Angioma in tufts

The angioma in tufts ( AT ), also named “ Angioblastome de Nakagawa ” is a vascular tumor (Angiome) characterized by a regular aggravation (certain regressing cases however spontaneously).
C' is a Rare disease, Congénitale or early (generally acquired very early; before 5 years)

The angioma in tufts was described by Wilson Jones in 1989, but of the Japanese authors had before identified it under the name of angioblastome of Nakagawa .

With not confusing with the congenital hémangiome of type NICH.

Prevalence

The Prévalence is unknown, but more than 200 cases were described in the literature (all are not published)

Symptoms

infiltrated red plates, badly delimited, sometimes strewn with red papules more intense, whose aspect varies in time.
more rarely (often in congenital cases), a tumoral mass important is present.
Lesions sensitive to palpating, being able to be painful with the shocks, sometimes covered with sleeping bag or encircled of a clear halation.

Diagnosis

The diagnosis is histological.

There is dispersion in all the derm of small capillary tufts laid out in “ lead shot ” and encircled of a blood-vessel.
One also often finds spaces lymphatic and a dermic fibrosis.

It is a negative tumor GLUT1 , contrary to the infantile Hémangiome.

Evolution, Forecast

Generally (there are cases of spontaneous remission), certain AT infiltrate in the lower extremities by producing a sclerous hardened tegument little by little (the Sclérose possibly gaining the muscle and the articulation close relations, by obstructing their functions. Complications: a Syndrome of Kasabach-Merritt (SKM) with a major Thrombopénie can lead to death.

Treatment

No effective specific treatment.
Corticothérapie general, Interféron alpha or Vincristine sometimes improved of the forms serious extents

See too

Internal bonds

Angioma

External bonds

Tufted Angioma on emedicine.com

Random links: