Lawrence syndromeE88.1
HistoryThis section has been translated automatically.
Lawrence, 1946
DefinitionThis section has been translated automatically.
Acquired form of generalized lipodystrophy with atrophy of subcutaneous fatty tissue, hepatomegaly and insulin-resistant diabetes mellitus following infection or brain tumors.
ManifestationThis section has been translated automatically.
Usually begins in childhood at around 5 years of age, sometimes not until or after puberty. The female sex is more frequently affected.
LocalizationThis section has been translated automatically.
Face, trunk, extremities.
Clinical featuresThis section has been translated automatically.
Clinically, there are circumscribed lipodystrophies with loss of adipose tissue in the face, body and extremities. See also Lipodystrophy, generalized. Hypertrichosis, macroglossia, acanthosis nigricans, eruptive xanthomas, phlebectasias, diencephalic astrocytomas, cerebral focal symptoms, diabetes insipidus.
TherapyThis section has been translated automatically.
Treatment of the underlying disease, otherwise symptomatic according to the clinical appearance.
Progression/forecastThis section has been translated automatically.
Variceal bleeding; early death with liver failure.
LiteratureThis section has been translated automatically.
- Lawrence RD (1946) Lipodystrophy and hepatomegaly with diabetes, lipaemia, and other metabolic disturbances: A case throwing new light on the action of insulin. Lancet I: 724-775
- Magre J et al (2003) Prevalence of mutations in AGPAT2 among human lipodystrophies. Diabetes 52: 1573-1578
- Mahmoud SF (1997) Lawrence-Seip syndrome: report of a case from Egypt. Cutis 60: 91-93
- Sollberg S (1987) Generalized lipodystrophy (Seip-Lawrence syndrome) of the congenital-hereditary type. Act Dermatol 13: 165-168