Angiokeratoma – the education on skin consisting of expanded subepidermalny capillaries and which is followed giperkeratozy and other violations of an orogoveniye. Represents new growths of various extent (from 2 to 15 millimeters) of reddish, brown or almost black color. Diagnostics is performed on the basis of survey of integuments of the patient, Dermoscopy, histologic studying of educations, at some types angiokeraty – genetic researches. Treatment comes down to removal of new growths by various methods – by surgical excision, cryodestruction, laser cauterization.
Angiokeratoma serve as manifestation of a special dermatosis of various origin at which on the surface of skin good-quality giperkeratotichesky vascular educations are formed. For the first time one of forms of such disease (Mibelli's angiokeratom) was described in 1889, other types of pathology were studied by various scientists during the period since the end of XIX before the beginning of the 20th century. As the emergence reason angiokeraty serve genetic and exchange factors, at the same time some kinds of a disease are inherited on the mechanism linked to the H-chromosome. Occurrence, age and sexual distribution are strongly variable for different forms of pathology, also localization of new growths on a body, and also their quantity – from single angiokeraty before multiple defeat of integuments similarly differs.
At the moment dermatologists allocate several main types angiokeraty which differ among themselves not only on a clinical picture, but also on an etiology. In the general plan development of such good-quality new growths on skin is caused by vascular disorders at which in a subepidermalny layer the center consisting of pathologically expanded capillaries is formed. It is confirmed by a clinical picture – practically at all types of a disease of an angiokeratoma originally represent small educations on skin of pink color and a gentle consistence. Further anomalies of an orogoveniye of epidermis in the form of a giperkeratoz and an akantoz join vascular disorders, new growths begin to coarsen and often change the color almost to black.
As an immediate cause of development of the majority of types angiokeraty (a limited neviformny angiokeratoma of a trunk, an angiokeratoma of a scrotum and a vulva, a solitary papular angiokeratoma) experts see embryonic malformations. As a starting factor of emergence of such educations on skin the trauma can sometimes act. The hereditary nature of a disease was revealed for the first time for Mibelli's angiokeratoma, now it is supposed that it is inherited on the autosomno-prepotent mechanism with an incomplete penetrantnost. Fabri's disease relating to group of lizosomny diseases of accumulation has also obviously expressed genetic character – development angiokeraty is one of key symptoms of this state. This pathology is caused by a mutation of a gene of GLA located on the H-chromosome, it is responsible for structure of enzyme of an alpha galactosidase And. Violations in structure of enzyme lead to accumulation in cages of a tseramidtrigeksozid and numerous anomalies, one of which is education angiokeraty.
Localization, the sizes and quantity angiokeraty are in direct dependence on a disease form. In certain cases it influences also the forecast of pathology therefore definition of its type plays an important role in diagnostics. Today in dermatology allocate five main forms angiokeraty.
Angiokeratoma Mibelli – presumably genetic disease with autosomno-prepotent type of inheritance, but with an incomplete penetrantnost. Strikes generally female persons, the first displays of pathology arise at the age of 10-15 years, often are followed by other vascular and vegetative disorders – akrotsianozy, a palmar or-foot gipergidroz. Angiokeratoma appear on skin of palms and the back of foot, at first have an appearance of small reddish points. Over time these educations reach the sizes of 3-5 millimeters, change the color on cherry or crimson, begin to tower slightly over the surface of skin, become covered by horn stratifications. It is noticed that local or general cooling accelerates education and development angiokeraty Mibelli. At a trauma or spontaneously new growths can and bleed.
Limited neviformny angiokeratoma – one of disease forms caused by embryonic malformations. Often signs of change of skin can be noticed already at the child's birth, however in certain cases new growths arise at children's and even adult age. Localization of an angiokeratoma can be various – hips, a trunk, forearms. Most often there is a single education with a diameter of 2-5 millimeters without tendency to further growth, its color varies from red to almost black. Angiokeratoma can easily be injured with development of bleeding.
Fabri's disease – the hereditary pathology relating to group of lizosomny diseases of accumulation. As its transfer is linked to the H-chromosome, men, however are ill mainly and at female heterozygotes cases of development of this pathology, though in easier form are described. One of the first displays of a disease of Fabri are pains of neyropatichesky character, then reveal sweating violations (up to an angidroz) and education angiokeraty. New growths on skin arise at children's or teenage age, they have multiple character and can affect skin of a trunk, extremities and even a mucous membrane of a mouth. Usually angiokeratoma at Fabri's disease small, light pink, a soft consistence, the phenomenon of a giperkeratoz are defined on them poorly. Among other symptoms of this disease is more often than others warm violations and damages of kidneys up to a chronic renal failure are found.
