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This is the full Polish name for a disease also known as Dercum’s disease. It is a rare disease, and one of its visible features is the formation of painful lipomas in various parts of the body.
Painful lipomatosis affects mainly postmenopausal, obese or overweight women. It also affects people with frequent family history of neoplastic diseases.
So far, scientists have not identified the causes of the disease. However, they suppose that its causes may be metabolic, lipid, carbohydrate, hormonal and immune disorders. Some scientists argue that inflammation and pro-inflammatory cytokines, which are involved in the control of fat and glucose metabolism, play an important role in the development of the disease.
Diagnosis
It is based on Brodowski’s criteria, developed in 1994. In order to establish the existence of Dercuma disease, four clinical features must be present simultaneously:
– painful lipomas in the subcutaneous tissue multiply and enlarge over time,
– generalized obesity,
– there is significant and unjustified (e.g. hard work) fatigue and weakness,
– emotional disturbances appear in the form of depression or other neurological symptoms.
According to experts from the World Health Organization, the diagnosis should be made when the patient is diagnosed with tumors of the subcutaneous tissue accompanied by severe pain.
Symptoms
Three types of lesions are characteristic for Dercum’s disease:
Periarticular changes – are subcutaneous, painful bumps appearing around the knee and hip joints, and rarely around the elbow joints.
Spilled changes – (generalized) is characterized by extensive, whole-body soreness on the dorsal surfaces of the torso, upper shoulders, armpits, buttocks, back, soles of the feet.
Nodular changes – lipomas may have a diameter of 0,5 to 4 cm, usually they are hemipipomas, classified as benign tumors. They are especially painful with even slight pressure. Patients are not obese.
Darcum’s disease is also accompanied by other symptoms that often prevent the patient from functioning normally and independently.
Pain is considered the most difficult to bear, disproportionately strong to the lipoma changes found on palpation. The pain is often burning or burning and appears symmetrically, e.g. in both hands, there is also a strong skin hyperalgesia, i.e. hypersensitivity to even the most delicate touch. Patients are rarely tormented by muscle and joint pain.
As we mentioned, lipomas can form all over the body. So far, they have not been found to form on the head and neck. The accompanying obesity is the result of metabolic disorders. Affected people cannot get rid of their excess weight, despite the use of even restrictive weight loss diets. The fatty tissue present in the disease stores water, making the body swollen, which increases the pain when touching the body.
The disease is also accompanied by neurological ailments. The most common are muscle fatigue, weakness, paresthesia, low-grade fever, headache, and epilepsy. Rest and sleep do not reduce discomfort. The sick also have a weak psyche. They often suffer from depression, mood swings, confusion, dementia and insomnia.
Endocrine disorders are manifested by hypothyroidism, Cushing’s disease (a set of symptoms resulting from long-term cortisol use, e.g. muscle weakness, acne, hirsutism, diabetes or glucose intolerance, thin skin). There is also arterial hypertension.
Differentiation of symptoms
Dercum’s disease is progressing steadily. No two cases are the same. But similar to Dercum’s disease, other disorders can manifest themselves. Therefore, making a correct diagnosis is not always easy. Therefore, the attending physician must exclude that the patient suffers from another dermatological disease: fibromyalgia, cellulitis, neurofibromatosis, progressive lipodystrophy, Cushing’s disease, myasthenia gravis, neoplasms, Madelung’s syndrome, Proteus syndrome, Fröhlich’s syndrome.
Treatment of ailment
There is no effective treatment. Non-steroidal anti-inflammatory drugs, painkillers are administered. Currently, paracetamol is the first-line drug, which is given first. The patient can be given lignocaine infusions. It is also necessary to take medications to alleviate neurological symptoms and psychological ailments. Local lymphatic drainage is also recommended. Surgical treatment is a last resort, and it is recommended only in the case of complications of the disease and the formation of necrotic lesions within the skin and deeper tissues. Sometimes patients undergo liposuction. Unfortunately, these are temporary measures, because after each treatment, changes in the subcutaneous tissue may not only recur, but also increase.