What is Adiposis Dolorosa?
Adiposis dolorosa is an uncommon condition where painful fat tissue growths occur throughout the body. It’s also known as Dercum disease, Ander syndrome, or a number of other names, and was first identified in the late 1800s by an American neurologist named Francis Xavier Dercum. This disease comes in four types, each presenting differently: generalized diffuse, generalized nodular, localized nodular, and juxta-articular forms.
The generalized diffuse type is characterized by widespread painful fat tissue with no obvious lumps of fat. Whereas the generalized nodular type includes widespread painful fat tissue that hurts more around any present fat lumps. The localized nodular type confines the pain to the areas within and around these fat lumps. Finally, the juxta-articular type presents as a painful solitary lump of fat near large joints.
Diagnosis of adiposis dolorosa is based on clinical symptoms, and it’s a kind of last resort diagnosis when everything else has been excluded. The suggested standards for diagnosis comprise chronic fat tissue pain for over three months in overweight or obese patients, although these standards still need to be confirmed. The pain is typically chronic, intense, and difficult to treat. Alongside this, the disease often brings weakness and psychological symptoms like depression.
Other symptoms that may appear include fat deposits, easy bruising, trouble sleeping, memory issues, concentration difficulties, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, and joint pain.
What Causes Adiposis Dolorosa?
The exact cause of this particular disease is still unknown. However, there are several theories, including problems with the nervous system, hormonal imbalance, issues with the lymphatic and blood system, fat tissue not functioning correctly, pressure on nerves, and trauma-induced. Although there have been a few patient cases where the disease seemed to be passed down through families (called autosomal dominant) with varying symptoms, this is not the norm. In most instances, the disease seems to occur randomly without any specific genetic changes.
Risk Factors and Frequency for Adiposis Dolorosa
Adiposis dolorosa, a condition often reported in medical cases, tends to be more common in overweight women aged between 35 and 50. Earlier, it was believed that this condition was more prevalent in women after menopause. However, newer studies have discarded this belief.
Signs and Symptoms of Adiposis Dolorosa
People with adiposis dolorosa often initially notice achy, stab-like, or burn-like pains from growths or nodules. The pain usually spreads evenly on both sides of the body, and touching the affected areas can make the pain worse. It is especially common in the upper parts of the limbs, the body trunk, and the buttocks.
Typically, when you meet patients with this disorder, they are likely to be overweight or obese. You might also notice that their experience of pain doesn’t always match up with what can be seen physically during an examination.
Testing for Adiposis Dolorosa
There aren’t any lab tests that can directly indicate the presence of this particular condition, however, it’s important to perform other tests to rule out similar illnesses. Doctors should check hormone levels to ensure conditions such as Cushing syndrome, thyroid issues, or other hormonal imbalances aren’t present. It’s been observed that patients with this condition often exhibit slightly to moderately increased cholesterol levels. Additionally, the rate at which red blood cells settle out in a tube of blood (known as the erythrocyte sedimentation rate or ESR) may be a bit higher than normal.
Alongside lab work, imaging techniques like ultrasound and magnetic resonance imaging (MRI) can assist in diagnosing this condition. An ultrasound might reveal multiple, small elongated areas that reflect more ultrasound waves (referred to as hyperechoic nodules). An MRI scan of patients might show numerous elongated fat lesions just beneath the skin surface, accompanied by an increase in fluid signal that gives a “blush-like” appearance.
Treatment Options for Adiposis Dolorosa
Although there are many treatment options available, there is no one-size-fits-all solution for this disease. Treatments are tailored to the specific symptoms of each patient, aiming to ease these symptoms and provide support. Weight loss doesn’t seem to affect lipomas, and in some instances makes symptoms worse. Since the pain can come and go and be severe, a pain management specialist might be needed.
Several treatments have been suggested for pain based on case reports, including painkillers, liposuction, lipectomy (surgical removal of fatty tissue), and massage therapy that helps to drain lymph fluids. However, traditional painkillers don’t seem to be very effective in managing pain from adiposis dolorosa, although some benefit has been seen using non-steroidal anti-inflammatory drugs (NSAIDs) and narcotics.
There are other medications and treatment methods which may or may not be beneficial, such as the use of local anesthetics like lidocaine, the anesthetic drug ketamine, the chemotherapy drug methotrexate, the remedy infliximab, interferon alpha-2b used in virial treatment, corticosteroids, calcium-channel modulators, transcutaneous electrical stimulation (electrical nerve stimulation treatment), and rapid cycling hypobaric pressure (a type of pressure treatment).
Liposuction and lipectomy may offer short-term relief from pain, but it’s common for the pain to return and these operations don’t usually improve quality of life much.
What else can Adiposis Dolorosa be?
Adiposis dolorosa is a condition that should only be diagnosed after other possibilities have been ruled out. Similar conditions could be:
- Fibromyalgia, which causes widespread muscle pain and fatigue
- Panniculitis, characterized by painful lumps beneath the skin
- Endocrine disorders such as Cushing syndrome and hypothyroidism, both of which can cause weight gain and generalized pain
- Depression, also associated with chronic pain
- Madelung syndrome, which leads to fat accumulation around the neck, upper limbs, and torso usually in non-overweight men who excessively consume alcohol
- Multiple familial lipomatosis, a genetic condition causing multiple fat lumps
- Proteus syndrome, a genetic disease causing overgrowth of various tissues
- Benign adipose tissue tumors, which are generally not painful but can present as multiple lumps on a patient
Fibromyalgia is worth mentioning in more detail as it can be misunderstood as adiposis dolorosa, especially in overweight or obese women. As fibromyalgia patients typically have no significant findings on a physical exam and lab tests, the diagnosis is made based on conditions such as general body pain, tender points in specific muscle and joint areas, fatigue, and mental and emotional distress.
Panniculitis, or the inflammation of fatty tissue, shows up as painful nodules under the skin. Specific types of panniculitis can be identified via a patient’s medical history, the location of nodules, and signs such as redness, ulceration, and scar-like tissue deformities. A biopsy can confirm the diagnosis.
Hormonal diseases that may resemble adiposis dolorosa include Cushing syndrome and hypothyroidism, which both entail weight gain and body-wide pain that is not restricted to the layer of fat beneath the skin. These symptoms make them distinguishable from adiposis dolorosa.
Sometimes, depression’s chronic pain resemblance to adiposis dolorosa can cause confusion.
Madelung syndrome, or multiple symmetric lipomatosis, results in large, symmetric, and nonpainful fat accumulation in usually non-overweight men with a history of alcohol abuse. These features make it distinct from adiposis dolorosa.
Multiple familial lipomatosis, a genetic condition where a mutation in a specific gene on chromosome 12 leads to the development of multiple lipomas, or fat lumps. As this disorder is hereditary, multiple family members are often affected.
Proteus syndrome is another genetic disease where an activating mutation in a distinct gene leads to lipomatosis, or fat accumulation, along with the overgrowth of various other tissues.
Finally, benign adipose tissue tumors, or harmless fat tissue tumors, are typically not painful but can occur as multiple lesions on one patient.
What to expect with Adiposis Dolorosa
Adiposis dolorosa, also known as Dercum’s disease, is a long-term condition that generally doesn’t get better over time. In fact, as time passes, it may worsen and become more disabling. However, it’s worth noting that different case studies have shown varied outcomes.
