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