(Kiosk 2) Petrified Ears and Multi-joint Contractures in a Patient with Addison’s Disease: A Case Report

Case Diagnosis: Petrified ears and multi-joint contractures in a patient with a 40-year history of Addison’s Disease.

Case Description or Program Description: Patient is a 59-year-old male with adrenal insufficiency diagnosed at age 18, treated with daily hydrocortisone. He presented with painful auricular cartilage that limited his ability to sleep, as well as chronic pain and stiffness in his bilateral hips worsening over last 20 years. More recently, he developed stiffness in his wrists, knees, and shoulders. Due to stiffness and pain with movement, he required assistance with dressing and toileting. Examination revealed hard and unmovable auricles, along with multiple painful large joint end-range contractures, most pronounced in the hips, but also in the small finger joints, limiting his hand use.

Setting: Outpatient musculoskeletal clinic

Assessment/Results: X-ray of the hips showed extensive enthesopathies around the pelvis, but otherwise normal appearing joints. Cervical and lumbar spine imaging showed age appropriate changes. Rheumatological workup was negative. Laboratory testing did not reveal hypercalcemia or other abnormality. Endocrinology did not recommend glucocorticoid dosing above baseline. He was referred to physical therapy to improve his ability to perform activities of daily living and maintain range of motion.

Discussion (relevance): 15 prior case reports describe muscle rigidity and joint contractures related to Addison’s disease. Some cases present acutely with severe muscle rigidity similar to Stiff Person Syndrome but are differentiated by an excellent response to glucocorticoids. In a small percentage of patients, pain and stiffness persist despite glucocorticoid treatment due to unclear causes and are difficult to treat. Bilateral calcification of the auricular cartilage, also termed “petrified ears”, is pathognomonic for ectopic calcification in endocrinopathies, and likewise treatment with glucocorticoids does not typically improve auricular symptoms.

Conclusions: This case highlights the musculoskeletal manifestations of adrenal insufficiency. Physicians should have high clinical suspicion for endocrinopathy in patients with painful joint contractures and petrified ears.