Pediatrics 21: 6-year-old male with bruising

User: Maryam Fadah

Email: 201315184@uaeu.ac.ae
Date: March 8, 2020 8:38PM

Learning Objectives

Perform an age-appropriate history exam for a school-aged child presenting with purpura/petechiae
Generate an age appropriate differential diagnosis and initial diagnostic and therapeutic plan for purpura/petechiae in a school-
aged child
Describe the epidemiology, clinical, laboratory, and radiographic findings of important causes of purpura/petechiae
List clinical findings that suggest that a patient requires urgent stabilization
Describe an approach to eliciting the patient's perspective on illness
List features that are important to consider in evaluating skin lesions
Compare and contrast features of accidental vs. non-accidental bruising.
Differentiate normal and abnormal findings on examination of the lymph nodes, liver, and spleen.
Summarize clinical features, lab findings, natural history, and complications of Henoch-Schönlein purpura.
Outline typical clinical and laboratory features of idiopathic thrombocytopenic purpura (ITP), acute lymphocytic leukemia (ALL),
and intussusception.

Knowledge

Mechanisms of Petechiae and Purpura

One approach to thinking about a clinical problem is to consider possible underlying mechanisms. Petechiae and purpura can be
caused by:

Trauma
Platelet deficiency or dysfunction (e.g. immune-mediated thrombocytopenia, bone marrow infiltration or suppression,
malignancy)
Coagulation abnormalities (e.g. hereditary or acquired clotting-factor deficiencies)
Vascular fragility (e.g., immune-mediated vasculitis)
Combinations of the above (e.g., infection causing coagulation abnormalities, vascular fragility, platelet consumption)

Differential diagnosis for bruising and leg pain

Diagnosis Comments

A coagulation disorder may present with petechiae or superficial bruising, but more often presents
with easy bruising in deep tissues or hemarthrosis.

Bleeding disorders (hemophilias, von Willebrand's disease) are characterized by easy bruising in
Coagulation disorder response to minor trauma. Spontaneous superficial bruising is less common.

Hemophilias may present with painful bleeding into joints (hemarthrosis).

Children with coagulation disorders often have a positive family history and/or personal history of
bleeding (e.g., after trauma, immunizations, circumcision, dental work).

HSP is a self-limited, IgA-mediated, small vessel vasculitis that typically involves the skin, GI tract,
joints, and kidneys.
Henoch-Schönlein
purpura (HSP) Often presents in an otherwise well appearing child with bruising and leg pain (due to arthritis).

In roughly 50% of cases, a URI precedes the diagnosis of HSP.

Idiopathic Often presents with asymptomatic petechiae.

thrombocytopenic
purpura (ITP) A nonspecific URI precedes ITP more than 50% of the time.

Usually presents with constitutional symptoms such as fever, malaise and weight loss.

Bone pain is also a common presentation of leukemia in children. The pain results from infiltration
Leukemia of the bone marrow by malignant cell.

© 2020 Aquifer 1/7

 Petechiae can be caused by thrombocytopenia due to bone marrow replacement by malignant cells.

Some viruses, such as enteroviruses, may present with a petechial rash.

Children usually have a low-grade fever.

Viral infection
Other constitutional complaints may be present or absent.

Prominent coughing and/or vomiting can also cause petechiae, generally above the nipple line.

Typical presenting complaints are fever, fatigue, and weight loss.

A petechial rash is commonly seen.

Bacterial endocarditis
Bruising is not characteristic.

Fever is usually present, but may be low-grade.

Children with meningococcal septiciemia may present with petechiae and purpura, which can be
confused with bruising.
Meningococcal
While the early stages of meningococcemia may have only mild symptoms, by the time the
septiciemia
hemorrhagic rash appears, patients are usually very ill appearing and require emergent care.

Fever is usually present.

Rocky Mountain The rash of RMSF is often petechial, and starts on the extremities before moving centrally.
spotted fever (RMSF) Fever is a hallmark of RMSF.

The rash of SLE can be variable.

Systemic lupus
More common in older children and in girls.
erythematosus (SLE)
SLE often presents with constitutional symptoms such as fever and malaise.

Evaluating a Skin Lesion

Features that are important to consider in establishing a differential diagnosis for skin lesions include:

Feature Examples

Shape (e.g., macules, plaques, papules, wheals, vesicles, pustules, nodules, cysts)

Size

Type Consistency (e.g., rubbery, fluctuant)

Color

Secondary features (e.g., scaling, crusting, lichenification, excoriation, hypopigmentation)

Symmetric

Scattered

Clustered
Arrangement
Linear

Confluent

Discrete

Scalp

Trunk
Location
Extremities

Sparing or including palms and soles

Flexural surfaces

Pattern of distribution Sun-exposed skin

© 2020 Aquifer 2/7

 Dependent areas

Spreading head to toe or peripheral to central

Progression over time
Changing from papules to vesicles to crusts

Hepatomegaly

Though a palpable liver edge may be normal or attributable to causes other than hepatomegaly (e.g., downward displacement of
the liver with air-trapping in pulmonary disease), a palpable liver edge should be further evaluated by percussion and
measurement of the full liver span.

Causes

Hepatomegaly may occur as a result of inflammation (e.g., viral hepatitis), infiltration (e.g., leukemia/lymphoma), accumulation of
storage products (e.g., glycogen-storage disease), congestion (e.g., congestive heart failure) or obstruction (e.g., biliary atresia).

Examination

Hepatomegaly usually causes the lower edge of the liver to extend beyond the right costal margin. Palpation of the lower edge of
the liver may be facilitated by:

Placing the child in a supine position with knees and neck flexed to relax the abdominal muscles,
Asking the child to breathe deeply while the examining hand is below the right costal margin, or
Palpating initially in the right-lower quadrant and gradually moving toward the costal margin.

Measurement

Liver size is determined by percussing to locate the upper edge of the liver, palpating to locate the lower edge, and measuring the
distance between the two in the midclavicular line.

In normal newborns, the liver edge may be palpable up to 3.5 cm below the costal margin. In older children, it may be palpable up
to 2 cm below the costal margin (though typically not more than 1 cm).

Splenomegaly

Causes

Infection (e.g. Epstein-Barr virus, cytomegalovirus, bacterial sepsis, endocarditis) is the most common cause of splenomegaly in
children. Other causes include hemolysis (sickle cell disease), malignancy (leukemia, lymphoma), storage diseases (e.g. Gaucher
disease), systemic inflammatory diseases (e.g. systemic lupus erythematosus, juvenile idiopathic arthritis), and congestion (a
complication of portal hypertension).

Examination

Examination of the spleen can be challenging. Studies show significant variability among examiners in detecting an enlarged
spleen. A few pointers:

As with the liver exam, it is helpful to have the patient lie supine and flex his or her legs to relax the abdominal muscles. In
this position, it may be possible to gently "bounce" an enlarged spleen, which is located somewhat posteriorly, forward using
a lifting maneuver along the left flank.
Alternatively, having the patient roll onto his or her right side may rotate the spleen anteriorly into a palpable position.
As in the liver exam, having the patient inhale deeply to lower the diaphragm, or palpating first in the pelvis then moving
superiorly may facilitate detection of an enlarged spleen.

Although reports vary, the spleen tip is palpable in up to one-third of neonates, approximately 10% of normal children, and 2% of
healthy adolescents. A spleen edge felt more than 2 cm below the left costal margin is considered abnormal.

Henoch-Schönlein Purpura (HSP)

HSP (also known as anaphylactoid purpura) is a self-limited, IgA-mediated, small vessel vasculitis that typically involves the skin,
GI tract, joints, and kidneys.

Epidemiology

HSP is the most commonly diagnosed form of vasculitis in children (about 50% of cases). Boys are more affected than girls.

Signs and Symptoms

The hallmark of HSP is non-thrombocytopenic purpura.

