| Dercum's disease (adiposis dolorosa) is a rare condition characterized by progressively painful fatty deposits, usually, in menopausal women with obesity, asthenia and mental phenomena. We report a case of a 48-year-old woman with recurrent neck swelling and pain in the neck and parotid region, and a review of management of this uncommon problem. ( info) |
| adiposis dolorosa is a rare condition characterized by multiple painful lipomas. We describe two typically affected siblings who were found to have at least 5 affected first-degree relatives in two generations. Manifestations were remarkably variable, ranging from complete disability to solitary asymptomatic tumors. review of histopathologic findings did not reveal any features that might distinguish the tumors from common sporadic lipomas. Mutational analysis excluded the 8344 A to G mitochondrial mutation seen in other patients with multiple lipomas. adiposis dolorosa may be an extreme manifestation of the more common condition of familial multiple lipomas. Further work is needed to define the genetic basis of these conditions. ( info) |
3/21. dysarthria, progressive parkinsonian features and symmetric necrosis of putamen in a family with painful lipomas (Dercum disease variant). We describe painful subcutaneous lipomatosis in four members of a two-generation family. Lipomas appeared in adulthood, were circumscribed, painful on touch and mainly localized to the trunk and proximal parts of the extremities. The disorder was associated with dysarthria, visual pursuit defect and progressive dystonia. MRI showed bilateral increasing cystic lesions in the basal parts of the putamen. No other abnormalities were detected. The lesions corresponded well with the clinical presentation in the patients. Investigation for mitochondrial disease with muscle biopsy and mitochondrial dna gave normal results. No consistent biochemical changes were found. The disorder in this family was considered to differ from MERRF with lipomatous lesions and multiple symmetric lipomatosis but compatible with a Dercum disease variant. ( info) |
4/21. Hormonal and metabolic study of a case of adiposis dolorosa (Dercum's disease). A case of a 43-year-old nonobese woman with adiposis dolorosa (Dercum's disease) is reported. Muscle glucose uptake and oxidation before and after ingestion of 75 g of glucose were similar to control group values, although a greater insulin release (16,578 vs 6,242 /- 1,136 microU/3 h) occurred simultaneously. in vitro studies of abdominal normal and painful subcutaneous adipose tissue of the patient revealed lower responsiveness to norepinephrine and lack of response to the antilipolytic effect of insulin in the painful adipose tissue (0.98 vs 1.43 microM FFA/10(6) cells at 5.0 microM of norepinephrine). The disease was not correlated with the HLA system and there were no alterations in hormonal secretion at the pituitary, adrenal, gonadal, and thyroid levels. These findings indicate the presence of peripheral insulin resistance in this patient with adiposis dolorosa. ( info) |
5/21. Painful fat syndrome in a male patient. Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg with tenderness, but excluding the feet. Lipoedema occurs almost exclusively in females, and no male patient has been reported in the literature published in English. We report on an extremely rare presentation of lipoedema in a male patient. A thorough study based on the case history, physical manifestations, and magnetic resonance imaging (MRI) provided valuable clues for a differential diagnosis. Conservative treatment using weight reduction, compression-stocking application, and diuretic therapy was not effective. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment. ( info) |
| Dercum's disease, or adiposa dolorosa, is a rare condition characterized by the development of multiple painful subcutaneous lipomas. Postmenopausal women are selectively affected. The ankle area is an uncommon site of involvement. We report a case with lipomas about the ankle, and we review the relevant literature. CASE-REPORT: This 46-year-old perimenopausal woman receiving follow-up for depression presented in December 1997 for swelling and mechanical pain in the right ankle. Lumps behind and under the malleoli were removed surgically and found to have the typical histological features of lipomas. In January 2000, a lump developed behind and under the malleoli of the left ankle, and the lumps on the right recurred. The physical examination showed obesity, with a body mass index of 32.04 kg/m(2). Firm, mobile, painless lumps were felt under and behind the malleoli on both sides. The ankles were painful, although range of motion was normal. Examination of the back found two similar lumps. The erythrocyte sedimentation rate was 10 mm/h and the c-reactive protein level was 6 mg/l. serum lipid levels were normal. Findings were normal from plain radiographs of the ankles. Ultrasound scanning of the ankles confirmed that the lumps were composed of adipose tissue. magnetic resonance imaging visualized an arc-shaped lipomatous mass on the left. Whole-body bone density measurement found an increase in fat mass of the lower limbs with normal bone mass. The ankle lipomas were removed surgically. At last follow-up 18 months later, she was free of recurrence. DISCUSSION: Dercum's disease is a rare condition of unknown etiology characterized by multiple, often painful lipomas. paresthesia in the overlying skin is common. Lipomas about the joints cause mechanical arthralgia. The diagnosis, which is often delayed, rests on ultrasonography and, above all, magnetic resonance imaging. A combination of medications, surgery, and psychiatric care is usually needed. The treatment aims at relieving the pain and restoring a normal appearance. ( info) |
7/21. Corticosteroid-induced juxta-articular adiposis dolorosa. Long-term treatment with high doses of corticosteroids leads to the development of truncal obesity and focal fatty deposition. These deposits characteristically are located on the face, the nuchal and truncal areas, and episternally, as well as in the mediastinum and epicardium. We studied a patient with juxta-articular adiposis dolorosa who had L-tryptophan-associated eosinophilia-myalgia syndrome and was treated with high doses of prednisone. This is the first reported case of adiposis dolorosa occurring as a complication of corticosteroid treatment. Alterations of fat metabolism induced by corticosteroid excess may have played a role in the development of this unusual painful syndrome. ( info) |
8/21. adiposis dolorosa (Dercum's disease): liposuction as an effective form of treatment. The clinical picture of adiposis dolorosa makes a lasting impression on the examining physician. The patient is typically an obese, asthenic woman who appears to have a low pain threshold. She has an unusual distribution of fatty tumors, and her complaint of pain in these tumors seems out of proportion to the physical findings. alcoholism, emotional instability, and depression are common, and narcotic pain medicine is frequently requested. The patient is easily dismissed as a malingerer after a brief examination. However, liposuctioning of the painful fatty tumors appears to be both practical and effective. While adiposis dolorosa is an unusual disease, it is one that plastic surgeons can recognize and treat. ( info) |
9/21. Liposuction in the treatment of juxta-articular adiposis dolorosa. Two patients are presented who had symptomatic relief of juxta-articular adiposis dolorosa of the knees after liposuction. Liposuction is recommended for the treatment of this condition. ( info) |
10/21. The regulation of subcutaneous blood flow in patient with Dercum's disease. Dercum's disease or adiposis dolorosa is a poorly understood disorder with painful fatty deposits in the skin localized to the lower extremities. The etiology is unknown. In such a patient the mechanisms of local regulation of blood flow in subcutaneous tissue was investigated by the local 133Xenon washout technique. The patient was reinvestigated one week after treatment with intravenous lidocaine. The local vasoconstrictor response to increase in venous transmural pressure was not present in this patient, but reappeared after lidocaine treatment. Autoregulation of blood flow in subcutaneous tissue was present before as well as after lidocaine treatment. It seems likely that a pain elicited increase in sympathetic activity in the vasoconstrictor fibres abolished the normal vasoconstrictor response to increase in venous transmural pressure. The mechanism of pain relief after intravenous lidocaine infusion is uncertain, but central as well as peripheral mechanisms may be considered. ( info) |