Rosary hair
Introduction
Introduction to the beaded hair Monilethrix is a congenital hair malnutrition, often systemic, with uneven dry hair, many nodules, beaded, and easy to break between nodules. basic knowledge The proportion of sickness: 0.01% Susceptible people: no special people Mode of infection: non-infectious Complications: cataracts, epilepsy
Cause
Rosary
(1) Causes of the disease
For autosomal dominant inheritance, this disease is a hair cortical disease and is thought to be related to the type II keratin gene family located at chromosome 12q13.
(two) pathogenesis
A heterogeneous mutation was found in the hHb6 of type II hair keratin and the conserved glutamate residue of the hHb1 gene.
Prevention
Rosary prevention
1) It is most important to eliminate the source of infection, that is, to cure all patients with rosary hair and sick animals. The beaded hair not only invades the scalp, but also invades the root of the hair follicle, so it is more complicated to treat than other rickets.
1 hair removal treatment If the range of rosary hair is very small, manual hair removal treatment can be used, that is, the flat head lice are pulled out in the direction of hair growth in the lesion area. All normal hair within 3mm around the lesion should be removed to prevent the spread of the lesion. The area is then cleaned and coated with 2% iodine once a day. So, pull out once a week for 3 to 4 times, and wash your hair frequently. Apply 5% sulfur ointment once a week. If the lesion area is large, you can use X-ray to make the hair loose and easy to remove. However, this method must have certain technical equipment and is not suitable for all patients, and it is rarely used at present.
2 griseofulvin comprehensive treatment is oral griseofulvin, with topical 2% iodine or 5% sulfur ointment, wash your hair every day, cut once every 1-2 weeks. This method is suitable for all types of rosary hair, but should pay attention to drug contraindications and side effects, pay attention to review liver function during medication. The dosage of griseofulvin is 10-20 mg per kilogram of body weight per day, and even for 15 to 20 days, depending on the condition, the dosage is adjusted. For jaundice and white diarrhea with less damage, it should also be combined with manual hair removal treatment, which can reduce the amount of oral medication to avoid possible side effects.
2) Cut off the route of transmission If a patient with a beaded hair is found, appropriate isolation measures should be taken. For example, children with rosary hair should not go to kindergarten or primary school for the time being, and then resume their collective life after they are completely cured. Avoid transmission to family members or other children at home. At the same time, the hats, headscarves, pillow covers, clothes, etc. used by the patients should be disinfected by means of sun exposure, boiled water and other methods. Contaminated barber tools should also be thoroughly disinfected, boiled or sterilized, or placed in a closed container with formaldehyde gas. The infected hair, scales and suede are burned by application.
3) Protecting susceptible people For the beaded hair, every child is susceptible, so the beaded hair is often spread in kindergartens or primary schools. Prevention of rosary hair is not based on taking medicine, but to strengthen publicity and education so that children can develop good hygiene habits, and often check the child's head. If suspicious circumstances are found, they should go to the hospital for treatment.
Complication
Rosary-like complications Complications cataract epilepsy
Some are associated with white nail disease, cataract and abnormal teeth, excessive skin elasticity, epilepsy, mental retardation and mental disorders.
Symptom
Rosary symptoms common symptoms mental disorders white nail nodules abnormal teeth
There is a family history, which is autosomal dominant. This disease begins in infants. Some patients see puberty, the disease gradually increases, the symptoms can gradually improve with age, and even have self-healing, but also in summer and pregnancy. Reduced, hair dry uneven thickness, the performance of the spindle-shaped swelling nodules, alternating with the narrow atrophy between the nodules, like a string of rosary, dry and rough, often fragile 1 to 2 cm from the scalp Broken joints, occasionally broken on the nodules, dry hair can have longitudinal cracks, in addition to hair (more serious occipital), eyebrows, eyelashes, pubic hair, mane, and even mane can be affected, most patients Accompanied by limb extension, sacral and posterior keratosis of the hair, some with white nail disease, cataract and abnormal teeth, excessive skin elasticity, epilepsy, mental retardation and mental disorders, microscopic observation of nodules at normal hair, knot The stenosis between the internodes is abnormal, and there is often no medulla. Under the electron microscope, there are normal shingled hairs in the nodules, or the hairy skin disappears; the small skins disappear between the nodules, showing parallel longitudinal Ditch There are holes in some of the grooves.
Examine
Rosary inspection
The microscopic observation of the nodules at the normal hair, the stenosis between the nodules is abnormal, often without medulla.
Under the electron microscope, there were normal shingled hairs in the nodules, or the hairy skin disappeared; the small skins disappeared between the nodules, and there were grooves in the parallel longitudinal rows and holes in some of the grooves.
Diagnosis
Rosary diagnosis
According to the clinical manifestations, the hair shaft thickness is uneven, showing a fusiform nodule, alternating with the narrow atrophy between the nodules, like a string of rosary, dry and rough, often fragile 1 to 2 cm from the scalp The nodules are broken, and occasionally they can be broken on the nodules. The hair shaft can be diagnosed by the characteristic of longitudinal fracture.
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