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Case Conference. K. Myra Lalas PGY 3. CC: rash and joint pain. History of Present Illness. 2 days PTA, (+) itchy, reddish wheals on back then spread to torso, chest, and extremities Went to PMD and was prescribed Benadryl and Hydrocortisone, which gave some relief.

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Case conference

Case Conference

K. Myra Lalas

PGY 3



History of present illness

History of Present Illness

2 days PTA, (+) itchy, reddish wheals on back then spread to torso, chest, and extremities

Went to PMD and was prescribed Benadryl and Hydrocortisone, which gave some relief


On the day of admission, (+) swelling, redness, warmth, and pain of both knees ( R > L), ankles (R > L), L elbow

No morning stiffness, nails don’t turn funny colors when cold


Review of systems

Review of Systems pain of both knees ( R > L), ankles (R > L), L elbow

No fever

No cough or runny nose

No vomiting

No diarrhea

Good PO

No sick contacts

No recent travel

1st episode


Past medical history

Past Medical History pain of both knees ( R > L), ankles (R > L), L elbow

Staghorn calculus s/p removal of stones in the right kidney 3/2010


Family history

Family History pain of both knees ( R > L), ankles (R > L), L elbow

Osteoarthritis- grandfather

No lupus, no JIA, no scleroderma

No childhood leukemia


Social history
Social History pain of both knees ( R > L), ankles (R > L), L elbow

  • Lives with both parents

  • No pets or smokers at home


Physical exam

Physical Exam pain of both knees ( R > L), ankles (R > L), L elbow

VS WNL

Gen awake, alert, no acute distress

HEENT PERRL, oropharynx clear, no LAD, TM’s normal

Lungs clear to auscultation

Heart Normal S1/S2, no murmurs

Abdomen soft, nontender, no organomegaly


Ext L knee: (+) effusion, limited ROM secondary to pain pain of both knees ( R > L), ankles (R > L), L elbow

R knee: limited ROM secondary to pain

B/L ankles: erythema over lateral malleoli, no swelling, FROM

No nail changes

Skin multiple erythematous, pruritic papules on back, buttocks with some excoriations


What are your differentials
What are your differentials? pain of both knees ( R > L), ankles (R > L), L elbow


Differential diagnoses
Differential Diagnoses pain of both knees ( R > L), ankles (R > L), L elbow

Viral Exanthems

Urticarial Vasculitis

Meningococcemia

Reactive Arthritis

Other drug reactions

Erythema Multiforme

Steven Johnson's Sydrome

Kawasaki's disease


Serum sickness rash
Serum Sickness Rash pain of both knees ( R > L), ankles (R > L), L elbow


Sjs rash
SJS rash pain of both knees ( R > L), ankles (R > L), L elbow


Meningococcemia rash
 Meningococcemia Rash pain of both knees ( R > L), ankles (R > L), L elbow


Kawasaki
Kawasaki pain of both knees ( R > L), ankles (R > L), L elbow


Labs pain of both knees ( R > L), ankles (R > L), L elbow

Parvovirus B19 IgG and IgM (normal)

ASLO normal (25)

Streptozyme negative

C4 28  C3 148

Lyme Ab titer 0.2 (normal)

CBC WBC 13.9 Hgb 12 Hct 35.4 platelets 429 N 52 L 43

Blood Culture negative

ESR 40

CRP 3.5

ua normal

Urine culture negative


Labs pain of both knees ( R > L), ankles (R > L), L elbow

Na 136 K hemolyzed Cl 102 HCO3 20 BUN 9 Creatinine 0.6

glucose 117 Ca 9.8

albumin 4.1 TP 6.7

ALT hemolyzed AST 11

Alk phos 145

TB 0.2  DB 0


Serum sickness
Serum Sickness pain of both knees ( R > L), ankles (R > L), L elbow

First described by von Pirquet and Schick. They described an illness that developed in some patients after they were given horse serum antitoxin for diphtheria and Scarlet fever. The illness developed a few weeks after administration of horse serum antitoxin.

Cardinal features: rash, fever, and polyarthralgias or polyarthritis, which begin 1-2 weeks after exposure to the responsible agent. 


Pathophysiology
Pathophysiology pain of both knees ( R > L), ankles (R > L), L elbow

Type III Hypersensitivity Reaction

1. Immune complex formation

2. Complement activation

3. Complement- independent mechanisms

Immune complexes in tissues can react directly with Fc gamma receptors on neutrophils, mast cells, and phagocytes, leading to release of cytokines, histamine, and other inflammatory mediators even without complement.


