Lipomatosis dolorosa
| Classification according to ICD-10 | |
|---|---|
| E88.2 | Lipomatosis, not elsewhere classified - Lipomatosis dolorosa [Dercum's disease] |
| ICD-10 online (WHO version 2019) | |
Lipomatosis dolorosa (also: Adiposis dolorosa, Adipositas dolorosa, Dercum disease, adipose tissue rheumatism, neurolipomatosis, lipalgia) is a disease of adipose tissue.
It was first described in 1888 by Francis Xavier Dercum (1856–1931).
etiology
The cause of the disease is unknown. There are individual case reports of autosomal dominant inheritance. Assumptions (proven in individual cases) range from disorders in the synthesis of C18 fatty acids, inflammation of the nerves, dysregulation of the nervous system or thyroid disorders to the hypothesis that the fat deposits could exert pressure on the nerves.
Diagnosis
The diagnosis is usually made clinically and then confirmed histologically.
Epidemiology
Women get sick much more often than men, mostly in middle age. Figures on the incidence or prevalence are not available.
Symptoms
The strongest pain in the area of fatty tissue deposits is typical. These occur primarily on the abdomen, knees, elbows, buttocks and on the sides of the upper arms and thighs. Even with minimal touch and low pressure (e.g. from clothing), severe pain occurs, which increases in intensity over the years. In addition, various accompanying symptoms occur such as B. dry mouth, swelling, carpal tunnel syndrome or headache.
The symptoms also usually lead to accompanying psychosocial problems.
Subtypes
Three subtypes are classified, which differ according to the location of the pain: juxta-articular, diffuse-generalized, and nodular.
therapy
A causal treatment is not possible. The pain cannot be influenced by classic pain-modulating treatment. The method of choice is intravenous therapy with lidocaine , which usually leads to a pain-free period of several weeks. Alternatively, mexiletine can be given. Surgical options can include removal and suction of the fat.
Individual evidence
- ^ Roche Lexicon Medicine . 5th edition. Elsevier GmbH, Urban & Fischer Verlag, Munich 2003, ISBN 3-437-15072-3 (keyword: http://www.gesundheit.de/roche/index.html?c=http://www.gesundheit.de/ roche / ro22500 / r22538.004.html Lipomatosis dolorosa).
