ichthyosis vulgaris
Introduction
Introduction to ichthyosis vulgaris Ichthyosis vulgaris, also known as ichthyosissimplex, xeroderma, dominantichthyosis vulgaris or ichthyosisnitida, is an autosomal dominant inheritance. , more frequently. The incidence rate is about 1/250. The disease is similar to the so-called snake body, snake skin, and snake fetus. basic knowledge The proportion of sickness: 0.01% Susceptible people: no special people Mode of infection: non-infectious Complications: xeroderma pigmentosum heart failure kidney failure
Cause
Etiology of ichthyosis vulgaris
(1) Causes of the disease
The disease is autosomal dominant.
(two) pathogenesis
The pathogenesis is still not clear, and may be related to the reduction or lack of filament aggregates in the skin, or it is considered to be associated with abnormal lipid metabolism, low vitamin A levels, impaired desquamation process and stratum corneum. The internal desmosome is abnormally related.
Prevention
Verticulosis prevention
The disease belongs to chromosomal diseases, and the cause of chromosomal abnormalities is unclear. It may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy, so this disease cannot be directly prevented. Regular examination should be carried out during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time. For serious genetic diseases, artificial abortion should be carried out in time to avoid the birth of the disease.
Complication
Complications of ichthyosis vulgaris Complications , xeroderma pigmentation, heart failure, renal failure
1 ichthyosis, spasticity, mental retardation syndrome
It is a rare syndrome consisting of congenital ichthyosis, spastic limb paralysis and triad disorder. Almost all patients have extensive ichthyosis damage at birth, and about half have varying degrees of erythroderma. The erythroderma gradually eases with age and disappears after adulthood. Mainly for mental retardation, spastic quadriplegia or paraplegia.
2 ichthyosis pygmy mental disorder syndrome:
3 intelligent disorder epilepsy ichthyosis syndrome:
Infancy, some patients also have genital hypoplasia, dwarf and other symptoms. The intelligence is very poor and you can't take care of yourself. Most epilepsy is a major episode.
4 ichthyosis hepatosplenomegaly ataxia syndrome:
There is a dry skin disease in children. After the age of 62, there is a progressive increase in dysarthria and gait instability. Dry skin disease is most obvious with the limbs stretched out and the palm prints are deep. Hepatosplenomegaly, hard, but normal liver function. Neurological examination has manifestations of Alzheimer's disease. Limb and trunk movements are ataxia, dysarthria, and difficulty gazing upwards.
5 hair shaft abnormal intelligence disorder ichthyosis syndrome:
Brittle hair caused by abnormal hair dryness, mental retardation, reduced fertility and short stature.
6 ichthyosis polyneuritis ataxia syndrome:
Acute polyneuropathy, renal failure, or heart failure.
7 ichthyosis, bamboo-like hair allergy syndrome:
The hair is sparse, soft, dull, and dry. It is characterized by an inset of brittle hair, which is called bamboo hair.
Symptom
Symptoms of ichthyosis vulgaris common symptoms skin edging scaly blood deficiency pimples
Asymptomatic at birth, often in infants and young children, on the back and extremities of the limbs appear pale brown to dark brown diamond or polygonal scales, close to the skin, the edges of which are free, symmetrically distributed, especially in the lower limbs, severe It can affect the torso, the limbs and other parts of the limbs, the armpits, the gluteal cleft are often not involved, the scalp may have mild squamous scales, the common follicular keratosis papules on the back of the hand, the common linear cleft palate and the palmprint deepening of the palmar, and the damage varies. Generally, there are no symptoms, the condition is closely related to the season, the summer is light, and the winter is heavy. Some patients may have personal history and family history of hereditary allergic diseases, such as eczema, rhinitis and asthma. The course of the disease is inconsistent. Some patients reach adulthood. improve.
Examine
Examination of ichthyosis vulgaris
Clinical skin examination: often in the onset of infants and young children. On the back and extremities, there are pale brown to dark brown diamonds or polygonal scales, which are close to the skin, and their edges are free and symmetrically distributed. Especially for the lower limbs. In severe cases, it can affect the torso, limbs and other parts of the limbs. The armpits and gluteal clefts are often not involved. The scalp can have mild scaly scales. Common follicular keratosis papules on the back of the hand, common linear cleft palate and palmprint deepening.
Diagnosis
Diagnosis and identification of ichthyosis vulgaris
Asymptomatic at birth, often in infants and young children, on the back and extremities of the extremities appear pale brown to dark brown diamond or polygonal scales, close to the skin, its edges are free, symmetric distribution, TCM pathogenesis and syndrome differentiation: Chinese medicine believes that the disease is not congenital endowment, the temper is disordered the day after tomorrow, and the blood is dry and spleen, the spleen is lost, and the skin is dying for a long time.
