Download Why is my Hyperparathyroid Patient Itching?

Why is my Hyperparathyroid
Patient Itching?
LCDR Amie Harvey, MD
LCDR Karen Kaufman, DO
CDR Jason Daily, MD
Naval Medical Center Portsmouth
”FIRST AND FINEST”
Disclaimer
• Nothing to disclose
• The views expressed in this presentation are
those of the author(s) and do not necessarily
reflect the official policy or position of the
Department of the Navy, Department of Defense
or the United States Government.
”FIRST AND FINEST”
Subjective
• 37 year old female with 2 week history of
itchy rash
• Fatigue, headaches, nausea, decreased
appetite, constipation
”FIRST AND FINEST”
Medical History
• No history of medical problems
• No known allergies
• No family history of thyroid or parathyroid
disease
• No tobacco, alcohol or illicit drug use
• No current medications
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Physical Exam
• Afebrile; HR 82; BP 125/86; RR 14
• Skin with erythematous, edematous
wheals without central pallor on lower
back, chest and upper extremities without
facial, periorbital or mucosal swelling
• Thyroid without diffuse enlargement or
palpable nodules
• No lymphadenopathy
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Skin Findings
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Laboratory Results
Complete Blood Count
Basic Metabolic Panel
WBC / mcL
6,000
Na (mmol/L)
141
HgB g/dL
14.1
K (mmol/L)
4.2
HCT (%)
41.5
Cl (mmol/L)
105
PLT/mcL
188,000
CO2 (mmol/L)
25
Neutrophils (%)
72.4
BUN (mgl/dL)
12
Lymphocytes (%)
21.9
Cr (mg/dL)
0.8
Eosinophils (%)
0.0
Ca (mg/dL)
11.4 ♦
”FIRST AND FINEST”
Laboratory Results
Additional Labs
Parathyrin Hormone (pg/mL)
136 ♦
(10-65)
Vitamin D, 25 Hydroxy (ng/mL)
57.3
(30-100)
24 h urine calcium (mg/24h)
366♦
(100-300)
1.62% ♦
FECA
1.45 ♦
1.16-1.32
Thyroglobulin AB (IU/mL)
<20
<20
Thyroperoxidase AB (IU/mL)
14
<35
Environmental Allergens sIgE
Negative
Ca ionized (mmol/L)
CU index (histamine%)
69 ♦
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(<16)
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Diagnosis and
Treatment
• Primary Hyperparathyroidism (PHP)
– Treatment: Surgical excision
• Chronic Urticaria (CU)
– H1 and H2 Antihistamines
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Pathology
• 1.2 gram, 3 x 0.9 cm homogenous, light tan
inconspicuous capsule
Parathyroid Adenoma 10x H&E
Parathyroid Adenoma 40x H&E
”FIRST AND FINEST”
PHP
• Demographics
• Symptoms
– Prevalence 1: 1000
– More common in
women (2:3)
– Most common in 5th or
6th decade
–
–
–
–
–
–
–
–
–
Skeleton, cortical bone
Nephrolithiasis
Chronic pancreatitis
Polyuria
Polydipsia
Anorexia
Hypertension
Nausea
Vomiting
”FIRST AND FINEST”
CU
• Daily, near daily, greater than 6 weeks
• Self limited in most patients
– Average duration is 2-5 years
– Spontaneous remission at one year for 30%
– Persists greater than 5 year in 20%
• Strong Association with Autoimmune
disease
Sarbjit S. Saini, Middleton’s Allergy: Principles and Practice 8th edition, 2013: 575-587.
Kulthanan K, et al. J Dermatol 2007; 34:294-301.
Kozel MM, et al. J Am Acad Dermatol 2001; 45:387-391.
Toubi E, et al. Allergy 2004; 59:869-873.
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Cutaneous
Associations
• Multiple Endocrine Neoplasia Type 1
– Angiofibromas (85%)
– Collagenomas (70%)
– Lipomas
– Café-au-lait spots
• Multiple Endocrine Neoplasia Type 2
– Cutaneous amyloidosis
• Sporadic Hyperparathyroidism
– Metastatic calcification
Fuleihan GE et al : Clinics in Dermatology 2006; 24; 281-288
”FIRST AND FINEST”
PHP and Urticaria
• PHP
– Parathyroid hyperplasia (1 case)
– Parathyroid Adenoma (3)
• 1st reported case of PHP with urticaria
– Reported in JAMA 1983 in 58 year old female
with 1 year of urticaria
– 4 gram adenoma
Liechty DR, Firminger HI: JAMA 1983; 250:789-790.
”FIRST AND FINEST”
Previous Theories
• 1983 JAMA – “circulating antigen antibody
complexes”
– Similar to other necrotic cancers
– Hemorrhagic areas, cyst formation, and fibrosis
induce antigen formation
• Hypercalcemia
– Induces mast cell degranulation
– Induces histamine release
• Calcium infusion did not reproduce urticaria
• Urticaria not present in other cases of hypercalcemia
Liechty DR, Firminger HI: JAMA 1983; 250:789-790.
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Subtypes
Urticaria
25%
Chronic Urticaria
Chronic
Idiopathic
Urticaria (CIU)
Physical Urticaria
Chronic Autoimmune
Urticaria (CAU)
Acute
Urticaria
Systemic
Disease
Malignancy
EAACI/GA2LEN/EDF/WAO guideline: definition, classification and diagnosis of urticaria, Allergy 2009: 64:1417-1426.
”FIRST AND FINEST”
• Persuasive, but not conclusive evidence
for the existence of autoimmune chronic
spontaneous urticaria was found by a
European taskforce panel 2013
Konstantinou GN, Asero R, Ferrer M, et al. EAACI taskforce position paper: evidence for
autoimmune urticaria and proposal for defining diagnostic criteria. Allergy 2013;68: 27
”FIRST AND FINEST”
CAU Testing
• 1986 Autologous Serum Skin Test (ASST)
– Administration and interpretation not
standardized
• CU Index testing
Cho , altrich et al. Ann Allergy Asthma Immunol 2013; 110: 29-33.
”FIRST AND FINEST”
CU Index Test
• Donor basophils combined with:
– patient’s serum
– buffer control
– anti-IgE
• Alliquots of cells lysed for histamine
content
• Histamine release is measured and
compared with total histamine in basophils
Cho , altrich et al. Ann Allergy Asthma Immunol 2013; 110: 29-33.
”FIRST AND FINEST”
CAU
Becky M, et al. Current Allergy and Asthma Reports 2005; 5:270-276.
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Follow up Testing
Preoperative
Calcium
Postoperative
11.4 ♦
Parathyroid
hormone
136 ♦
CU Index
69 ♦
Calcium
9.0
Parathyroid
Hormone
26
CU Index
< 16 (negative)
”FIRST AND FINEST”
Our Case
• Resolution of urticaria after adenoma
excision
• Resolution of positive index supports IgG
against IgE or IgE receptor is stimulated
by parathyroid adenoma
• Further studies of possible subclinical
antibody presence in PHP warranted
”FIRST AND FINEST”
Questions?
Thank you.
”FIRST AND FINEST”