- Adiposis dolorosa
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Adiposis dolorosa Classification and external resources ICD-10 E88.2 ICD-9 272.8 OMIM 103200 DiseasesDB 29660 eMedicine derm/839 MeSH D000274 Adiposis dolorosa, also known as Dercum's disease[1] is a rare condition characterized by multiple, painful lipomas. These lipomas mainly occur on the trunk, the upper arms and upper legs. The diagnosis of Dercum's disease implies a long, chronic pain syndrome of debilitating nature. The exact cause of Dercum’s disease is unknown.
Contents
History
Dercum's disease was originally described in the medical literature in 1892 by the American doctor Frances Xavier Dercum.[2]
Symptoms
Multiple painful lipomas is the cardinal symptom of this disease. The pain can be very intense and can be described as aching, stabbing, smarting or burning. The pain is chronic and progressive, but varies much in cycles.
Other common symptoms are:
- Various areas of the body may swell for no apparent reason. The fingers becomes clumsy, a person may drop things and sometimes the fingers go numb.
- Disturbed sleep, many have difficulties going to sleep because of the pain, but other forms of sleeping disorders also occur.
- General fatigue, worsening with even mild activity.
- Tendency to become black and blue; this seems to arise spontaneously or after alleviated blow.
- Stiffness after resting especially in the mornings.
- Skeletal pain in wrist, elbows, hips, tail bone and the long bones of the arms and legs.
- Headache, usually a combination between tension headache and classic migraine.
- Memory lapses and concentration difficulties making it difficult to learn new things and to accomplish intellectually demanding jobs.
- Feeling hot is often felt by the patients, some have 37.5 to 39 Celsius degree fever several weeks in a row, with increased pain and incapacity to work as a consequence.
- Tenderness under the feet, akin to walking on glass.
- Tenderness in the skin, difficulties in wearing tight fitting clothes or taking a shower.
- Infection sensitivity. Frequently increased pain during infections or active allergy attacks.
Causes
The cause of the disease is unknown and poorly researched. The symptoms and the lack of treatments can seriously affect the quality of life.
Diagnosis
A diagnosis of Dercum's disease is based on patient history and the physical findings. There are no specific laboratory test for this disease. Since the disorder does not show on the outside it can be hard for others to see the level of difficulties and discomfort the patient experiences. More than half of the patients are unable to work. The knowledge about the disorder is not well known and many patients are poorly treated. Some lab tests are currently being used to aid in the diagnosis but are not diagnostic themselves. Ultrasound and MRI are also useful in seeing the nodules found in visceral fat. Magnetic resonance imaging can play a role in diagnosis.[3]
Treatment
Treatment of Dercum's disease is usually targeted towards pain relief rather than removal. Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease. [4] There are not enough studies done to prove that diet and supplements could help with the disease.[5]
Treatment methods include:
Surgery
- Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases.[6] It may temporarily relieve symptoms although recurrences often develop.
Medication
- Fentanyl
- Cannabis
- Lyrica
- The use of infliximab and methotrexate has been proposed.[7]
- Intravenous infusions of the local anaesthetic drug lidocaine may give temporary relief from pain.[8] Additional treatments of lidocaine may be necessary periodically to sustain the effect.
- Another analgesic drug, peroral mexiletine, may also be effective in eliminating pain for variable periods of time.[9]
References
- ^ synd/1453 at Who Named It?
- ^ Dercum, F. X. Three cases of a hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special nervous symptoms: adiposis dolorosa. Am. J. Med. Sci. 104: 521-535, 1892.
- ^ Amine B, Leguilchard F, Benhamou CL (March 2004). "Dercum's disease (adiposis dolorosa): a new case-report". Joint Bone Spine 71 (2): 147–9. doi:10.1016/S1297-319X(03)00139-8. PMID 15050201. http://linkinghub.elsevier.com/retrieve/pii/S1297319X03001398.
- ^ [1] Natural Standard Research Collaboration
- ^ [2] Rowena Santos, MD
- ^ De Silva M, Earley MJ (June 1990). "Liposuction in the treatment of juxta-articular adiposis dolorosa". Ann. Rheum. Dis. 49 (6): 403–4. doi:10.1136/ard.49.6.403. PMC 1004110. PMID 2383065. http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=2383065.
- ^ Singal A, Janiga JJ, Bossenbroek NM, Lim HW (May 2007). "Dercum's disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate". J Eur Acad Dermatol Venereol 21 (5): 717. doi:10.1111/j.1468-3083.2006.02021.x. PMID 17448013. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0926-9959&date=2007&volume=21&issue=5&spage=717.
- ^ Devillers AC, Oranje AP (May 1999). "Treatment of pain in adiposis dolorosa (Dercum's disease) with intravenous lidocaine: a case report with a 10-year follow-up". Clin. Exp. Dermatol. 24 (3): 240–1. doi:10.1046/j.1365-2230.1999.00466.x. PMID 10354189. http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0307-6938&date=1999&volume=24&issue=3&spage=240.
- ^ Steiner J, Schiltz K, Heidenreich F, Weissenborn K (February 2002). "[Lipomatosis dolorosa--a frequently overlooked disease picture]" (in German). Nervenarzt 73 (2): 183–7. doi:10.1007/s00115-001-1240-9. PMID 11975097.
External links
- NORD (National Organization of Rare Disorders)
- Dercum's Disease (www.dercum.org)
Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215) Not otherwise specified (8800–8809) Connective tissue neoplasm Fibromatous (8810–8839)Fibroma/fibromatosis: Aggressive infantile fibromatosis · Aponeurotic fibroma · Collagenous fibroma · Diffuse infantile fibromatosis · Familial myxovascular fibromas · Fibroma of tendon sheath · Fibromatosis colli · Infantile digital fibromatosis · Juvenile hyaline fibromatosis · Plantar fibromatosis · Pleomorphic fibroma · Oral submucous fibrosisHistiocytoma/histiocytic sarcoma: Benign fibrous histiocytoma · Malignant fibrous histiocytoma · Atypical fibroxanthomaSolitary fibrous tumorMyxomatous (8840–8849)Myxoma/myxosarcoma (Cutaneous myxoma, Superficial acral fibromyxoma) · Angiomyxoma · Ossifying fibromyxoid tumourFibroepithelial (9000–9039)Synovial-like (9040–9049)Lipomatous (8850–8889) Chondroid lipoma · Intradermal spindle cell lipoma · Pleomorphic lipoma · Benign lipoblastomatosis · Spindle cell lipoma · HibernomaMyomatous (8890–8929) general: Myoma/myosarcomaskeletal muscle: Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma (Sarcoma botryoides) · Alveolar rhabdomyosarcomaLeiomyoma · Angioleiomyoma · Angiolipoleiomyoma · Genital leiomyoma · Leiomyosarcoma · Multiple cutaneous and uterine leiomyomatosis syndrome · Multiple cutaneous leiomyoma · Neural fibrolipoma · Solitary cutaneous leiomyomaComplex mixed and stromal (8930–8999) Adenomyoma · Pleomorphic adenoma · Mixed Müllerian tumor · Mesoblastic nephroma · Wilms' tumor · Rhabdoid tumour · Clear-cell sarcoma of the kidney · Hepatoblastoma · Pancreatoblastoma · CarcinosarcomaMesothelial (9050–9059) see also Template:Connective tissue
Categories:- Rare diseases
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