Angiokeratom Fordyce most of researchers treats embryonic malformations though there are assumptions and of the hereditary nature of a disease. At this form of a new growth arise on a scrotum or a penis, slightly less often Fordyce's disease comes to light at women – in that case angiokeratoma appear on vulvar lips. The first displays of pathology can be found at various age – from 15 to 70 years, usually have educations on skin single character. The sizes of an angiokeratoma of Fordyce make 2-5 millimeters, a surface more often smooth, sometimes with giperkeratichesky stratifications. The new growth can be the cause of an itch, when combing it is easily damaged and bleeds.
The solitary papular angiokeratoma also belongs to embryonic malformations. The vascular new growth on skin is found aged from 10 up to 40 years, localization can be various – integuments of hands, trunks, the heads. The size of an angiokeratoma can reach 10 mm, is quite frequent and violations in vessels become the reason of almost black coloring of a new growth. This circumstance quite often leads to the wrong diagnosis of a melanoma of skin that is disproved by histologic researches.
Diagnostics of an angiokeratoma
Diagnostics of an angiokeratoma is made on the basis of data of survey of the dermatologist, Dermoscopy, a biopsy with the subsequent histologic studying of structure of new growths. Clinical diagnosis of a disease of Fabri also includes genetic researches and studying of some biochemical indicators of an organism. At survey comes to light one or several (depending on angiokeratoma type) educations on skin, several speakers over its surface, color varies from pink to almost black. As a rule, new growths are painless, except for cases when there was their travmatization. At accurate pressing on angiokeraty its pobledneniye occurs glass. Often are defined on the surfaces of education , stratifications, but sometimes (for example, at Fabri's disease) them it can not be observed.
Despite a variety of the reasons causing education angiokeraty and some distinctions in their appearance, the histologic structure of such educations in general is similar. In the thickness terms reveal is lacunary expanded capillaries which can sometimes be followed by the kavernozny cavities covered endoteliy. They raise epidermis, in the last often are found various degree of expressiveness, sometimes the epidermalny layer can be thinned. In case of a solitary papular angiokeratoma in expanded capillaries trombotichesky masses is quite often postponed, in many respects they also become the reason of quite dark coloring of education.
At Fabri's disease besides dermatological changes in diagnostics symptoms of violations of other bodies are considered: for example, neyropatichesky pains, signs of a hypertrophy of the left ventricle according to the ECG, violations of work of kidneys. At ophthalmologic survey of an eye bottom expansion of veins of a retina, microaneurism, hyperaemia of disks of optic nerves comes to light. Genetic diagnostics can reveal GLA gene mutations, it is the most exact, though rather complex analysis. Also large role in definition of the reasons angiokeraty at Fabri's disease is played by biochemical techniques – such as definition of activity of an alpha galactosidase And in fabrics and leukocytes, concentration of sfingolipid in blood plasma. Differential diagnostics is made with some forms of a gemangioma, a melanoma, certain hereditary diseases (for example, Randyu-Osler's disease).
Taking into account that in most cases immediate causes of development angiokeraty are unknown, etiotropny treatment of these educations does not exist today. Only to Fabri's disease apply fermentozamestitelny therapy to elimination of deficiency of an alpha galactosidase of A. S this purpose use such medicines as a beta and an alpha of an agalsidaz, however they can only slow down or prevent education new angiokeraty whereas such therapy does not affect already arisen educations.
For elimination angiokeraty use a set of local procedures – surgical excision, a diatermokoagulyation, cryodestruction, laser cauterization. Before purpose of treatment it is necessary to make histologic studying of education for confirmation of the diagnosis and an exception of such states as a gemangioma and a melanoma. In case of elimination of such forms angiokeraty as limited, solitary or Fordyce's disease a recurrence most often does not arise. But, if Mibelli or Fabri's diseases are the reason of development of a new growth, even after full removal of vascular anomaly emergence new on the same or other sites of skin is possible.
Forecast and prevention angiokeraty
In most cases the forecast for an occasion angiokeraty favorable, even at refusal of removal of new growths. In rare instances a frequent travmatization of vascular anomalies of skin to become the reason of easy forms of anemia, cases of a malignization or other complications at the same time it is not described. An exception is Fabri's disease, in the absence of treatment of this disease of accumulation severe defeats of vessels, heart and kidneys are possible that can lead to a lethal outcome. Before development of fermentozamestitelny therapy most of sick men died of a myocardial infarction, a stroke and a chronic renal failure to forty-year age.