Congenital aplaziya of skin – group of conditions of not clear etiology which are characterized by focal violation of formation of integuments with development of hems. Symptoms of this state come to light right after the birth of the child at which one or several erosion or ulcerations on head skin or, very seldom, on other parts of the body are observed. Diagnostics of a congenital aplaziya of skin is performed on the basis of data of survey by the dermatologist, histologic studying of fabrics in the defeat center. Treatment only symptomatic for the purpose of prevention of development of a secondary infection, but surgical correction of hems for reduction of cosmetic defect is possible.
Congenital aplaziya of skin
The congenital aplaziya of skin is a focal defect of development of skin at which formation of epidermis, terms, appendages, and in especially hard cases and hypodermic cellulose is broken. This state is known to mankind more than 250 years, however it was not succeeded to establish the reasons of its development still, in dermatology there are only theories in this respect. Occurrence of a congenital aplaziya of skin is precisely unknown, most of researchers estimate it at the level of 1:10000. Sometimes such state is combined with some genetic diseases, other defects of pre-natal development. The congenital aplaziya of skin in most cases does not lead to serious consequences, however cosmetic defect in the form of a hem on the place of the pathological center remains at the person for the rest of life.
Reasons of a congenital aplaziya of skin
Today there is no uniform and conventional theory which would explain development of this congenital defect of development of skin. It is supposed that the whole group of various pathological factors which lead to violation of process of closing of a nervous tube is the reason of a congenital aplaziya of skin or terms and epidermis slow down development of embryonic rudiments. Sometimes, presumably, autosomno-prepotent is possible to reveal family forms of this state, at the same time the mechanism of its inheritance. But much more sporadic forms of a congenital aplaziya of skin, and sometimes in combination with other malformations, caused by genetic diseases or influence of teratogenny factors meet more often. It gives a reason to consider this state as a result of influence on the formed fruit of various damaging factors.
Symptoms of a congenital aplaziya of skin
Aplaziya of skin comes to light right after the child's birth. Most often on parietal area the center of rounded shape with a diameter of 1-3 centimeters is found. Approximately in a third of cases there are two centers, three and more sites of an aplaziya of skin meet even less often. The pathological site represents the erosion or an ulcer covered with a crust and granulations, indumentum on it is absent. However around an ulcer longer and dark hairs grow that received the name of a symptom of "a hair collar". Color of education varies from pink to bright red.
Over time, in the absence of complications (a secondary infection, for example), the site of a congenital aplaziya of skin begins to be resolved with formation of a hem of white color. On it further hair also do not grow, and it remains at the person for the rest of life. Besides skin symptoms, at the child with the center of a congenital aplaziya of skin formation violations of more deeply located fabrics and other malformations – a cleft lip, a wolf mouth, an atrophy of eyes can be registered. At children of advanced age and adults on the place of a hem malignant new growths can sometimes develop.
Diagnostics of a congenital aplaziya of skin
Recognition of this disease usually does not represent work for the dermatologist – his symptomatology is rather specific and to confuse it with other congenital skin states quite difficult. However in certain cases both other pathological processes and states can have a picture, similar to a congenital aplaziya of skin. Therefore it is necessary to make differential diagnosis of this pathology with such diseases as a focal sklerodermiya, diskoidny red a wolf cub, and also with consequences of a perinatal trauma (from nippers and other obstetric tools). Family forms of a hypoplasia of face skin are very similar to an aplaziya, however at the same time the atrophic centers are observed in temples.
Histologic studying of fabrics of the pathological center can give the most exact diagnostic data. At a congenital aplaziya of skin sharp reduction of thickness (is observed up to the 1st layer of cages) epidermis, terms, sometimes hypodermic cellulose. Signs of an inflammation and leykotsitarny infiltration (in the absence of a secondary infection) are not observed, also do not reveal skin appendages.
Treatment and forecast of a congenital aplaziya of skin
Treatment of a congenital aplaziya of skin is conditionally divided into two stages. The first is made directly after the child's birth – during this period only preventive and ukhodovy actions (processing of erosion the antibacterial ointments moisturizing with means), imposing of a bandage for reduction of risk of traumatizing are shown. In several weeks on the place of the pathological center the hem which though remains for the rest of life will be created, but it can be covered with surrounding hair. The second stage comes down to surgical elimination of defect (most often, for the cosmetic reasons), and it is possible to carry out it at late children's or adult age. At correction of sites of a congenital aplaziya of skin, considerable on the area, skin transplantation can be applied. The forecast of a disease in general favorable, some researchers indicate the need of annual survey of a hem at the dermatologist because of risk of development of oncological processes.