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Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Bones of the Lower Limb: Femur and Patella01:16

Bones of the Lower Limb: Femur and Patella

The femur is the body's longest and strongest bone spanning the thigh region. Its head articulates with the acetabulum of the hip bone to form the hip joint. A minor indentation on the medial side of the femoral head, called the fovea capitis, serves as the site of attachment for the ligament of the head of the femur. This weak ligament spans the femur and acetabulum and supports the hip joint. The narrowed region below the head is the neck of the femur. The inclination angle between the neck...

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En coup de sabre accompanied by pachydermoperiostosis: a case report.

M Ozdemir1, S Yildirim, I Mevlitoğlu

  • 1Department of Dermatology, Meram Medical Faculty, Selçuk University, Konya, Turkey. mustafaozdemir@yahoo.com

Clinical and Experimental Rheumatology
|June 5, 2007
PubMed
Summary

Scleroderma en coup de sabre, a localized scleroderma variant, co-occurred with pachydermoperiostosis in a young female. This rare association highlights potential shared pathways in connective tissue disorders.

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Area of Science:

  • Dermatology
  • Genetics
  • Rheumatology

Background:

  • Scleroderma en coup de sabre is a localized scleroderma variant.
  • Pachydermoperiostosis is a rare genetic disorder causing skin and bone changes.

Observation:

  • A 21-year-old female presented with scleroderma en coup de sabre and pachydermoperiostosis.
  • The patient had a family history of pachydermoperiostosis in her sister.
  • Diagnosis was confirmed via clinical examination, histology, and X-ray.

Findings:

  • Scleroderma en coup de sabre involves connective tissue fibrosis.
  • Pachydermoperiostosis is a hypertrophic process with periosteal and skin changes.
  • This case details the coexistence of these two distinct disorders.

Implications:

  • The co-occurrence suggests potential shared genetic or etiological factors.
  • Further research may elucidate underlying mechanisms linking these conditions.
  • Understanding this association can improve diagnosis and management of rare connective tissue diseases.