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Olmsted syndrome.

C S Sirka1, M Ramam, R Mittal

  • 1Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi - 110029, India.

Indian Journal of Dermatology, Venereology and Leprology
|October 6, 2010
PubMed
Summary
This summary is machine-generated.

Olmsted syndrome, a rare skin disorder, involves palmoplantar keratoderma and alopecia. Acitretin treatment significantly improved a patient's symptoms, demonstrating its therapeutic potential for this condition.

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Area of Science:

  • Dermatology
  • Genetics

Background:

  • Olmsted syndrome is a rare genetic disorder characterized by palmoplantar keratoderma, periorificial keratotic plaques, alopecia, and contractures.
  • It is often associated with mutations in the TRPV3 gene.

Purpose of the Study:

  • To report a case of Olmsted syndrome in a 20-year-old Sikh man.
  • To evaluate the efficacy of acitretin in managing the clinical manifestations of Olmsted syndrome.

Main Methods:

  • Clinical diagnosis of Olmsted syndrome based on characteristic features.
  • Treatment with oral acitretin, initiated at 25mg/day and adjusted over a 6.5-month period.

Main Results:

  • The patient presented with palmoplantar keratoderma, flexion deformities, universal alopecia, and keratotic plaques.
  • Significant clinical improvement was observed following acitretin therapy.

Conclusions:

  • Acitretin is an effective treatment option for managing the symptoms of Olmsted syndrome.
  • This case highlights the importance of timely diagnosis and therapeutic intervention for rare dermatological conditions.