Kaposiform Hemangioendothelioma (KHE) in Children
About Kaposiform Hemangioendothelioma
Kaposiform Hemangioendothelioma is sometimes confused with a birthmark but is actually a benign tumor of the blood vessels. KHE is rare, typically occurs in infancy and affects 1 out of every 100,000 children. While the exact symptoms of KHE depend on its location, it most commonly presents itself as a growing lesion on the skin that can:
- Feel firm and warm to the touch
- Appear anywhere on the body, including head and neck stomach, back, arms and legs — even inside the chest, abdomen or bones
- Display bruise-like discoloration, or red or purple spots on the body (petechiae)
- Swell and become painful
- Result in low platelet counts and develop into a Kasabach-Merritt syndrome
Complications From Kaposiform Hemangioendothelioma
Medical therapy may not be necessary for most patients if the tumor is superficial; and some mild KHE tumors go away without treatment. However, some patients with KHE develop a Kasabach-Merritt syndrome (KMS), in which a rapidly growing tumor traps platelets and keeps them from circulating through the bloodstream. Since platelets help prevent bleeding, patients with KMS develop a bleeding disorder, also called coagulopathy. KMS is an infrequent but potentially fatal complication.
When to Consult a Birthmark Specialist for Kaposiform Hemangioendothelioma
It’s important to see a specialist quickly when KHE is suspected. Because KHEs can grow and lead to long-term complications, close observation and often early treatment is required to shrink the tumor and prevent further growth before it affects muscles or other tissues nearby.
The severity and location of the tumor will determine the treatment course prescribed by the specialist including: observation, steroids and other medications, surgery, and blood product transfusions when necessary.
Tufted Angioma (TA)
About Tufted Angioma
Tufted Angioma is a mild form of kaposiform hemangioendothelioma. The evolution of the disease can vary considerably, including complete or partial spontaneous regression or persistence over the years. Kasabach-Merritt syndrome is the most serious complication, which is why patients with TA should be monitored on a regular basis by a vascular birthmarks specialist.
Laser therapy or surgical intervention may be used to manage the disease.
About Pyogenic Granulomas
Pyogenic granulomas are small, raised, and red bumps on the skin which often bleed. They are a common occurrence in children, and head, neck, upper trunk, hands and feet are the most frequent sites of these lesions.
Congenital Melanocytic Nevus
About Congenital Nevus
Found in infants at birth, congenital nevus, or a mole, occurs in an estimated 1 percent of infants worldwide; it is located in the area of the head and neck 15 percent of the time. The characteristics of congenital melanocytic nevi include:
- Light brown to black patch or plaque, covering any size surface area and any part of the body
- Frequent presence of excess terminal hair, a condition called hypertrichosis
- Continued growth proportional to the body size as the child matures
- Developing thickness and elevation, although these features can also be present from birth
Surgical removal of the mole is the standard of care. Our specialists have developed unique approaches to remove congenital nevi, especially those occurring in the head and neck region.
About Spider Angiomas
Spider angiomas, also known as nevus araneus, are common and benign lesions found on 10-15 percent of adults and kids. The abnormal collection of blood vessels near the surface of the skin with a red dot in the center and reddish extensions give the appearance of a spider. Frequently found on the face, neck, upper part of the trunk, and arms, they may also be present on the backs of the hands and fingers in young children.
While most cases of spider angiomas will not require treatment, in cases of spider angiomas on the face, laser treatment can be used to remove the lesion.