A Case of
Recurrent
Anaphylaxis
Lynne M. Zheutlin, MD
Case presentation
History of present illness
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43 YO WF seen in the ER six times
for anaphylaxis
Initial episode after gardening and
taking a walk
Developed swelling of hands and
feet, trouble breathing and
swallowing
Treated with oxygen, IV Benadryl
and prednisone
History of present illness
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She presented to the ER 3 more
times over the next 4 weeks.
Breathing progressively worse with
each. All began with swelling of
hands and feet, but no urticaria.
No consistent pattern of exercise
or food ingestion before-hand.
Used Epipen at onset of fourth
episode; unknown benefit
Developed pyschosis secondary to
steroid treatment
History of present illness
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She was seen first by another
allergist.
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Diagnosed as vascular leak
syndrome and referred to a
vascular surgeon
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Fifth episode associated with loss
of consciousness.
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Admitted to pulmonary service
History of present illness
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CXR abnormal and TB suspected;
PPD negative and diagnosis R/O
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Sent home on daily loratadine
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Sixth episode described as trouble
breathing without swelling. Seen
in ER and sent home
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Presented for a second opinion
Past Medical History
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Pneumonia
Bronchitis, sinusitis
No hx of asthma or rhinitis or hives
Bipolar disorder
Hypertension
Herniated cervical discs
Stevens-Johnson Syndrome
(tegretol)
Rash from penicillin, Demerol,
codeine
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Family HX negative for
anaphylaxis or angioedema
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ROS positive only for mild cough
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Physical exam – BP 140/80, P 72,
PF 400, mild pedal edema
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Skin testing for inhalants negative
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RAST from other allergist negative
for foods
Laboratory Findings
(from other allergist)
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WBC 9000, Hgb 11.8, Hct 35.7,
MCV 105, MCH 34.6, Normal diff
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Metabolic panel WNL
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Urinalysis 3+ occult blood, 15-20
RBC/HPF
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C1 esterase inhibitor 30 mg/dl (2840)
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C1q 13.5 (7.9-25)
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Anti-DNA (DS) Ab 25 U/ml (0-99)
Hospital Records
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6/28/03: Presented with swelling of
throat and hands, shortness of
breath, hoarseness, dizziness
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BP 143/84, P 95, R 28, O2 Sat 98%
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Exam showed swelling of uvula,
tongue, hands, hoarse voice
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Lungs clear; no respiratory distress
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Meds: lithium, Depakote, Restoril,
Cardizem, Vistaril, Nexium, Seroquel
Hospital Records
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Treated with IV Decadron,
epinephrine, Benadryl, Pepcid,
2L O2
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Received second dose of
epinephrine and Benadryl
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At discharge: BP 141/81, P78,
R20, O2 Sat 99%
Hospital Records
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8/15/03 (fifth episode) Presented to
ER with swelling of throat and tongue
after a wasp sting. Noted to be
lethargic. BP 146/85, P 110, R 19, O2
Sat 99%
RX IV Solumedrol, epinephrine,
Benadryl.
WBC 26,000. Given Rocephin and
admitted. Tequin begun.
CXR: RUL infiltrate. CT scan: nodular
disease RUL. PPD neg.
At discharge WBC 10,000. Folate 7.1,
Vit B12 505. Told to discontinue
Seroquel. Began daily loratadine
Follow-Up
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Reported one episode of shortness
of breath and leg swelling in
recovery room after back surgery.
Treated with epinephrine and
Benadryl.
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No further episodes for 4 months
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Current meds: Depakote, Restoril,
Nexium, Benicar, Lithium, Celebrex,
Vit B12. Taking Benadryl daily to
prevent recurrence.
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Switched to Zyrtec.
Follow-Up
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Phone call 7/8/04: Seen at
Washington Adventist for 7th
allergic reaction. Had eaten ice
cream at a lacrosse game.
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Could not swallow, lost voice,
swelling of hands and feet. Used
Epipen.
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Received second dose of
epinephrine in ER
Idiopathic Anaphylaxis?
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No consistent history of food
ingestion before episodes. RAST
for common foods negative.
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Insect sting associated with only
one episode. Should she be tested
for hymenoptera?
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Seroquel considered as cause, but
had reactions after discontinuation.
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No association with exercise
Idiopathic Anaphylaxis
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Anaphylaxis with no definable
etiology and not triggered by
exogenous allergens
335 cases reported by AllergyImmunology Division,
Northwestern University
Categorized as idiopathic
anaphylaxis- generalized or
idiopathic anaphylaxis-angiodema.
Further classified as frequent
(>6/y) or infrequent
Manifestations of
Idiopathic Anaphylaxis
Urticaria or
angioedema
Upper airway
obstruction
335
100%
210
63%
Bronchospasm
132
39%
Hypotension or
syncope
Gastrointestinal Sx
78
23%
75
22%
Therapeutic Regimen
Idiopathic Anaphylaxis
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Infrequent episodes (<6/y): Treat
acute episodes with epinephrine,
prednisone and antihistamine
Frequent episodes (>6/y): Initially
prednisone 60 mg qAM,
hydroxyzine 25 mg TID, albuteral
2mg TID, Epipen to carry
After episodes disappear,
prednisone converted to every
other day and tapered by 5-10 mg
monthly
Questions
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Is this Idiopathic anaphylaxisangioedema? Should she receive
prophylactic treatment?
Could this be undifferentiated
Somatoform IA?
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History mimics IA but lacks
correlating objective physical
findings, shows no response to
therapeutic regimen, and meets
criteria for undifferentiated
somatoform disorder
How does the history of psychosis
secondary to corticosteroids
impact treatment?