Title: A Rare Case of ACROGERIA from Odisha

Authors: Sunil Kumar Agarwalla, Nasreen Ali

 DOI:  https://dx.doi.org/10.18535/jmscr/v6i4.65

Abstract

Acrogeria, Gottron type is a rare premature aging syndrome. Characteristic signs include fragile, thin skin on the hands and feet. Other parts of the body (e.g., face, forearms, and lower legs) are variably affected. It is generally considered to be a mild, non progressive skin atrophy due to the loss of the fatty tissue directly under the skin. Although most cases are sporadic, both autosomal recessive and autosomal dominant inheritance have been reported with a female predominance. Prognosis of these patients is good as they have no tendency to develop atheroma or diabetes mellitus. We report a case 11 year old female child of acrogeria for its rarity.

References

  1. Gottron H. Familiare acrogerie. Arch Dermatol Syphilogr. 1941;181:571–583.
  2. Gilkes J.J., Sharvill D.E., Wells R.S. The premature ageing syndromes. Br J Dermatol. 1974;91:243–262.
  3. Hjortshoj A., Heydenreich G. Acrogeria, case report. Dermatologica. 1977;154: 335–339. 
  4. NORD: National organization for Rare diseases
  5. Pope FM, Narcisi P, Nicholls AC, et al. (1996). "COL3A1 mutations cause variable clinical phenotypes including acrogeria and vascular rupture". Br J Dermatol. 135: 163–181.  doi:1046/j.1365-2133.1996.d01-971.x.
  6. Stolle C.A., Reed E., Pyeritz A., Myers J., Prockop D.J. Synthesis of an altered type III procollagen in a patient with type IV Ehlers–Danlos syndrome. A structural change in the alpha1 (III) chain which makes the protein more susceptible to proteinases. J Biol Chem. 1985;260:1937–1943. [PubMed]

Corresponding Author

Nasreen Ali

Junior Resident, Department of Pediatrics,

M.K.C.G Medical College, Berhampur, Ganjam, Odisha-760004, India