The Musical Footprint of Cushing Syndrome

Endogenous pathologic hypercortisolism, or Cushing syndrome, is associated with poor quality of life, morbidity, and increased mortality.¹ The clinical presentation of Cushing syndrome varies, in part related to the extent and duration of cortisol excess.² When hypercortisolism is severe, its signs and symptoms are unmistakable. In particular, proximal muscle weakness; increased fat in the abdomen, torso, and face; and wide purple striae suggest marked hypercortisolism.

Because of the variety in presentation, patients are often referred to a dermatologist for red facial skin, poor wound healing, and striae. Early diagnosis may mitigate the progression of the disease. We present the case of a 19-year-old musician presenting with a history of weight gain of 10 kg in 2 years, muscular weakness in lower limbs, skin fragility, and repeated ecchymosis. Purple marks developed on the thorax, thighs, shoulders, and arms.³

The patient was mainly concerned about “flame-shaped” abdominal striae, that according to him, appeared several months before. These wide purple striae had a very typical appearance, forming the shape of a lyre (Figure 1A), an instrument associated in the Greek mythology with Apollo, God of art, music, and poetry (Figure 1B).

These signs and symptoms suggested Cushing syndrome, which was confirmed by endocrine evaluation showing a high level of 24-hour urinary free cortisol (2036 nmol/24 hours; reference range, 30-200 nmol/24 hours)⁴ and brain magnetic resonance imaging (MRI) exploration showing the presence of a pituitary microadenoma located inside the left pituitary wing (Figure 2A).

The adenoma was surgically removed by a transsphenoidal approach. The patient's postoperative course was uneventful. Normalization of 24-hour urinary free cortisol level was achieved 4 days after surgery (200 nmol/24 hours). Histological examination confirmed the diagnosis of corticotroph pituitary adenoma. At 6 months, postoperative MRI confirmed total resection of the adenoma (Figure 2B). The patient had lost 12 kg, the purple marks had significantly decreased, endocrine functions were normalized, and he now continues to play his guitar.

Corresponding Author: Aymeric Amelot, MD, PhD, Department of Neurosurgery, Hopital La Pitié-Salpétrière, 80 Boulevard de l’Hopital, 75013 Paris, France (aymmed@hotmail.fr).

Published Online: May 31, 2016. doi:10.1001/jamainternmed.2016.2078.

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.