Acrogeria

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Acrogeria
Other namesAcrogeria, Gottron type, Gottron's syndrome[1]
Fibers of Collagen Type I - TEM
SpecialtyDermatology

Acrogeria (Gottron's syndrome) is a skin condition characterized by premature aging, typically in the form of unusually fragile, thin skin on the hands and feet (distal extremities).[1] The prefix "acro" stems from the Greek akros which alludes to "extremity, tip" while the suffix "geria" comes from the Greek gerôn which means "elder".[citation needed]

This is one of the classic

Werner's syndrome) and progeria (Hutchinson–Gilford's syndrome), and was characterized in 1940.[2] Acrogeria was originally described by Gottron in 1941, when he noticed premature cutaneous aging localized on the hands and feet in two brothers. The problem had been present since birth.[2]

Onset is often in early childhood, it progresses over the next few years and then remains stable over time with morphology, colour and site remaining constant. A bruising tendency has been observed.[3] Mutations in the COL3A1 gene, located at chromosome 2q31–q32, have been reported in varied phenotypes, including acrogeria and vascular rupture in Ehlers-Danlos' syndrome (more especially type IV).[4]

See also

  • Hutchinson–Gilford syndrome
  • List of cutaneous conditions

References

  1. ^ .
  2. ^ a b Gottron, H. Familiaere Akrogerie. Arch. Derm. Syph. 181: 571-583, 1940.
  3. PMID 10694296
    .
  4. .

External links