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Why is my Hyperparathyroid Patient Itching?. LCDR Amie Harvey, MD LCDR Karen Kaufman, DO CDR Jason Daily, MD Naval Medical Center Portsmouth. Disclaimer. Nothing to disclose
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Why is my Hyperparathyroid Patient Itching? LCDR Amie Harvey, MD LCDR Karen Kaufman, DO CDR Jason Daily, MD Naval Medical Center Portsmouth
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.
Subjective • 37 year old female with 2 week history of itchy rash • Fatigue, headaches, nausea, decreased appetite, constipation
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
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
Diagnosis and Treatment • Primary Hyperparathyroidism (PHP) • Treatment: Surgical excision • Chronic Urticaria(CU) • H1 and H2 Antihistamines
Pathology • 1.2 gram, 3 x 0.9 cm homogenous, light tan inconspicuous capsule Parathyroid Adenoma 10x H&E Parathyroid Adenoma 40x H&E
PHP • Demographics • Prevalence 1: 1000 • More common in women (2:3) • Most common in 5th or 6th decade • Symptoms • Skeleton, cortical bone • Nephrolithiasis • Chronic pancreatitis • Polyuria • Polydipsia • Anorexia • Hypertension • Nausea • Vomiting
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 AcadDermatol 2001; 45:387-391. Toubi E, et al. Allergy 2004; 59:869-873.
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
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.
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.
Subtypes EAACI/GA2LEN/EDF/WAO guideline: definition, classification and diagnosis of urticaria, Allergy 2009: 64:1417-1426.
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
CAU Testing • 1986 Autologous Serum Skin Test (ASST) • Administration and interpretation not standardized • CU Index testing Cho , altrich et al. Ann Allergy Asthma Immunol2013; 110: 29-33.
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.
CAU • <img height="1" width="1" src="http://usadmm.dotomi.com/v?dtmid=475702197181741608&magic=42&comId=2206&msgCampId=77419&trid=245902528404169869&tid=78231&etype=91&edtl=12"/> • Medical Websites • Medical info • http://www.colorectaltumor.com • http://www.genitaldischarge.com • http://www.hepaticcarcinoma.com • http://www.hpvvaccination.com • http://www.ovarydisease.com • Medicine Info • <img height="1" width="1" src="http://usadmm.dotomi.com/v?dtmid=475702197181741608&magic=42&comId=2206&msgCampId=77419&trid=140702528404220272&tid=78234&etype=91&edtl=12"/> • Simple template. Powered by Blogger. • 21 of 27 Becky M, et al. Current Allergy and Asthma Reports 2005; 5:270-276.
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
Questions? Thank you.