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. 2014 Nov;59(6):633.
doi: 10.4103/0019-5154.143579.

Rabson-mendenhall syndrome

Iffat Hassan  1 , Hinah Altaf  1 , Atiya Yaseen  1
Affiliations

Rabson-mendenhall syndrome

Iffat Hassan et al. Indian J Dermatol. 2014 Nov.

Abstract

Rabson-Mendenhall syndrome (RMS) is a rare genetic disorder characterized by growth retardation, dysmorphisms, lack of subcutaneous fat, acanthosis nigricans, enlarged genitalia, hirsutism, dysplastic dentition, coarse facial features, abnormal glucose homeostasis, hyperinsulinemia and pineal hyperplasia. Herein, we describe a 13-year-old girl with physical features of RMS who presented to us on account of acanthosis nigricans.

Keywords: Acanthosis nigricans; Rabson-Mendenhall syndrome; hirsutism; hyperinsulinemia.

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Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Acanthosis nigricans seen extending beyond the neck
Figure 2
Figure 2
Fissured tongue with overcrowding of teeth
Figure 3
Figure 3
Abdominal distension with loss of subcutaneous fat
Figure 4
Figure 4
Clitoromegaly
Figure 5
Figure 5
Premature graying of hair
See this image and copyright information in PMC

References

    1. Rabson SM, Mendenhall EN. Familial hypertrophy of pineal body, hyperplasia of adrenal cortex and diabetes mellitus; report of 3 cases. Am J Clin Pathol. 1956;26:283–90. - PubMed
    1. West RJ, Lloyd JK, Turner WM. Familial insulin resistant diabetes, multiple somatic anomalies, and pineal hyperplasia. Arch Child Dis. 1975;50:703–8. - PMC - PubMed
    1. Longo N, Singh R, Griffin LD, Langley SD, Parks JS, Elsas LJ. Impaired growth in Rabson-Mendenhall syndrome: Lack of effect of growth hormone and insulin-like growth factor I. J Clin Endocrinol Metab. 1994;79:799–805. - PubMed
    1. Longo N, Singh R, Elsas LJ. Decreased half-life of insulin-like growth factor I in Rabson-Mendenhall syndrome. J Inherit Metab Dis. 2001;24:546–50. - PubMed
    1. Alaei Mohammad Reza, Mir Javadi Seyed Alirez, Shiraz I. Rabson Mendenhall Syndrome: A case report. Iran J Child Neurol. 2010;4:1.

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