Adiposis dolorosa
Lipomatosis dolorosa (also: adiposis dolorosa, dolorosa obesity, Crohn's Dercum, fatty tissue rheumatism, Neurolipomatosis, Lipalgie ) is a disorder of adipose tissue.
First described it in 1888 by Francis Xavier Dercum (1856-1931 ).
Etiology
The cause of the disease is unknown. There are case reports of autosomal dominant Erbgängen. Conjecture (proven in some cases ) go from disturbances in the synthesis of C18 fatty acids, nerve inflammation, dysregulation of the nervous system or thyroid disorders to the hypothesis that the deposits of fat may exert pressure on the nerves.
Diagnostics
The diagnosis is made clinically, as a rule, and then fused histologically.
Epidemiology
Women are affected much more frequently than men, usually in middle age. Figures on incidence or prevalence are not available.
Symptoms
Typical are severe pain in the area of Fettgewebsablagerungen. These generally occur on the abdomen, knee, the elbow, on the buttocks and on the sides of the upper arms and thighs. Even with minimal contact and low pressure ( eg clothing) there is severe pain that increases in intensity over the course of years. In addition, occur several accompanying symptoms such as dry mouth, swelling, carpal tunnel syndrome or headaches.
Due to the symptoms, it also usually comes also to accompanying psychosocial problems.
Subtypes
There are three subtypes classified, which differ according to the location of the pain: juxta - articular, diffuse - generalized and nodular.
Therapy
A causative treatment is not possible. The pain is due to classical pain- treatment can not be influenced. Treatment of choice is intravenous therapy with lidocaine, which usually leads to a multi-week pain-free phase. Alternatively, mexiletine are given. Surgical options may be the removal and extraction of the fat.