Endocrine Abstracts

Decrum’s disease or adiposis dolorasa is a rare progressive syndrome of unknown aetiology. It is characterised by painful fatty deposits, general obesity, weakness and unexplained emotional disturbance.¹

We describe a case of a 55-year-old man who presented with pneumonia. On examination he was noted to be overweight and have multiple skin nodules. His chest X-ray was abnormal; he underwent further workup with a CT thorax/abdomen/pelvis. This revealed no lung abnormality but demonstrated bilateral adrenal lesions. Biochemical testing revealed these were non-functioning lesions. He underwent an MRI of the adrenals, which showed changes consistent with bilateral lipomas (2.6 cm on left, <2 cm on right). He denied any previous past medical history or any family history of note.

One of his skin lesions was biopsied; this showed mature adipose tissue with focal loose stroma, containing mast cells, consistent with a lipoma.

The WHO classifies Decrum’s disease as rare. It is typically associated with overweight, postmenopausal women. It may be inherited in an autosomal dominant manner, however most reported cases are sporadic.² It has multiple associated conditions including pickwickian syndrome, dry eyes and mouth; sleep disturbance, irritable bowel and carpel tunnel syndrome.

On review of the literature, we were unable to identify any case reports of Decrum’s disease and adrenal lipomas, however typically it is believed they can occur anywhere in the body except the head and neck.³ This case illustrates an unusual cause of adrenal lipomas.

References: 1. Brodovsky S, Westreich M, Leibowitz A & Schwartz Y. Adiposis dolorosa (Dercum’s disease): 10-year follow-up Ann Plast Surg. 1994 33 (6) 664–8.

2. Lynch HT & Harlan WL. Hereditary factors in adiposis dolorosa (Dercum’s Disease). Am J Hum Genet. 1963 15 (2) 184–90.

3. Hansson E, Svensson H & Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet Journal of Rare Diseases. 2012 7 23.