Pseudoxanthoma -elasticum-like syndrome Q82.8

Rongioletti F, 1992

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)

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).

Especially women beyond the age of 6

Lateral neck, intertriginal, abdominal, bilateral forearms,

• 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.

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

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