Precipitin curve
Precipitin Curve pain of both knees ( R > L), ankles (R > L), L elbow


Serum like sickness
Serum-like sickness pain of both knees ( R > L), ankles (R > L), L elbow

May be caused by drugs, viral infections

Has different pathophysiology than serum sickness

Levels of circulating immune complexes and serum complement are often unaffected

Commonly implicated drugs: Cefaclor Penicillin (amoxicillin) Trimethoprim-sulfamethoxazole


Heterologous proteins causing serum sickness
Heterologous Proteins Causing Serum Sickness pain of both knees ( R > L), ankles (R > L), L elbow

Microbial antitoxins

  • Equine anti-diphtheria

  • Equine or ovine anti-rabies

  • Equine anti-botulinin toxin

    Venom anti-toxins

  • Equine, rabbit, ovine anti-snake venom (crotalidae (pit vipers, rattlesnakes) antivenin, micrurus (coral snake) antivenin)

  • Equine anti-spider venom (lactrodectus) antivenin


Heterologous proteins causing serum sickness1
Heterologous Proteins Causing Serum Sickness pain of both knees ( R > L), ankles (R > L), L elbow

Immunomodulators

  • Equine or rabbit antithymocyte globulin Murine antiCD3 (OKT3)

  • Rituximab (murine/human chimeric anti-CD20) Infliximab

  • (murine/human chimeric anti-TNF alpha) Alemtuzumab (humanized anti-CD52, Campath)

    Immunizations

  • Rabies antigens (human diploid cell rabies vaccine)


Cefaclor and bactrim
Cefaclor and Bactrim pain of both knees ( R > L), ankles (R > L), L elbow

Metabolites toxic to lymphocytes. The predisposing drug metabolism is genetically influenced.

Penicillin

May be caused by drug-specific immune complexes, not complexes with heterologous serum proteins.


Signs and symptoms
Signs and Symptoms pain of both knees ( R > L), ankles (R > L), L elbow

Arthralgias

Lymphadenopathy

Urticarial rash

Fever, when present, is typically low-grade.

Acute onset of joint pain, often leading to inability to walk


Labs pain of both knees ( R > L), ankles (R > L), L elbow

  • CBC

  • ESR, CRP

  • Urinalysis

  • Complement levels

  • If infectious etiology is to be ruled out, cultures, titers should be obtained.


Treatment
Treatment pain of both knees ( R > L), ankles (R > L), L elbow

Discontinuation of the offending agent

Supportive care

Antihistamines for urticaria

Nonsteroidal anti-inflammatory drugs (NSAIDs) for arthritis, arthralgia, or both

Steroids (Prednisone or Methylprednisolone)- Patients with higher fever (eg, temperature >38.5ºC), more severe arthritis and arthralgias, or more extensive rashes including extensive vasculitic rashes may be treated with short courses of glucocorticoids.


References
References pain of both knees ( R > L), ankles (R > L), L elbow

Brucculeri, M. et al. Serum sickness-like reaction associated with cefazolin. BMC Clin Pharmacol. 2006; 6: 3.

Hay Jr., W. et al. Current Pediatric Diagnosis and Treatment, 15th ed. McGraw-Hill. 2001: pp. 958-960.

www.emedicine.com

www.uptodate.com


Prep questions
PREP Questions pain of both knees ( R > L), ankles (R > L), L elbow

  • You have been asked by a local school to provide recommendations about the use of self-injectable epinephrine for anaphylaxis. The school supervisor is concerned about the increased incidence of peanut and tree nut food allergy. School officials have requested that each child who has a diagnosis of "food allergy" have two self-injectable epinephrine devices at the school nurse's office.Of the following, the BEST response regarding anaphylaxis is that


A. A patient should not receive a second dose of epinephrine unless a clinician is presentB. Epinephrine reaches higher peak plasma concentrations if injected into the thigh rather than the armC. Families should keep one epinephrine autoinjector in the car in case a reaction occurs after schoolD. Skin manifestations (eg, flushing, itching, urticaria) are rare in severe anaphylaxisE. Subcutaneous injection of epinephrine is preferable to intramuscular injection


In the past, outpatient administration of epinephrine was subcutaneous, but research has demonstrated that intramuscular injection, specifically in the thigh, is the preferred route and location due to higher and faster peak plasma concentration. If epinephrine is administered, parents or school should call emergency services to evaluate the child and transport him or her to the ER for further evaluation. The effects of a single dose of epinephrine typically last for 5 to 15 minutes; up to 20% of individuals experiencing anaphylaxis may require a second epinephrine dose. When symptoms persist, a second (or third) dose should be administered, even if the parent or school professional still is awaiting the ambulance.


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