis
• blood on stool == intussusception
• colicky abd pain == HSP, +/- intussusception
• arthralgia

Thrombocytopenia is not characteristic of HSP, but is commonly seen in idiopathic thrombocytopenic purpura (ITP). Decreased platelets are often the delineating finding between HSP and ITP.

HSP is an IgA-mediated vasculitis involving the skin, joints, GI tract, and kidneys. Serum IgA may be elevated, occurring in 50% of patients.

Question 33

A mother brings her 8-year-old son to his primary care physician for pain in his knees and ankles that have been present for the past three days. She also notes that he has had a rash since yesterday, but otherwise feels well. The patient has no chronic illnesses, but he was brought in three weeks ago for an upper respiratory infection. Exam is significant for pain elicited on passive movement of the ankles and knees. Additionally, the patient is found to have an erythematous, slightly raised, non-blanching, maculopapular rash over the legs, buttocks, and posterior portion of the elbows. CBC shows WBC 8.9, Hgb 12.5, Hct 36.1, and Plt 327. Urinalysis is unremarkable. Skin biopsy shows leukocytoclastic vasculitis with IgA deposition. Which of the following is the best next step in management?

 Single Choice Answer:
Please select one answer.  
A		Observation	
B		Corticosteroids	
C		Intravenous immunoglobulin (IVIG)	
D		Intravenous hydration	
E		Platelet transfusion

Answer 33

A

arthralgia
rash = palpable purpura + non-blanching in dependnent areas
post-URI
non-thrombocytopenic

igA deposition with vasculitis

no abd pain

The joint pain, purpuric rash, and IgA deposition on skin biopsy support the diagnosis of HSP. Most cases of HSP resolve within approximately one month and do not require treatment. However, symptomatic treatment for joint pain, initially with NSAIDs, may be indicated.