An extremely rare genetic disorder causing severe deficiency of insulin resistance. Characterized by growth retardation and small stature, dysmorphic facies, lipoatrophy, hirsutism, enlarged genitalia, and muscular hypotrophy. Distinctive facial features include low-set ears, thick lips, hypertelorism, bulging eyes, and flaring nostrils. Treatment with recombinant insulin-like growth factor 1 (IGF1) can be considered, though life expectancy typically does not exceed 2 years.
ICD10CM: E34.8 – Other specified endocrine disorders