Angiokeratoma

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Angiokeratomas (purpura papulosa haemorrhagica Hebrae) are found in galactosialidosis such as alpha-galactosidase deficiency. They have also been described in Turner's syndrome. There is a systemic variety, called angiokeratoma corporis diffusum and rare forms such as angiokeratoma circumscriptum and angiokeratoma circumscriptum naeviforme (present at birth).

Contents

Appearance

One or several dark red papules, mostly with a verrucous surface

Pathology

Show ectasias of the papillary dermal blood vessels with secondary epidermal changes in the form of acanthosis and/or hyperkeratosis.

Classification

  1. Angiokeratoma of Mibelli
  2. Angiokeratoma of Fordyce
  3. Angiokeratoma corporis diffusum
    • Lesions are usually symmetrically distributed and are mostly concentrated between the umbilicus and knees
    • Commonest cause is alpha-galactosidase deficiency
  4. Angiokeratoma circumscriptum
  5. Solitary or multiple angiokeratomas, smaller than angiokeratoma circumscriptum

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