Wednesday, January 5, 2011

Adiposis Dolorosa - neurolipomatoza

Adiposis Dolorosa, Dercum's disease or syndrome neurolipomatoza is an unusual progressive, of unknown etiology characterized by multiple painful lipomas that arise in adulthood, most frequently affect women who are obese postmenopausale. The onset is insidious. Pain due to compression of the nerve lipomas and is described by patients as "fat that hurts." Pain intensifies with the increase lipomas in connection with her periods. Reinlocuirea estrogen at menopause does not reduce pain.
Since the original description of Dercum disease, the clinical spectrum has changed today, including obesity, fatigue, weakness, mental changes, such as depression, confusion and dementia.Diagnostic criteria include four cardinal symptoms: painful multiple fat mass, generalized obesity, especially during menopause, fatigue, weakness and fatigue, mental changes with emotional instability, depression, epilepsy, confusion and dementia. Associated conditions include sleep disorders, dry eyes and mouth, irritable bowel, coccidodinie, vulvo, vulvodinie, carpal tunnel syndrome, Tietze's syndrome, thyroid dysfunction, trochanter, tendonitis, fibromyalgia.
No treatment can change the course of the disease, and those available are only symptomatic. Indicate systemic corticosteroids, intravenous anesthetics, analgesics. Liposuction produces significant improvement in pain and quality of life.
Pathogenesis and causes
It is considered hereditary disease that is transmitted by an autosomal dominant pattern, the mother-daughter line. Disease mechanisms are still unknown. The origin of pain is uncertain. Fatty deposits cause nerve compression with weakness and pain. Histological examination of these tumors did not show differences from ordinary lipomas.suggested that the disease is an expression of familial multiple lipomas, autosomal dominant disorder.
Signs and symptoms
Adiposis Dolorosa occurs in people 45-60 years old. There is never seen in children and rare in women under 45 years. Women observe nodule arising de novo, or existing ones are growing. They describe their pain and discomfort associated with weakness in the region. Before the onset of disease the patient is just slightly obese, but soon it becomes overweight. The pain increases with increased fatty tissue in relation to menstruation.Lipomas occur in any location except the head and torso.
Different types can be identified according to the extension of pain:I-type, juxta-ear type, with painful folds or grease inside of the thighs and knees-Type II, diffuse, generalized, pain extending into the upper arm, feet-Type III lipomatosis, nodular type, with intense pain in and around multiple lipomas attached to surrounding tissue.Pain can range from discomfort, mild pressure, burning, stinging, until the paroxysmal attacks. Pain is symmetric but can become localized in the knees and legs. Pain can be felt in the oases were fat.Hiperalgia can see and touch the gentle pressure of fatty tissue under the skin. The pain is dependent on temperature and time decreases when it is hot and dry, and when the pressure is high. Warm baths have a calming effect of short duration. Penbtru menopausal estrogen therapy does not reduce pain.
Other symptoms and signs include:-Swelling tendency of the fingers, with numbness in median nerve compression, General fatigue, moderate physical activity worsens fatigue or sleep disturbance-Tendency to bruise the delicate vessels in fat formation, coagulation tests are normalMorning stiffness after rest--Headache, sore throat, headache, eye pain-Cognitive dysfunction, concentration and memory problem-Episodes of depression, feeling of heat.
Diagnosis
Laboratory studies:Slightly elevated levels of cholesterol-Erythrocyte-sedimentation rate is slightly elevatedCoagulation tests are normal--Is type 2 diabetes and hypertensionIncreasing alpha-1-antitrypsin, orosomucoidului, haptoglobinei, complementHeat produced by fat cells, is measured twice above the overweightMonounsaturated fatty acids-rate is high, the level of substance P in CSF is low.
Histological examination. The elements described can not differentiate between ordinary lipomas and adiposis Dolorosa. Accumulation of lymphocytes was detected perivascylara. Tumors can be encapsulated or diffuse.The differential diagnosis is made with the following conditions: progressive lipodystrophy, Proteus syndrome, fibromyalgia syndrome, Madelung, neurofibromatosis, Cushing's syndrome, myasthenia gravis.
Treatment
Medical therapy.It is administered corticosteroids: prednisone. Induction therapy is associated with disease. Anesthetics: intravenous lidocaine can relieve pain from 10 hours to several months. The exact mechanism of action is unknown. Long-term intravenous therapy is associated with neurotoxicity. Traditional analgesics such as non-steroidal anti-inflammatory effect are weak. Lipomas do not respond to analgesics. Localized pain can be treated with injections of cortisone / anesthetic.
Surgical therapy.Liposuction is a supportive treatment for the disease. Skeletal pain is not affected. This method significantly improves quality of life and the pain decreases. Beneficial results diminish over time. Liposuction is indicated for patients with generalized low fat or fat deposits located at the knees, arms, stomach. Excision of isolated painful lipomas is not preventive but relieves local pain.

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