Type I is characterized by the gradual
disappearance of most subcutaneous adipose tissue from the lower extremities
and gluteal areas at puberty. Muscles and superficial veins become prominent
in these areas. In type II, there is also disappearance of adipose tissue
from the upper extremities and trunk, with simultaneous accumulation of
adipose tissue on the face and neck, giving a cushingoid appearance. Adipose
tissue may accumulate in the axillae, back, labia majora, and intraabdominal
region. Acanthosis nigricans and hirsutism occur infrequently in both types.
Females may have menstrual abnormalities as a result of polycystic ovaries.
After the age of 20 years, affected patients become insulin resistant and may
develop glucose intolerance or type II diabetes mellitus. Lipid
abnormalities include hypertriglycideridemia and low levels of high-density
lipoprotein (HDL) cholesterol. Hypertriglyceridemia may result in acute pancreatitis.
Affected patients have a higher incidence of atherosclerosis leading to hypertension and
coronary artery disease. Women have a higher incidence of atherosclerosis and diabetes
mellitus than men. Females are more likely to be diagnosed because of the relative
muscularity and reduced body fat in normal males. Characterization of affected or
unaffected status is not possible in prepubertal children. In type III, insulin-dependent
diabetes mellitus and severe hypertension have been reported.