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Disorders of elastic fibres

5  Non-tumorous skin diseases

5.9  Disorders of elastic fibres

5.9.1  Anetoderma

Clinical signs:

  • affects especially upper trunk
  • macular atrophy
  • soft, slightly elevated areas, palpable

Histology:

Slight or moderate dermal inflammation, accompanied by fragmentation of elastic fibres.

5.9.2  Mid dermal elastolysis

Clinical signs:

  • very rare condition
  • sharply demarcated areas of wrinkling of the skin

Histology:

Absence of elastic fibres in the mid dermis, no inflammation.

5.9.3  Actinic degeneration of the corium

Clinical signs:

  • atrophic, yellowish, wrinkled, coarse or facetated skin
  • affects insolated areas, the changes are irreversible
  • location: face, back and lateral aspects of the neck
  • common in elder people, especially of the facial skin
  • senile comedones
  • epidermal cysts
  • thickening on nuchal skin: cutis rhomboidalis nuchae

Histology:

Degeneration of corium characterized by deposits of amorphous material in upper dermis (pink gray in HE staining, stains dark in orcein stains for elastic fibres)

5.9.4  Pseudoxanthoma elasticum

Introduction:

Genetically determined disorder of elastic fibres

Clinical signs:

  • starts at second or third decade, worsens with age
  • wrinkled skin
  • soft papules
  • affects eyes, arteries

Histology:

Masses of irregularly clumped elastic fibres, sometimes granulomatous reaction and rarely transepidermal elimination of degenerated elastic fibres.

5.9.5  Focal linear elastosis

Clinical signs:

  • rare disorder
  • linear striae, palpable
  • location: back, symmetric

Histology:

Coarse elastic fibers in small clumps in the reticular dermis (stain for elastic fibres is necessary).

5.9.6  Elastosis perforans serpiginosa

Clinical signs:

  • rare disease
  • types:
    • primary (cause unknown)
    • secondary (Ehlers Danlos, Marfan, Down syndromes and other)
    • drug induced
  • age: any (often young persons, sometimes children)
  • papules, sometimes serpiginous; red; size: several milimeters, up to several centimeters
  • papules have central depression, sometimes foreign material may get extruded

Histology:

Accumulation of fragmented elastotic material within the papillary corium; transcutaneous elimination of elastotic fibres.


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