Acquired generalized lipodystrophy

Acquired generalized lipodystrophy belongs to the group of mucopolysaccharidoses lysosomal storage diseases, and is associated with the loss of subcutaneous adipose tissue, especially in the face, arms, legs, palms and feet. Symptomatically, insulin resistance continues to develop with increased cardiovascular risk (diabetes mellitus).

Early symptoms (especially in infancy and childhood) are well-defined muscles with prominent superficial veins. Tonsils and adenoids may be hyperplastic, hands and feet are often enlarged, and umbilical hernia or similar umbilical abnormalities are often detected. Also hepatomegaly and splenomegaly occurs often. 

Dermatological manifestations include e.g. xanthomas, hirsutism, thick curled hair and acanthosis nigricans. Female patients often suffer from amenorrhoea, irregular menstruation, clitoromegaly, and infertility.

Triggers of the disease are not yet known, but are suspected to be infections such as measles, TB and hepatitis. Furthermore, the causal involvement of a genetic factor is assumed.

Three forms of Lawrence syndrome are distinguished:

- Form with panniculitis (inflammatory nodules) and subsequent lipatrophy
- Autoimmune form, often associated with hepatitis, Hashimoto's thyroiditis, hemolytic anemia, dermatomyositis and Sjogren's syndrome
- Idiopathic form.

The prognosis varies and is mainly influenced by the cardiovascular risk and the strength of further organ involvement.