| • | a group of benign vascular neoplasms in which marked dilated, thin walled vessels are situated beneath a hyperplastic and hyperkeratotic epidermis |
| • | blue-black hyperkeratotic vascular papules |
FIVE TYPES:
| 1. | Localized Angiokeratomas |
| • | usually solitary and found on an extremity |
| 2. | Angiokeratoma circumscriptum |
| • | Presents at birth as unilateral plaques on an extremity |
| 3. | Angiokeratoma of Mibelli |
| • | Develops in childhood or adolescence |
| • | Over the dorsal surface of the hands and feet |
| 4. | Angiokeratoma of Fordyce |
| • | Most commonly seen on the scrotum |
| 5. | Angiokeratoma corporis diffusum (Fabry’s disease): |
| • | lack enzyme ceramide trihexosidase (aka alpha-galactosidase) |
| • | ceramide trihexoside accumulates in vascular epithelium (à vessels become ectatic = lesion) |
| • | deposit in renal tubules à malignant hypertension |
| • | Multiple skin lesions between umbilicus and knees |
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