About ichthyosis hystrix curth macklin type

What is ichthyosis hystrix curth macklin type?

Ichthyosis hystrix, Curth-Macklin type is a rare inherited skin disorder. It is characterized by scaling skin (ichthyosis) ranging from mild to severe. The patches of scaly, thickened skin range from spotty to severe, and may appear on almost any part of the body.

What are the symptoms for ichthyosis hystrix curth macklin type?

T symptom was found in the ichthyosis hystrix curth macklin type condition

Ichthyosis hystrix, Curth-Macklin type is characterized by patches of abnormally thick, hard skin that may appear almost anywhere on the body. Some clinicians describe the skin as “horn-like”. With treatment, the patches may be considerably reduced. Under examination by electron microscopy, numerous cells (keratinocytes) with two nuclei are found. Frequently, the nuclei are surrounded by shells of a primitive, precursor protein of the skin.

What are the causes for ichthyosis hystrix curth macklin type?

The cause of ichthyosis hystrix, Curth Macklin type is a defect in the development of the precursor, structural protein (tonofilaments). The defect occurs as a result of a change (mutation) in the gene that produces (codes for) these proteins. The faulty gene has been mapped to chromosome 12q13.

Ichthyosis Hystrix, Curth-Macklin type is an inherited disorder, transmitted as an autosomal dominant trait.

Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Human body cells normally have 46 chromosomes. Pairs of human chromosomes are numbered from 1 through 22 and the sex chromosomes are designated X and Y. Males have one X and one Y chromosome and females have two X chromosomes. Each chromosome has a short arm designated “p” and a long arm designated “q”. Chromosomes are further sub-divided into many bands that are numbered. For example, “chromosome 12q13” refers to band 13 on the long arm of chromosome 12. The numbered bands specify the location of the thousands of genes that are present on each chromosome.

Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother.

All individuals carry a few abnormal genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.

Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child.

What are the treatments for ichthyosis hystrix curth macklin type?

Ichthyosis hystrix, Curth-Macklin type is treated by applying skin softening (emollient) ointments, preferably plain petroleum jelly. This can be especially effective after bathing while the skin is still moist. Salicylic acid gel is another particularly effective ointment. The skin should be covered at night with an airtight, waterproof dressing when this ointment is used. Lactate lotion can also be an effective treatment for this disorder.

What are the risk factors for ichthyosis hystrix curth macklin type?

Ichthyosis Hystrix Curth Macklin Type is an uncommon inherited skin condition. It is characterised by mild to severe scaling of the skin (ichthyosis). The scaly, thickened skin patches can range in severity from mild to severe and can develop on practically any portion of the body. Patches of excessively thick, hard skin can occur practically anywhere on the body in ichthyosis hystrix, Curth-Macklin type. Clinical experts have described the skin as "horn-like." The patches may be significantly decreased with treatment.

Risk factors Ichthyosis Hystrix Curth Macklin Type

The risk factors for Ichthyosis Hystrix Curth Macklin Type are:

  • The interference of the genes for a specific trait found on the chromosomes inherited from the mother and father determines the presence of genetic diseases.
  • Consanguineous parents are more likely than unrelated parents to share a defective gene, which raises the chance that their offspring may develop a recessive genetic condition.
  • When just one copy of a defective gene is required for the disease to develop, dominant genetic diseases result. The faulty gene may be brought on by a new mutation (gene alteration) in the sick person, or it may be inherited from either parent.
  • Regardless of the sex of the offspring, there is a 50% chance that the faulty gene will be passed from the affected parent to their offspring throughout each pregnancy.


Symptoms
Skin that is thick and hard and can appear practically anyplace on the body,Numerous cells (keratinocytes) with two nuclei are visible under electron microscope examination,The nuclei frequently have shells made of a primitive, skin-related protein,The skin is malodorous and frequently infected,Nail dystrophy may be present,Anhidrosis,Pruritus,Fissuring,Decreased joint mobility.
Conditions
Scaling skin (ichthyosis) ranging from mild to severe,Atypical naevi,Palmoplantar contractures,Dehydration,Electrolyte,Bacterial colonisation
Drugs
Ointment,Salicylic acid gel, Salicylic acid tablet,Lactate lotion,Skin softening (emollient) ointments, preferably plain petroleum jelly

Is there a cure/medications for ichthyosis hystrix curth macklin type?

Ichthyosis hystrix, Curth-Macklin type, is an uncommon inherited skin condition.

Treatment

  • Application of skin-softening (emollient) ointments, particularly plain petroleum jelly, is the treatment of choice for Ichthyosis Hystrix, Curth-Macklin type.
  • When the skin is still moist from a bath, this can be extremely beneficial.
  • The gel form of salicylic acid is another highly efficient ointment.
  • When using this ointment, the skin should be covered at night with an airtight, waterproof dressing.
  • Another successful treatment for this condition is lactate lotion.


Symptoms
Skin that is thick and hard and can appear practically anyplace on the body,Numerous cells (keratinocytes) with two nuclei are visible under electron microscope examination,The nuclei frequently have shells made of a primitive, skin-related protein,The skin is malodorous and frequently infected,Nail dystrophy may be present,Anhidrosis,Pruritus,Fissuring,Decreased joint mobility.
Conditions
Scaling skin (ichthyosis) ranging from mild to severe,Atypical naevi,Palmoplantar contractures,Dehydration,Electrolyte,Bacterial colonisation
Drugs
Ointment,Salicylic acid gel, Salicylic acid tablet,Lactate lotion,Skin softening (emollient) ointments, preferably plain petroleum jelly

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