One-third of children with HSP have renal involvement, the most common manifestation of which is hematuria. Renal involvement
is less common in children under two years of age, occurring in about 25% in that age group.

Arthritis or arthralgia, mainly of the knees and ankles, is seen in about 75% of children with HSP.

Colicky abdominal pain is present in 65% of patients. Some children will develop abdominal pain before the onset of the rash.
About 50% of children with HSP may develop intestinal bleeding, with guaiac positive stool.

Two-thirds of patients report a recent upper respiratory tract infection.

© 2020 Aquifer 3/7

 Additional Evaluation

It is rare to require additional evaluation in patients with HSP. However, in some cases the presentation is atypical and
pediatricians may seek consultation from their dermatology colleagues to help confirm the diagnosis. If a biopsy is performed for
this reason, the typical finding is leukocytoclastic vasculitis with IgA deposition.

Complications

About 5% of children with HSP progress to chronic renal failure. Fewer than 1% will develop end-stage renal disease.

Although HSP is considered a benign childhood disease, it occasionally requires hospitalization for management of severe
abdominal pain, GI bleeding, intussusception, and renal involvement.

Treatment

Treatment with steroids remains controversial. However, a recent analysis suggests early corticosteroids in hospitalized children
with HSP may confer benefits in reducing gastrointestinal manifestations. Systematic reviews have not demonstrated a consistent
benefit for corticosteroids in preventing serious renal involvement. There is a need for a high-quality multicenter, randomized
controlled trial to help us understand the potential benefits of early corticosteroids for children HSP.

Idiopathic Thrombocytopenic Purpura (ITP)

ITP is caused by the binding of an antiplatelet antibody to the platelet surface, leading to removal and destruction of platelets in
the spleen and liver. It is the most common cause of isolated thrombocytopenia in otherwise healthy children.

ITP and HSP share many features. Here is a table comparing the two diagnoses.

Henoch-Schönlein Purpura: Talking to Parents

In HSP, for reasons that are not well understood, blood vessels in various parts of the body become inflamed or irritated. An auto-
immune response is a possibility (while the body's immune system is fighting a cold or other infection, it inadvertently attacks the
blood vessels at the same time).

Children with HSP often have joint pain and stomachache along with the rash.

The inflammation can also affect the kidneys, so that we see blood or protein in the urine.

Prognosis

HSP usually lasts a month or so and goes away on its own without any treatment.

Followup

Usually all that is required is to check repeat urine samples to monitor kidney inflammation and to follow blood pressure.

Instructions for Parents

Acetaminophen may be given for pain.

Watch for stomach pain or vomiting.

Reasons to seek urgent medical attention:

If the stomach pain is sudden or severe,

Blood in the stool,
Vomiting with inability to keep fluids down,
Child appears "puffy" in face, hands, or feet (a sign of renal malfunction), or
Any other symptoms or changes that worry the parent.

Parent handout about HSP and what to expect.

Intussusception

Intussusception is the most common form of bowel obstruction in children between 6 months and 6 years of age.

Overall incidence: 1-4 cases per 1,000 live births

Eighty percent of cases occur in children under 2
Boys are affected more often than girls

Pathophysiology

Intussusception occurs when a proximal segment of bowel invaginates or telescopes into the distal segment adjacent. This
invagination is often intermittent. The accompanying mesentery becomes entrapped, causing vascular compression and eventual
ischemia.

Most cases of intussusception do not involve a discrete, identifiable lead point. Hypertrophied intestinal lymphoid tissue (e.g., in
response to a concurrent viral infection) has been suggested as a possible trigger.

Occasionally, a pathological lead point (e.g., polyp, Meckel's diverticulum) starts the telescoping process. Intussusception in HSP
likely begins at points of intestinal edema or submucosal hemorrhage.

Idiopathic intussusception (in which there is no identifiable lead point) occurs in otherwise healthy infants and toddlers. The
majority of cases involve the ileocecal junction.

© 2020 Aquifer 4/7

 Intussusception in HSP is usually ileo-ileal.

