Beginning >> Diseases >> Tumors >> Angiokeratoma

Angiokeratoma

Angiokeratoma — high-quality giperkeratotichesky vascular education.

Epidemiology

Fabri's illness is shown at boys at youthful age.
Mibelli's angiokeratoma develops usually at the age of 10 — 15.
The angiokeratoma of a scrotum and vulva (Fordyce's illness) develops aged from 16 till 70 flyings.

Classification

Limited angiokeratoma.
Mibelli's angiokeratoma.
Angiokeratoma of a scrotum and vulva (Fordyce's illness).
Diffusion angiokeratoma of a trunk (Fabri's illness).

Etiology and pathogeny

The diffusion angiokeratoma of a trunk is the illness of accumulation of glycolipids or sphingolipids inherited on the H-linked recessive type. Accumulation in skin and in internals results from insufficiency of lizosomalny enzyme — and - galactosidases And (tseramidtrigeksidaza). Supervision of a diffusion angiokeratoma at a fukozidoza, a sialidosis, insufficiency are described beta mannozidazy, and also without visible exchange disturbances.
Mibelli's angiokeratoma is inherited on autosomal dominantly type in families with frostbites in the anamnesis.
For a limited angiokeratoma and an angiokeratoma of a scrotum and a vulva — are not established

Clinical signs and symptoms

The limited form can exist since the birth or develop within the first decades of life originally in the form of the grouped papules of red color which create a plaque with a warty surface over time.

Fabri's illness is characterized by existence of hundreds of micropapules with a diameter of 1 — 2 mm of red color covering skin of the lower extremities, the lower part of a trunk.

At Mibelli's angiokeratoma the set of roundish, red papules with a diameter of 1 — 5 mm with a superficial hyperkeratosis on skin of dorzalny surfaces of palms, feet, fingers comes to light.

At a scrotal form (Fordyce's illness) dark red papules are localized on skin of a scrotum, a vulva.

The diagnosis and the recommended clinical trials
Are shown:
• research of peripheral blood in the polarized light for identification of lipids;
• determination of level and - galactosidases;
• analysis of urine;
• biopsy of the limited center.
At a limited angiokeratoma the acanthosis, a papillomatosis and a hyperkeratosis are characteristic. Sharply expanded capillaries are near epidermis and are sometimes put into its epidermal outgrowths. Though the majority of vessels is filled with blood, some of them, with thin walls, contain a lymph. Expansion of all vascular network of a derma and hypodermic fatty tissue is characteristic. Expanded capillaries are sometimes thrombosed.
At a diffusion form pathological changes are similar.

Differential diagnosis
At a limited form of an angiokeratoma differential diagnosis is carried out with a verrukozny form red flat depriving, a melanoma.
The diffusion form does not demand carrying out differential diagnosis.

General principles of treatment
At a limited form — surgical excision, laser evaporation. At a diffusion form — a symptomatic treatment, including soothing medicines, a hemodialysis, according to vital indications — a renal transplantation.

Forecast

Favorable at limited forms, uncertain at systemic lesions. Complications of a disease are connected with damage of internals (a hypertrophy of a left ventricle, opacification of a cornea, incidental fever, renal failures, pain).
 
"Actinic keratosis   Diseases of heavy chains"