Pseudoxanthoma -elasticum-like syndromeQ82.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Pseudoxanthoma elasticum-like papillary dermal elastolysis; PXE-like PDE

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HistoryThis section has been translated automatically.

Rongioletti F, 1992

DefinitionThis section has been translated automatically.

Disease of the collagenous connective tissue of the skin that appears in the 6th or 7th decade of life, reminiscent of a pseudoxanthoma elasticum, but shows an almost complete loss of the elastic fibre material (Rongioletto F et al. 1992), but does not show any manifest systemic changes (see below)

EtiopathogenesisThis section has been translated automatically.

Unexplained; intrinsic skin aging or UV radiation is discussed; associations of the disease with hematological disorders such as thalassemia (D56.9) and sickle cell disease (D57.1) are described (Fabbri E et al. 2009; Hassan S et al. 2010).

ManifestationThis section has been translated automatically.

Especially women beyond the age of 6

LocalizationThis section has been translated automatically.

Lateral neck, intertriginal, abdominal, bilateral forearms,

Clinical featuresThis section has been translated automatically.

  • Regionally limited, round or oval, symmetrically arranged, striped, spotted or hearth-shaped, slightly raised, soft, possibly confluent papules. Colour: white to yellowish, "ivory". In the "mature" stage of development, yellowish papules and plaques with a granular surface relief reminiscent of plucked chicken skin (also cobblestone-like bumps).
  • In contrast to Pseudoxanthoma elasticum, there are no changes in the oral mucosa.
  • No other organ diseases.

HistologyThis section has been translated automatically.

Thickening, clumping or focal loss of elastic fibers in the papillary dermis.

LiteratureThis section has been translated automatically.

  1. Fabbri E et al (2009) Pseudoxanthoma-elasticum-like syndrome and thalassemia: an update. Dermatol Online J 15:7
  2. Hassan S et al (2010) Pseudoxanthoma elasticum-like syndrome in a patient with sickle cell anaemia. Br J Haematol 148: 342
  3. Meyer S et al (2005) Elastosis perforans serpiginosa-like pseudoxanthoma
  4. elasticum in a child with severe Moya Moya disease. Br J Dermatol 153:431-434
  5. Revelles JM et al (2012) Pseudoxanthoma elasticum-like papillary dermal elastolysis: immunohistochemicalstudy
    using elastic fiber cross-reactivity with an antibody against amyloid Pcomponent
    . At J Dermatopathol 34:637-643.
  6. Rongioletti Fet al. (1992) Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol 6: 648-650

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer