Angiokeratoma

 Usually on extremities

• Angiokeratoma of Fordyce
image Elderly men
image Multiple lesions, often on scrotum
• Angiokeratoma of Mibelli
image Childhood
image Warty lesions over bony prominences of acral locations
• Angiokeratoma corporis diffusum
image Associated with Anderson-Fabry disease
• Angiokeratoma circumscriptum
image Presents in children
image Least common subtype

Microscopic

• Marked ectasia of papillary dermal blood vessels
• Vessels herniate into epidermis, resulting in appearance of intraepidermal vessels
• Hemorrhage in stratum corneum common
• May have evidence of thrombosis and intravascular papillary endothelial hyperplasia
• Reactive epidermal changes: Acanthosis, hyperkeratosis, and elongation of rete ridges around ectatic vessels

Top Differential Diagnoses

• Verrucous hemangioma
image Verrucous epidermal hyperplasia
image Superficial and deep vascular proliferation
• Pyogenic granuloma (lobular capillary hemangioma)
image Polypoid lesion with epidermal collarette
image Granulation tissue-like dermal vascular proliferation
image
Clinical Image of AngiokeratomasClinical image demonstrates numerous grouped angiokeratomas in a patient with Anderson-Fabry disease.

image
Angiokeratoma at Low MagnificationAngiokeratomas are characterized by ectatic vessels image in the superficial dermis that appear to herniate into the epidermis. There is often overlying hemorrhage image, epidermal acanthosis, and frequently compact hyperkeratosis.
image
Angiokeratoma With Epidermal Acanthosis and HyperkeratosisThe epidermis overlying angiokeratomas typically demonstrates acanthosis image and compact hyperkeratosis image. Numerous, dilated, superficial dermal blood vessels are also seen in this field.
image
Angiokeratoma at High MagnificationThe vessels of angiokeratoma herniate up into the epidermis image. The vessels frequently undergo thrombosis, and in this case demonstrates an early thrombus characterized by fibrin image admixed with erythrocytes.

TERMINOLOGY

Definitions

• Ectatic superficial dermal blood vessels associated with reactive epidermal hyperplasia

CLINICAL ISSUES

Presentation

• 5 clinical variants
image Solitary and multiple angiokeratoma
– Usually solitary
– Multiple lesions may have zosteriform presentation
– Wide age range

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiokeratoma

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