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Ecase # D-6
Dena Kennedy, M.D.
Feb 17-21, 2003
A 16 yo F is brought to you by her mother for consultation regarding
4 month hx of moderately severe refractory urticaria, angioedema and intermittent
fevers to as high as 102 degrees for as long as ½ hr, sometimes associated
with shaking chills accompanied by worsening hives.
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PMH: chronic allergic rhinitis, hx bronchitis and RAD, prior sinusitis,
acne and benign tremor (MRI of head, urinary copper, ceruloplasmin
level, LFTs and PFT's were negative on prior neurology evaluation.)
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Soc: HS cheerleader, 4.0 student, non-substance abuser who has missed
an average of 3 days of school each month due to these problems. Denies
sexual activity.
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Allergic: Penicillin and probably yellow dye (100 mcg thyroid dose
worsened swelling.)
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Rx: Allegra 180 mg/d, Levoxyl 50 mcg/d, Ranitidine 150 mg BID and
rare ibuprofen (no ASA)
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Fam Hx: goiter, DM, ASCVD and early breast CA
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ROS: 10-15 # wt gain in 4 mos, minimal seasonal rhinitis with drainage
She has been evaluated by multiple providers including her PCP, an allergist,
ENT surgeon and endocrinologist for help in managing her symptoms. Allergy
testing: 3-4+ for grasses but otherwise negative. Extensive lab positive
only for anti-thyroid antibodies in mod-high titer and a mild anemia,
NCNC. (ESR 6, ANA-,SPEP, IgE and complement levels and TSH are normal.)
She was placed on levothyroxine 100 mcg/d which caused racing heartbeat,
worsening tremor and was poorly tolerated. Dose was reduced to 50 mcg/d
but neither dose reduced or eliminated her Sx. CXR negative. CT scan of
sinuses documented L maxillary and ethmoid sinusitis, R maxillary thickening,
septal deviation and concha bullosa. Four weeks of biaxin failed to improve
her Sx. Old record review notes T11-T12 "compression fractures" noted
on spine films obtained after a fall. (Repeat x-rays are read as T11-12
and L1 wedging suggestive of juvenile kyphosis, or Scheurermann's Disease.)
Exam reveals classic urticarial lesions and mild angioedema but no fever.
Frequent clearing of post-nasal drainage noted. Cloudy middle ear fluid
present with retracted TMs. Thyroid upper normal in size. Otherwise negative
exam.
Questions
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Which of her conditions could be causing or have been associated
with angioedema and urticaria?
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What treatment changes would you try to alleviate if not eliminate
her symptoms.
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What studies might you obtain or repeat?
Comments
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Chronic urticaria and angioedema have been associated with both Hashimoto's
thyroidits and Graves' Disease, though the association with Graves's
Disease is less common. An auto-immune mechanism appears to be most
likely, but 60 percent of cases remain idiopathic. There is no clear
benefit demonstrated to thyroid suppression or anti-thyroid therapy
in resolution of chronic urticaria and angioedema. Sinusitis has been
associated with these conditions and treatment should be continued
until CT scan of sinuses are normal and symptoms have resolved. The
tremor does not appear to relate to her anti-thyroid antibodies.
Her spinal abnormalities appear to be a developmental anomaly and
are likely incidental. There does not appear to be a malignant or
rheumatologic problem (such as connective tissue disorder or vasculitis.)
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Levothyroxine should be tapered off or D/C'd. Zyrtec 10 mg QD to
BID is much more effective than Allegra for urticaria and Doxepin
cream 5% up to QID may be helpful for the pruritis. Sarna lotion OTC
is a good moisturizer with anti-pruritic benefits as well. Antibiotics
with good anaerobic coverage in a longer course may be needed to clear
likely sinusitis/otitis media. Aggressive management of allergic
rhinitis and environmental control re: allergens is important.
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Repeat CBC with diff/plts, comprehensive metabolic panel, blood cultures
and possibly repeat TFT's should be considered. (Complement determinations
are not indicated for patients who have hives alone, nor do they need
to be done when angioedema accompanies chronic urticaria, since patients
with a hereditary or acquired C1 deficiency do not have hives.) Skin
biopsies may be helpful in patients who have fever, arthralgias, a
high ESR, lesions which last 36 hrs or more or with associated petechiae
or purpura.
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Yellow dye in all medications and foods and probably salicylates
should be avoided.
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Patient also encouraged to stop cheerleading as exercise may worsen
urticaria. She declined to do this.
Afterword
She went to see a naturopath as well, who recommended a "specialized
diet to eliminate highly allergenic foods with reintroduction of individual
foods after one month, supplementation of bioflavonoids including quercetin
to stabilize mast cells and decrease histamine response for 6 months,
multivitamin for nutritional support during elimination diet, supplementation
to support digestion and decrease inflammation for 6 months."
Reference
Chronic Urticaria and Angioedema.
Allen Kaplan. NEJM, Vol.346, No.3. Jan. 17, 2002. pp 175-179.
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