Presentation

The classic presentation of intussusception includes:

Paroxysms of severe abdominal pain with inconsolable crying

Passage of "currant jelly" stool containing blood and mucus
Palpation of a "sausage-shaped" mass in the right abdomen

A minority of patients present with the classic triad of findings. Diagnosis requires a high index of suspicion. The intermittent
nature of intussusception makes diagnosis difficult. Additional signs and symptoms may include vomiting (bilious or non-bilious),
lethargy and a toxic appearance, with or without signs of pain.

Diagnosis and Treatment

The standard approach to diagnosis and treatment of idiopathic intussusception is to perform an air or barium enema.

Ultrasound has increasing utility in the initial evaluation of intussusception. However a negative ultrasound does not rule out the
diagnosis definitively.

Once the intussusception is visualized, the telescoped segment of bowel is reduced by air or hydrostatic pressure. Occasionally,
the procedure is unsuccessful and patients require surgical reduction.

Because intussusception occurring in HSP is usually ileo-ileal, not ileo-colic, it will not be reduced by air or barium enema.
Diagnosis requires abdominal ultrasound, and treatment is generally surgical.

Supine AP abdomen radiograph of a young boy (NOT with HSP) shows a RUQ mass (black arrows), representing an
intussusception. Central ring of hypoattenuation (arrowheads) corresponds to mesenteric fat in the intussusceptum.
There is no small bowel dilatation or radiographic evidence of small bowel obstruction.

During air enema reduction (same patient), the colon fills with air and appears relatively white on these serial
fluoroscopic images. The intussusceptum is seen as a gray intraluminal filling defect (I) that moves progressively
toward the ileocecal valve (icv). Rectal tube, arrow.

Final image of a successful air enema reduction (same patient) shows presence of air in multiple loops of small
intestine (arrows) and resolution of the previously demonstrated intracolonic filling defect.

HSP Recurrence

The recurrence rate for HSP is about 30%. Symptoms may return weeks to many months after the first episode.

Like the initial episode, a recurrence may start with abdominal pain, rather than rash, so it is important to keep HSP in mind if a
patient has significant abdominal pain in the future.

If a recurrence does occur, it is important to recheck the urine to evaluate for renal involvement.

Clinical Skills

Emergent vs. Urgent Findings

The findings selected are possible signs of early or impending respiratory or circulatory failure and require emergent attention:

Mental status changes in children may manifest as lethargy, agitation, confusion, poor eye
contact or lack of age-appropriate response to parents or medical providers.

Altered mental status A change in mental status may indicate inadequate cerebral perfusion.

Significant alteration in level of consciousness may compromise a child's airway, breathing, and/or
circulation.

Respiratory
Respiratory distress (tachypnea, grunting, increased work of breathing) or respiratory depression
distress/respiratory
(slow or shallow breathing) suggest impaired oxygenation and ventilation.
depression

Mottled skin/cyanosis These skin color changes are signs of inadequate perfusion and oxygen delivery to the tissues.

The remaining findings, though not necessarily life-threatening, should prompt quick notification of the preceptor:

Fever In the setting of bruising, fever raises concern for the possibility of a rapidly progressive, life-threatening infection.

Pallor Pallor suggests the presence of anemia.

© 2020 Aquifer 5/7

 Pain Pain should be treated as promptly as possible. If the pain seems severe, the preceptor should be notified
immediately so that evaluation for serious conditions can be expedited.

The Patient's Perspective

Deliberately exploring the patient's perspective with every encounter often yields crucial information to guide history-taking,
examination, lab testing, and advice to patients. Visits become more efficient and effective when providers make an effort to
understand patients' views.

The following table combines two frameworks for exploring the patient's perspective on illness.

Stewart: Patient-centered Medicine Kleinman: Explanatory Models

▪ What do you call the problem?

▪ What do you think has caused the problem?

Questions to elicit the patient's ideas about what is wrong
▪ Why do you think it started when it did?

▪ What do you think your illness does? How does it work?

▪ How severe is the illness? Will it have a short or long course?

