progressive lipodystrophy(redirected from Simons disease)
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.
a condition characterized by a complete loss of the subcutaneous fat of the upper part of the torso, the arms, neck, and face, sometimes with an increase of fat in the tissues about and below the pelvis.
acquired partial lipodystrophyA rare, acquired, partial lipodystrophy of unknown aetiology and onset during childhood, eventually leading to complete loss of subcutaneous fat in the face, neck, trunk and upper limbs. It is more common in females, and may be associated with glomerulonephritis, diabetes, hyperlipidaemia and complement deficiency.
Barraquer Roviralta,Luis, Spanish physician, 1855-1928.
Barraquer disease - a condition characterized by a complete loss of the subcutaneous fat of the upper part of the torso, the arms, neck, and face. Synonym(s): Barraquer-Simons disease; progressive lipodystrophy; Simons disease
Barraquer-Simons disease - Synonym(s): Barraquer disease