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Vascular and Lymphatic Neoplasms

»Which is the most common benign vascular neoplasm of childhood?
»What are the clinical subtypes of HOI?
»Name the complications of hemangiomas.
»What is the most common complication of hemangiomas?
»Are there any residua of hemangiomas after regression?
»What is Kasabach-Merritt syndrome?
»Name the two vascular tumors most commonly associated with the Kasabach-Merritt syndrome.
»Port wine stains and lymphangiomas are not neoplasms. What are they?
»What is the difference between a vascular malformation and a vascular neoplasm?
»What is Klippel-Trénaunay syndrome?
»How does Klippel-Trénaunay-Weber syndrome differ from Klippel-Trénaunay syndrome?
»What are blue-black hyperkeratotic vascular papules?
»Where and in whom are cherry angiomas most commonly seen?
»Where do you find venous lakes?
»What is the most common presenting feature of a pyogenic granuloma?
»Where are you likely to find a lesion of angiolymphoid hyperplasia?
»What vascular tumor is associated with the Sucquet-Hoyer canal?
»Matlike telangiectasias on the face, lips, tongue, ears, hands, and feet associated with internal bleeding is known as what syndrome?
»What is the most common cause of acquired facial telangiectasia?
»Which benign acquired vascular disease is often initially confused with Kaposi’s sarcoma?
»What is a cystic hygroma?
»Name the types of lymphatic malformations.
»Can you treat deep macrocystic lymphatic malformations?

 
 
 

What are blue-black hyperkeratotic vascular papules?


Angiokeratoma circumscriptum.
Fig. 42.5 Angiokeratoma circumscriptum.
Angiokeratomas. There are five types of angiokeratomas:
  • Localized angiokeratomas: Usually solitary and found on an extremity
  • Angiokeratoma circumscriptum: Presents at birth as unilateral plaques on an extremity (Fig. 42-5)
  • Angiokeratoma of Mibelli: Develops in childhood or adolescence over the dorsal surface of the hands or feet
  • Angiokeratoma of Fordyce: Most commonly seen on the scrotum
  • Angiokeratoma corporis diffusum (Fabry’s disease): An X-linked recessively inherited disease that results from a deficiency of the lysosomal enzyme a-galactosidase A, leading to the accumulation of glycolipids (mainly globotriaosylceramide) in the cells from various tissues. Multiple skin lesions between the umbilicus and knees.
Frequent development of hypohidrosis, paresthesias, cardiac, and renal disease Rozenfeld PA: Fabry disease: treatment and diagnosis, IUBMB Life 26:1043–1050, 2009.