Dercum disease


Dercum disease

Description, Causes and Risk Factors:

Alternative Name: Adiposis dolorosa, Anders' syndrome, Dercum-Vitaut syndrome, lipalgia, adiposalgia, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome.

ICD-10: E88.2

A chronically progressive, symmetrical accumulation of fat in the subcutaneous tissue with orthostatic edema occurring almost exclusively in women. Primarily the lower extremities are affected. Dercum disease is characterized by tenderness and easy bruising. The rare cases in which men are affected involve severe hormonal functional disorders such as testosterone deficiency.

Dercum disease

Stage:

    Stage I: Skin surface normal, nodular (small) fatty tissue structure.

  • Stage II: Skin surface uneven (peau d'orange), nodular (big) fatty tissue structure.

  • Stage III: Lobular deformation due to increased fatty tissue.

The exact cause of dercum disease remains unknown. The origin of the pain is obscure, and the disease is better known as a description of its symptoms rather than as a physiologic or metabolic process. The fatty deposits (lipomas) cause nerve compression and result in weakness and pain.

Some reported cases have suggested possible causes for Dercum disease, such as the use of corticosteroids, a disturbance of endocrine function, or a genetic cause since it seems to run in some families. Some researchers have suggested that Dercum disease is an autoimmune disorder. However, no single cause has been pinpointed.

Symptoms:

This condition consists of four cardinal symptoms:

    Multiple, painful, fatty masses.

  1. Generalized obesity, usually in menopausal age.

  2. Weakness and tiredness.

  3. Mental disturbances, including emotional instability, depression, epilepsy, confusion and dementia

Other symptoms may include:

    Hematemesis.

  • Early epistaxis.

  • Early menopause.

  • Slight pigmentation of the skin.

  • Atrophy of the muscle.

Diagnosis:

The differential diagnosis may include fibromyalgia, other multiple lipoma syndromes such as familial symmetric lipomatosis, Proteus syndrome, MERRF syndrome with lipomatous lesions, neurofibromatosis type 1 (NF1) and multiple endocrine neoplasia type 1.

There are no definitive tests for Dercum's Disease at this time; all diagnosis will be made clinically through a process of elimination.

Diagnosis of Dercum disease is based onclinical features and can be supported by otherdiagnostic methods like histopathology.Computed tomography (CT) and magnetic resonance imaging (MRI)can be used to assess the extent and location of fat deposits in Dercum disease. Ultrasound can also be used to assess quantitative andqualitative aspects of the fatty tissue. While healthy subcutaneous tissuetends to be of low echogenicity, in Dercum diseasepatients it is homogenously thickened, with higher echogenicity in color Doppler images.

Treatment:

There is no known 'cure' for Dercum disease. At the present time, treatment for this condition is symptomatic, meaning that it focuses on one symptom at a time rather than the whole condition. Depending on the person, options can include weight reduction, surgery for the most painful lipomas, and medications to control pain. Liposuction has been used in some cases.

Pain may be relieved by intravenous administration of lidocaine, local corticosteroid (prednisone) injections and combinations of mexiletine and amitriptyline, or infliximab and methotrexate.

Conservative therapy aims to reduce the volume of interstitial fluid. In very early stages, orthostatic edema can be prevented by wearing compression garments. Combined decongestive therapy (CDT) is used for edema that is no longer spontaneously reversible.

It is highly recommended that painful and pressing lipomas that may develop be surgically removed, especially if they are causing tingling or numbness.

The one most successful treatment to address the root cause of the symptoms of Dercum's Disease is liposuction. When the abnormal Dercum's fat has been removed via liposuction, patients report that many of their symptoms subside greatly and for a prolonged period of time, thus dramatically increasing the quality of life.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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