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


• 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

Clinical Image of Angiokeratomas
Clinical image demonstrates numerous grouped angiokeratomas in a patient with Anderson-Fabry disease.

Angiokeratoma at Low Magnification
Angiokeratomas 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.

Angiokeratoma With Epidermal Acanthosis and Hyperkeratosis
The epidermis overlying angiokeratomas typically demonstrates acanthosis image and compact hyperkeratosis image. Numerous, dilated, superficial dermal blood vessels are also seen in this field.

Angiokeratoma at High Magnification
The 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.



• Ectatic superficial dermal blood vessels associated with reactive epidermal hyperplasia



• 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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