II. Epidemiology

  1. Prevalence: <1% (U.S.)
  2. More common in white patients
  3. Age of onset >40 years

III. Symptoms

  1. Typically asymptomatic
  2. May rarely bleed or be pruritic

IV. Signs

  1. Red-blue, benign vascular Papules
  2. Typically 1-2 mm in size (up to 6 mm)
  3. Distribution
    1. Clustered on the glans penis, often linear along the margin
    2. May occur on Scrotum, groin, thighs, and abdominal wall
  4. Red Flag: Lesions on the penile shaft, suprapubic region or Sacrum
    1. Suggests Fabry Disease and requires referral (see below)

V. Differential Diagnosis

  1. Pearly Penile Papules
  2. Penile Cancer
  3. Fabry Disease (Lysosomal Storage Disease)
    1. May present with Angiokeratomas on the penile shaft, suprapubic region or Sacrum
    2. Fabry Disease is associated with Renal Failure, Cardiomyopathy, Hypertension, Anhidrosis and neuropathic pain
    3. Requires urgent Consultation

VI. Management: Symptomatic Cases (e.g. Bleeding)

  1. Electrocautery
  2. Cryotherapy
  3. Laser ablation
  4. Surgical excision

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