Insulin resistance syndrome, Type A

This code represents a rare genetic disorder characterized by severe insulin resistance, typically presenting in females. It involves a defect in the insulin receptor, leading to hyperinsulinemia, acanthosis nigricans, and often hyperandrogenism. Patients may also experience growth abnormalities and other endocrine dysfunctions.

Use this code when documentation explicitly states "insulin resistance syndrome, Type A" or "Rabson-Mendenhall syndrome." This diagnosis is typically made based on clinical presentation, genetic testing confirming insulin receptor mutations, and laboratory findings of severe hyperinsulinemia with normal or elevated glucose levels.

• Ensure documentation specifies "Type A" to differentiate from other insulin resistance syndromes.
• Do not confuse with general insulin resistance (R13.11) or other specific types of insulin resistance without the "Type A" designation.
• Code any associated manifestations, such as acanthosis nigricans (L83) or hyperandrogenism (E28.8), as secondary diagnoses.