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Watercolor of the histology of an angiokeratoma, from Fabry's original article (1898)

"Hematoxylin staining. Cutis papillae dark blue, almost violet, epidermis light blue, blood foci greenish-yellow, section through a larger nodule, near the blood papules the epidermal papillae are elongated. Some blood dropped out. The hemorrhage is divided into several chambers by septa of an epithelial nature; low-grade parakeratosis; in the Curls foci of small cell infiltration." (Source: Fabry J. Ein Beitrag zur Kenntniss der Purpura haemorrhagica nodularis [Purpura papulosa haemorrhagica Hebrae]. Archiv für Dermatologie und Syphilis 1898;43:187-200.)

Associated Disorders

  • Atypical heterozygotes
  • Autonomic neuropathy
  • Cerebral infarction
  • Glycosphingolipidosis
  • Hypertrophic cardiomyopathy
  • Myocardial infarction
  • Renal failure