Questions to understand the impact of illness on functioning
▪ What are the chief problems the illness has caused?

▪ What do you fear most (what worries you most) about the
Questions to elicit the patient's feelings, especially fears
illness?

▪ What kind of treatment do you think you/the patient should

Questions to understand the patient's expectations about what receive?
should be done ▪ What are the most important results you hope to receive from
this treatment

Examination of Lymph Nodes

Lymph nodes should be examined for size, location, distribution, texture, and mobility.

Size and location

In healthy children, small (< 2 cm) lymph nodes are often palpable in the cervical, axillary, and inguinal regions.
Lymph nodes > 2 cm (or nodes palpable in other areas) suggest the presence of disease.
Supraclavicular nodes are unlikely to be reactive, and are highly concerning for lymphoma.

Distribution

Localized enlargement usually occurs with infection in an area that drains to the affected node.
Diffuse adenopathy occurs with generalized infection, malignancy, storage diseases, and chronic inflammatory disease.

Texture

Normal lymph nodes are generally smooth, soft to mildly firm, non-tender, and mobile.
Tenderness, warmth, fluctuance, overlying erythema or edema suggest local infection or infection of the node itself
(lymphadenitis).

Mobility

Nodes that are hard, rubbery, matted together, or affixed to skin or soft tissue raise concern for malignancy.

Studies

Laboratory Evaluation of Suspected HSP

The platelet count is essential to determine whether this is non-thrombocytopenic (HSP) or thrombocytopenic
(ITP or leukemia) purpura.
Platelet count
The results of the hemoglobin and leukocyte count can provide helpful information when there is concern for
leukemia.

With HSP it is important to determine whether the patient has renal involvement.

© 2020 Aquifer 6/7

 Urinalysis A urinalysis will detect hematuria and/or proteinuria.

BUN and If either hematuria or proteinuria is present, a BUN and creatinine are indicated to determine the extent of renal
creatinine disease.

Clinical Reasoning

Findings in HSP, ITP, and Leukemia

Certain rash distributions suggest specific diagnoses.

The rash of HSP is somewhat unique in its tendency to involve primarily the lower extremities.

HSP often causes a periarticular vasculitis presenting as arthritis or arthralgia.

HSP
HSP is not associated with splenomegaly. A palpable spleen in a child with suspected HSP should prompt
evaluation for other causes.

ITP is not associated with splenomegaly. If discovered in a child with thrombocytopenia, splenomegaly should
ITP
prompt evaluation for other processes, including leukemia or other malignancy.

Bone pain secondary to bone marrow expansion may cause pain localized to the joints.
Leukemia
Splenomegaly and lymphadenopathy are common findings in leukemia. Read more.

References

Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-
Schönlein Purpura (HSP). Cochrane Database of Systematic Reviews 2009, Issue 3. Art. No.: CD005128.
DOI:10.1002/14651858.CD005128.pub2.

Kleinman A, Eisenberg L, Good B. Culture, Illness, and Care: Clinical lessons from anthropologic and cross-cultural research. Annals of
Internal Medicine 1978; 88: 251-58.

Sandborg C, Rheumatology. In Bernstein D, Shelov SP (eds), Pediatrics for Medical Students, Baltimore: Lippincott, Williams and
Wilkins; 2003:501-514.

Stewart M, Brown J, Weston W, McWhinney I, McWilliam C, Freeman T. Patient-Centered Medicine: Transforming the Clinical Method.
Sage, Thousand Oaks, 1995.

Weiss et al. Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schönlein purpura. Pediatrics (2010) vol.
126 (4) pp. 674-81.

Wolf AD, Lavine JE. Hepatomegaly in Neonates and Children. Pediatrics in Review , September 2000; 21(9): 303-310.

Wong DL. FACES pain rating scale from Hockenberry MJ: Wong's Essentials of Pediatric Nursing , 7th ed. St. Louis, MO: Mosby;
2005:1301. Copyrighted by Mosby, Inc. Reprinted by permission.

© 2020 Aquifer 7/7