Dercum's disease

Dercum's disease is a rare condition characterized by multiple painful fatty tumors, called lipomas, that can grow anywhere in subcutaneous fat from scalp to plantar surface of the foot.^[1] Sometimes referred as adiposis dolorosa in medical literature, Dercum’s disease is more of a syndrome than a disease (because it has several clinically recognizable features, signs, and symptoms that are characteristic of it and that tend to occur together). While the term adiposis dolorosa may be correct, the term Dercum's disease is more often used, along with the acronym DD.^[1]

The onset of Dercum's Disease can be rapid or insidious and progressive, beginning most often in the third decade. Obesity and rapid weight gain is common in DD, with chronic fatigue and pain unresponsive to analgesics.^[1] Pain associated with Dercum’s disease can often be severe and may be caused by lipomas pressing on nearby nerves or inflamed connective tissue, also called fascia, which is commonly associated with the lipomas.^[2]

The cause and mechanism of Dercum's disease remains unknown.^[3] Possible causes include genetics (autosomal dominant inheritance), trauma, nervous system dysfunction, disturbances in endocrine system and metabolism of fat, or it maybe an autoimmune disorder.^[2][4] Some cases of Dercum's may occur in one or more people in a family with familial multiple lipomatosis.^[5] Although surgical resection or liposuction improves pain, regrowths occur in 50% cases.^[1] DD mainly occurs in adults of ages 35–50 years and more women are affected than men.^[6]

The disease was described for the first time by an American neurologist Francis Xavier Dercum, who headed the University of Pennsylvania’s Neurological Clinic and was the personal physician to President Woodrow Wilson.^[1][7] He published two papers on the disease in 1888 and 1892, and he used the term “adiposis dolorosa”.^[5] Further, the disease was reported also in Philadelphia by the American physician James Meschter Anders (1854–1936) and the British physician and medical biographer at Guy’s Hospital in London, Sir William Hale White (1857–1949). In the past, Dercum's was considered synonymous with Lipedema, but it is now regarded as a separate condition. It has been recognized by the World Health Organization in ICD-10. Orphanet and the National Organization of Rare Disorders also listed the disease.^[5]