Adiposis dolorosa

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Adiposis dolorosa , also known as Dercum disease or Anders disease , is a rare condition characterized by general obesity and fatty tumors in adipose tissue. Tumors are usually painful and are found in multiples of the extremities. The cause and mechanism of Dercum's disease is still unknown. Possible causes include nervous system dysfunction, mechanical pressure on the nerves, adipose tissue dysfunction, and trauma.

Dercum's disease was first described at Jefferson Medical College by nerves Francis Xavier Dercum in 1892.

Video Adiposis dolorosa

Signs and symptoms

Four cardinal symptoms are sometimes used as diagnostic criteria:

1. painful lipid lipomas (benign fatty tumors) throughout the anatomy
2. overweight, often in the age of menopause
3. weakness and fatigue
4. emotional instability, depression, epilepsy, confusion, and dementia.

There are also potential signs of the disease identified as follows:

However, since it is unclear which cardinal and which symptoms are minor signs of Dercum disease, it is unclear which one should be used as a diagnostic criterion. Researchers have proposed the 'minimal definition' based on symptoms that most often become part of Dercum's disease: 1) Overweight or general obesity. 2) Chronic pain in adipose tissue. Related symptoms in Dercum disease include obesity, fat deposition, easy bruising, sleep disorders, memory impairment, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint and muscle pain. Regarding the related symptoms in Dercum disease, only published case reports. There is no study involving a medical examination performed on a large group of patients.

Maps Adiposis dolorosa

Cause

Currently unknown cause of this disease. There are currently studies proposing several theoretical culprits that include inflammation of adipose tissue, nervous system damage and endocrine damage. None of the theories currently proposed have been found worthy. Because little is known about Dercum's disease, there is currently no known prevention way.

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Epidemiology

Dercum disease most commonly occurs between the ages of 35 and 50 years. It's five to thirty times more common in women than in men. Initially, Dercum proposed that conditions primarily affect postmenopausal women. However, a 2007 survey has revealed that 85.7 percent of patients who included it developed Dercum disease before menopause. The prevalence of Dercum disease has not been determined.

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Mechanism

Currently there is no known mechanism for this disease.

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Diagnosis

The diagnosis of Dercum disease is done by physical examination. To diagnose the patient correctly, the physician must first exclude all possible differential diagnoses. The basic criteria for Dercum disease are patients with chronic pain in adipose tissue (body fat) and patients who are also obese. Although rare, the diagnosis may not include obesity. Dercum disease can also be inherited and family medical history may help in the diagnosis of the disease. There are no special laboratory tests for this disease. Ultrasound and magnetic resonance imaging can play a role in the diagnosis.

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Treatment and prognosis

Common treatments for Dercum disease are directed to treat individual symptoms. Pain relief medication may be given to reduce temporary discomfort in patients. Cortisone shots have also proven to be effective for temporary chronic pain relief. Surgical removal of damaged adipose tissue can be effective, but often the disease will recur.

Several large studies convinced about the treatment of Dercum disease have been done. Most of the different treatment strategies present are based on case reports. Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum disease. Not enough research is done to prove that diet and supplements can help overcome this disease.

Treatment methods include the following modalities:

Surgery

Surgical excision of fatty tissue deposits around the joint (liposuction) has been used in some cases. Liposuction can relieve symptoms temporarily, although often relapse.

Medication

Traditional analgesics

Pain in Dercum disease is often reported to be refractory to analgesics and non-steroidal anti-inflammatory drugs (NSAIDs). However, this has contradicted the findings of Herbst et al. They reported that pain was reduced in 89% of patients (n = 89) when treated with NSAIDs and in 97% of patients when treated with narcotic analgesics (n = 37). The required dose and duration of pain relief are not exactly stated in the article.

Lidocaine

A preliminary report from 1934 showed that procaine intralesi injection (NovocainÃ,®) relieved pain in six cases. Recently, another type of local treatment of painful sites with lidocaine patch (5%) (LidodermÃ,®) or lidocaine/prilocaine (25 mg/25 mg) cream (EMLAÃ,®) has shown pain reduction in some cases.

