autosomal dominant with high penetrance (the frequency with which this feature is controlled by one or another gene) gene. Initial signs
disease detected in the first months of life. maximum
the development reaches to 10 years of age and continues throughout
life with improvements in the summer and during puberty.
The disease can occur with varying intensity. Disease
common, is characterized by dryness, thickening of the skin, but without the inflammatory changes, without
erythema (redness of the skin strong). Especially pronounced dryness and flaking on the extensor
surfaces of the extremities, in the elbow and knee joints. Leather
axillary, elbow, popliteal pits, perineum, palms and soles
It is not changed. In children up to 4-5 years involved in the process and the skin of the face. AT
forehead and cheek areas can be seen lamellar or scaly
scales grayish muddy color.
Depending on the color of the flakes, their appearance, the expression
dry skin, there are several types of clinical vulgar
Xeroderma (xerodermia) - Initial, the easiest
kind of ichthyosis, in which there are skin dryness, peeling
pityriasis nature, mainly on the front surfaces
upper and lower extremities, and partly on the skin of the trunk.
Simple ichthyosis (ichthyosis simplex) - A second
intense expression variety. Dry skin is expressed
more significant, there are copious amounts of lamellar scales, tightly
adjacent to the thickened, dry, hard and rough skin.
Brilliant ichthyosis (ichthyosis nitida) manifest (manifest)
significant accumulation of horny scales, different pearlescent
shine or have a slightly shiny, as if varnished surface.
In the presence of whitish pearly scales use the term "white
ichthyosis. " Sometimes the scales become thicker, denser, acquiring
dark gray in color and are arranged ribbon-like, tightly adhering to the skin
and distinguishing between deep grooves. This option clinical form
ichthyosis called serpentine (ichthyosis serpentina). The most severe
is a kind of styloid ichthyosis (ichthyosis hystrix), while
wherein the front surfaces of the limbs are arranged clusters
thick horny masses in the form of pins protruding by 1 - 10 mm above the
skin surface resembling porcupine quills.
Hair and nails with milder forms of ichthyosis are not changed and the total
the condition of patients is satisfactory. However, in infants with
severe forms of ichthyosis, dry hair, thinning, sparse.
Observe various onychodystrophy. Patients ichthyosis due
low flow and sebaceous excretions and breach of thermoregulation
astenizirovany lag behind in the physical and intellectual development. they
marked immunodeficiency, predisposition to the development of
piodermitov, pneumonia, otitis media, the emergence of eczema, neurodermatitis,
psoriasis. Imbetsillopatiya and disadvantages of forming phenotype characterized
children with severe forms of ichthyosis.
Diagnosis vulgarngo ichthyosis
Clinical and morphological manifestations of all varieties
ichthyosis vulgaris differ constancy. All forms of the disease
occur in childhood. Therefore, the process of diagnosis is not
presents difficulties. The differential diagnosis with acquired
ichthyosis based on the presence in acquired ichthyosis
avitaminoznogo state, especially vitamin A deficiency, blood diseases
such as leukemia or Hodgkin's disease.