Derm

Angiokeratoma

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Angiokeratoma, Angiokeratoma of the Glans Penis, Fabry Disease, Angiokeratoma of Fordyce, Angiokeratoma of Mibelli, Angiokeratoma circumscriptum

  • See Also
  • Epidemiology
  1. Prevalence: <1% (U.S.)
  2. More common in white patients
  3. Age of onset >40 years
  • Pathophysiology
  1. Benign vascular lesion
  • Causes
  1. Sporadic onset in most cases
    1. Differentiate from Fabry Disease, which is X-Linked
  2. Enoxaparin
    1. Rare adverse effect
  • Symptoms
  1. Typically asymptomatic
  2. May rarely bleed or be pruritic
  • Signs
  1. Characteristics
    1. Red-blue to black, benign vascular Papules
    2. Typically 1-2 mm in size (up to 6 mm)
  2. Dermoscopy
    1. Dark lacunae
    2. White veil
    3. Keratinization
    4. Henmorrhagic cysts
  3. Distribution: Clustered Genitourinary Lesions (Angiokeratoma of Fordyce)
    1. Clustered on the glans penis (or Vulva in women), often linear along the margin
    2. May occur on Scrotum, groin, thighs, and abdominal wall
  4. Solitary Angiokeratoma
    1. Single, isolated benign lesions that typically require no treatment
  5. Distribution: Palms and Soles in Children (Angiokeratoma of Mibelli)
    1. Multiple acral (palms/soles) verrucous (warty) Angiokeratomas in children
  6. Distribution: Leg lesions in newborns (Angiokeratoma circumscriptum)
    1. Congenital clustered lesions present at birth, typically on the lower extremities
  7. Red Flag: Lesions on the penile shaft, suprapubic region or Sacrum
    1. Suggests Fabry Disease and requires referral (see below)
  1. Pearly Penile Papules
  2. Penile Cancer
  3. Fabry Disease (Lysosomal Storage Disease)
    1. Presents with multipls Angiokeratomas on the penile shaft, suprapubic region, thighs, buttocks or Sacrum (bathing suit distribution)
    2. Fabry Disease is associated with Renal Failure, Cardiomyopathy, Hypertension, Anhidrosis and neuropathic pain
    3. Requires urgent Consultation
  • Management
  • Symptomatic Cases (e.g. Bleeding)
  1. Electrocautery
  2. Cryotherapy
  3. Laser ablation
  4. Surgical excision