Probable Rabson-Mendenhall syndrome presenting with acanthosis nigricans: a case report.

Authors

Mira Akhras, Mohammad Hmaidan, Rahaf Ghazal, Asad Alebrahem, Nemat Alsaghir

Published in

Oxford medical case reports. Volume 2026. Issue 10. Pages omag199. Epub Oct 25, 2026.

Abstract

Rabson-Mendenhall syndrome (RMS) is a rare autosomal recessive disorder caused by mutations in the insulin receptor gene (INSR), resulting in severe insulin resistance. We report the case of an 8-year-old boy who presented with progressive acanthosis nigricans of the neck, coarse facial features, generalized hypertrichosis, dental abnormalities, a fissured tongue, thickened nails, penile hypertrophy, and hepatomegaly. Laboratory results indicated hyperinsulinemia, severe insulin resistance and fasting hypoglycemia. Based on the clinical and laboratory findings, Rabson-Mendenhall syndrome was considered. Genetic confirmation was not performed because it was unavailable in our country. This case highlights the importance of recognizing acanthosis nigricans as a clinical marker of inherited metabolic syndromes, and the important role of dermatologists in its early recognition.

PMID:
42928893
Bibliographic data and abstract were imported from PubMed on 26 Oct 2026.

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