Rabson-Mendenhall Syndrome: Two Case Reports and a Brief Review of the Literature

The document reports on two cases of Rabson-Mendenhall syndrome (RMS), a rare genetic disorder characterized by severe insulin resistance and various physical abnormalities. The first case involves a 4.5-year-old girl with symptoms such as acanthosis nigricans, hirsutism, and precocious puberty, who showed skin improvement with metformin treatment despite high blood sugar levels. The second case is her 16-year-old sister with stunted growth, insulin-resistant diabetes, hyperaldosteronism, and bilateral nephromegaly. Both cases had abnormal dentition and faced socioeconomic barriers to dental care. The paper highlights the importance of oral examinations in RMS patients and potential dental treatments. It also discusses the role of insulin receptor mutations in RMS and the lack of specific treatments, noting that recombinant human IGF-I therapy and metformin have shown some benefits. The document underscores the need for novel therapies to address the underlying defects in insulin resistance syndromes like RMS.