lipodystrophy

1. any disturbance of fat metabolism.

2. a group of conditions due to defective metabolism of fat, resulting in absence of subcutaneous fat; they may be congenital or acquired and partial or total. (See Atlas 2, Part F.)

congenital generalized lipodystrophy an autosomal recessive condition marked by the virtual absence of subcutaneous adipose tissue, large body size, splenomegaly, hirsutism, acanthosis nigricans, and reduced glucose tolerance in the presence of high insulin levels.

intestinal lipodystrophy former name for Whipple's disease.

partial lipodystrophy a condition seen mainly in females in the first decade of life, characterized by symmetrical loss of subcutaneous fat, usually beginning on the face and gradually extending to the chest, neck, back, and upper limbs; this gives the lower part of the body an apparent, and possibly real, adiposity of the buttocks and lower limbs. Some affected patients develop insulin-resistant diabetes mellitus, triglyceridemia, and renal disease.

progressive lipodystrophy progressive and symmetrical loss of subcutaneous fat from the parts above the pelvis, facial emaciation, and abnormal accumulation of fat about the thighs and buttocks.

total lipodystrophy an autosomal recessive disorder occurring mainly in females, characterized by a generalized loss of subcutaneous fat and extracutaneous adipose tissue, present at birth or appearing later in life, and associated with hepatomegaly with abdominal protuberance, hypoglycemia and insulin-resistant nonketotic diabetes, hyperlipemia, marked elevation of the basal metabolic rate, accelerated somatic growth, advanced bone age, acanthosis nigricans and hirsutism.