How are GL and PL classified?

The disease is classified in 4 main groups. First, there is the division between the fat being absent in the whole body (generalised) and only in certain parts of the body (partial). Both forms are then also subdivided into congenital (familial) and acquired. GL and PL are very rare and occur only in 1 and 3 people per million for GL and PL respectively. The familial form has a number of genetic causes. The cause of the acquired forms is not yet understood. There is a possible connection with immune reactions of the body to its own tissue.

Classification of GL and PL

Subtypes and characteristics of the generalised lipodystrophy

 

Congenital generalised lipodystrophy
(Berardinelli-Seip Syndrome)

Acquired generalised lipodystrophy
(Lawrence Syndrome)


            Little girl with generalised lipodystrophy and typical lack of subcutaneous fat

Raeya, congenital lipodysthrophy GL

Appearance


            Illustration of disturbed fat distribution which shows the total lack of subcutaneous body fat

Lack
of fat

Variable fat
accumulation


            Illustration of disturbed fat distribution which shows the total lack of subcutaneous body fat

Loss
of fat

Variable fat
accumulation

Average age of
occurrence 

0.3 years
(range 0.0-12.0 years )

5 years
(range 0.0-15.0 years)

Sex ratio
(male : female)

1:1-2

1:3

Subtypes and characteristics of the partial lipodystrophy

 

Congenital (familial) partial lipodystrophy
(Dunnigan or Köbberling Syndrome)

Acquired partial  lipodystrophy
(Barraquer-Simons Syndrome)


            Woman with congenital partial lipodystrophy and typical lack of subcutaneous fat combined with fat accumulation around the neck.

Linda, familial
lipodysthrophy PL

Appearance


            Illustration of disturbed fat distribution which shows the partial lack of subcutaneous body fat

Loss
of fat

Variable fat
accumulation


            Illustration of disturbed fat distribution which shows the partial lack of subcutaneous body fat

Loss
of fat

Variable fat
accumulation

Average age of
occurrence

9.9 years
(range 0.0-16.0 years)

8.2 years
(range 0.5-16.0 years)

Sex ratio
(male : female)

1:1-2

1:4

Collage of seven people with GL and PL, male and female, of varying ages and appearances

The appearance of patients with GL and PL can vary greatly.
Patients with GL are often very thin and mostly have a muscular appearance. With PL, especially the familial form, the loss of subcutaneous fat may not be so obvious, especially because there can be increased fat accumulation in other parts of the body. In the acquired form of PL, fat loss is gradual and develops from the head down towards the lower stomach. However, there can be fat accumulation in the buttock, hip and leg area.
The typical “saddlebags” appearance develops.