Lawrence Syndrome
| Classification according to ICD-10 | |
|---|---|
| E88.1 | Lipodystrophy, not elsewhere classified |
| ICD-10 online (WHO version 2019) | |
The Lawrence's syndrome is one of the non-familial syndromes lipodystrophen and by a loss of adipose tissue (especially in subcutaneous face, arms, legs, palms and soles of the feet), and an insulin resistance associated. Hence, it is also a rare cause of diabetes mellitus and a cardiovascular risk factor.
Synonyms are: Acquired Generalized Lipodystrophy; Diabetes, acquired lipoatrophic; Lawrence Seip Syndrome
The name refers to the author of the first description from 1946 by R. D. Lawrence.
In contrast to the Berardinelli type lipodystrophy , it is not a congenital, but an acquired form of lipodystrophy.
distribution
The frequency is given as 1–9 per 100,000 people, over a hundred people worldwide are affected. The female sex is affected more often in a ratio of 3 to 1.
causes
The triggers are not yet known; infections such as measles and hepatitis are suspected to be triggers.
Classification
Three forms of the disease have been described:
- Form with panniculitis and subsequent lipatrophy
- Autoimmune form , often with hepatitis , Hashimoto's thyroiditis , Hemolytic anemia , dermatomyositis and Sjogren's syndrome associated
- Idiopathic form
Clinical manifestations
Clinical criteria are:
- secondary lip atrophy in children, adolescents, or later
- Cravings and Accelerated Growth in Adolescence
- in one third acanthosis nigricans and polycystic ovary syndrome
- Hepatomegaly with fatty liver
diagnosis
When blood tests fall hyperinsulinemia and diabetes mellitus with insulin resistance on, often hypertriglyceridemia and decreased leptin and adiponectin in blood plasma . Furthermore, proteinuria and disorders of production of growth hormones occur.
Differential diagnosis
Must be distinguished are other forms of extreme insulin resistance as Rabson-Mendenhall syndrome , Leprechaunism , congenital generalized lipodystrophy and insulin resistance syndrome type A and insulin resistance syndrome type B .
therapy
The therapy of the disease is aimed particularly at the metabolic symptoms (insulin resistance), especially since their prognosis is probably determined by the occurrence of cardiovascular diseases.
Web links
Individual evidence
- ↑ a b c d e f g Lipodystrophy, acquired generalized. In: Orphanet (Rare Disease Database).
- ^ RD Lawrence: Lipodystrophy and Hepatomegaly with Diabetes, Lipemia and Other Metabolic Disturbances . In: The Lancet , Volume 1, 1946, pp. 724-731.
- ↑ Lawrence Syndrome. German Diabetes Foundation