In the 1980s, treatment with intravenous lidocaine infusion (XylocaineÃ,®) in various doses was reported in nine patients. The resulting pain relief lasts from 10 hours to 12 months. In five cases, lidocaine treatment was combined with mexiletine (MexitilÃ,®), which is an anti-arrhythmic 1B class with the same pharmacological properties as lidocaine. The mechanism by which lidocaine relieves pain in Dercum disease is unclear. It can block the conduction of impulses in the peripheral nerves, and thus break the circuit of the abnormal nerve impulses. However, it may also suppress brain activity which may cause an increase in pain threshold. Iwane et al. performing EEG during intravenous administration of lidocaine. The EEG shows slow waves appearing 7 minutes after the start of the infusion and disappear within 20 minutes after the end of the infusion. On the other hand, the pain-relieving effect is the greatest at about 20 minutes after the end of the infusion.

Based on this, the authors concluded that the effects of lidocaine on peripheral nerves most likely explain why the drug has an effect on the pain on Dercum's disease. In contrast, Atkinson et al. have suggested that the effects on the central nervous system are more likely, because lidocaine can suppress awareness and decrease brain metabolism. In addition, Skagen et al. showed that patients with Dercum disease did not have a vasoconstrictor response to arm and leg decline, suggesting that the local venous-arteriolar reflex simpaticusmediate did not exist. This may indicate an increase in sympathetic activity. Lidocaine infusion increases blood flow in the subcutaneous tissues and normalizes the vasoconstrictor response when the limbs are lowered. The authors suggest that pain relief is caused by the normalization of regulated sympathetic activity.

Methotrexate dan infliximab

One patient's symptoms improved with methotrexate and infliximab. However, in other patients with Dercum's disease, the effects of methotrexate are prudent. The mechanism of action is not clear. Previously, methotrexate has been shown to reduce neuropathic pain caused by peripheral nerve injury in studies in rats. Mechanisms in case studies of rats are considered as decreased microglial activation after nerve injury. Furthermore, a study has shown that infliximab reduces neuropathic pain in patients with central nervous system sarcoidosis. This mechanism is thought to be mediated by inhibition of tumor necrosis factor.

Interferon? -2b

Two patients successfully treated with interferon? -2b. The authors speculate on whether the mechanism could be a drug antiviral effect, the production of endogenous substances, such as endorphins, or interference with the production of interleukin-1 and tumor necrosis factor. Interleukin-1 and tumor necrosis factor are involved in skin hyperalgesia.

Kortikosteroid

Some patients noted some improvement when treated with systemic corticosteroids (prednisolone), while others experienced a worsening of pain. Weinberg et al. treated two patients with Juxta-articular Dercum disease with intralesional injection of methylprednisolone (Depo-Medrol). The patients had a dramatic increase. The mechanism for the ability to reduce corticosteroid pain in some conditions is unknown. One theory is that they inhibit the effects of substances, such as histamine, serotonin, bradykinin, and prostaglandins. Because the etiology of Dercum disease may not be inflammatory, it is plausible that the increased experience of some patients when using corticosteroids is not caused by anti-inflammatory effects.

Alternative medicine

CVAC session

Cyclic variation in adaptive adaptation (CVAC) is a touch-free, cyclic hypobaric pneumatic compression method for the treatment of tissue edema and, therefore, edema-related pain. As a pilot study, 10 participants with AD completed the pain and quality of life questionnaires before and after 20-40 minutes of CVAC process daily for 5 days. After treatment, there was a significant reduction in pain as measured by Catastrophizing Pain Scale and Visual Analogue Scale, but no change in pain quality by McGill Pain Questionnaire. However, there was no change in the Pain Endurance Index or the Pittsburgh Sleep Quality Index. This study demonstrates the potential role of treatment for CVAC, and the authors recommend randomized, controlled clinical trials.

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Transcutaneous power stimulation

Transcutaneous electric stimulation in one case is proven safe and effective in reducing symptoms. Treatments include 10 consecutive sessions over a 6 month period. After 4 months the pain of treatment is reduced and after 6 months the pain of treatment decreases.

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External links

• Dercum Disease Research
• Dercum disease in e-medicine

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References

Source of the article : Wikipedia