Lamellar Ichthyosis


Lamellar Ichthyosis uses color pictures and clear explanations to teach about this inherited skin disorder. It also suggests specific products that are beneficial for moisturizing and softening the dry, scaly skin of lamellar ichthyosis.  

Table of contents:
Chapter 1. What causes Lamellar Ichthyosis?
Chapter 2. What are the symptoms of Lamellar Ichthyosis?
Chapter 3. What are the investigations for Lamellar Ichthyosis?
Chapter 4. What is the treatment for Lamellar Ichthyosis?
Chapter 5. What are the complications of Lamellar Ichthyosis?
Chapter 6. How can Lamellar Ichthyosis be prevented?

Book Excerpt
What Causes Lamellar Ichthyosis?

Lamellar ichthyosis is a keratinization disorder which is usually inherited in an autosomal recessive fashion. This means that both parents have to be carriers of the abnormal gene to pass it on to their children.

Patients with lamellar ichthyosis have a proliferative hyperkeratosis which means that the rate of production of new skin cells is faster than the rate at which the body can shed them.

What are the Symptoms of Lamellar Ichthyosis?
1. The baby is born covered with a thick collodion membrane which is a shiny, yellow film that covers the baby. This tough film later dries up and is shed in 10 to 14 days to reveal erythematous (red) skin covered with scales. 

2. 2. These scales of lamellar ichthyosis can be fine and white or large and dark. They are arranged in a mosaic pattern resembling the scales of a fish (ichthys is a Greek word which means fish). 

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Ichthyosis

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Ichthyosis uses color pictures and clear explanations to teach about this skin disorder. It also gives specific products that are beneficial for moisturizing and softening the dry, scaly skin of both acquired and inherited ichthyosis.   

Table of contents:
Chapter 1. What is Ichthyosis?
Chapter 2. What causes Ichthyosis?
Chapter 3. What are the symptoms of Ichthyosis?
Chapter 4. What is the treatment for Ichthyosis?
Chapter 5. What are the complications of Ichthyosis?
Chapter 6. Ichthyosis Vulgaris
Chapter 7. Congenital Ichthyosiform Erythroderma or Non-Bullous Ichthyosiform Erythroderma
Chapter 8. Epidermolytic Hyperkeratosis Ichthyosis or Bullous Ichthyosiform Erythroderma
Chapter 9. Lamellar Ichthyosis
Chapter 10. X-Linked Ichthyosis
Chapter 11. Acquired Ichthyosis

Book Excerpt
What is Ichthyosis?
Ichthyosis is derived from the Greek word ichthys which means fish. It consists of a group of around 20 disorders of keratinization which are characterized by persistently dry skin with a fish-like scale. These disorders affect all races and occur all over the world.
The widespread dry scaling of ichthyosis can develop either as a result of:
1. The production of new skin cells at a rate that is faster than the body can shed (proliferative hyperkeratosis)
2. The production of new skin cells at a normal rate but coupled with a slower than normal rate of shedding the old skin cells (retention hyperkeratosis)   

What are the Causes of Ichthyosis?
Ichthyosis can be inherited or acquired.

The inherited ichthyosis are due to genetic mutations (changes in the genes) and include:
1. Ichthyosis vulgaris
2. Lamellar ichthyosis
3. Epidermolytic hyperkeratosis
4. Congenital ichthyosiform erythroderma
5. X-linked ichthyosis

The acquired ichthyosis develop later on in life and are associated with diseases such as of the kidney and thyroid or the use of medications such as nicotinic acid.

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Linear IgA Dermatosis

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Linear IgA Dermatosis uses color pictures and clear explanations to teach about this autoimmune skin disease.
Table of contents:
What is Linear IgA Dermatosis?
What causes Linear IgA Dermatosis?
What are the symptoms and signs of Linear IgA Dermatosis?
What investigations are done for Linear IgA Dermatosis?
What are the differential diagnosis for Linear IgA Dermatosis?
What is the treatment for Linear IgA Dermatosis?
What are the complications of Linear IgA Dermatosis?

Book Excerpt
What is Linear IgA Dermatosis?
Linear IgA Dermatosis (LAD) is an autoimmune disease in which the patient develops vesicles (small blisters) and bullae (large blisters). Though it was formerly known as chronic bullous disease of childhood, it affects both adults and children.

The age of onset for this immunobullous disease in children is usually around 4 years though it can range from less than a year to 10 years of age. The age of onset for adult LAD ranges from the teen years to the eighties with most developing symptoms at around 50 years.
What Causes Linear IgA Dermatosis?
Linear IgA Dermatosis can be caused by:
1. Drugs such as vancomycin which is the most commonly associated drug. The skin lesions begin to develop from 1 day to 2 weeks after the patient takes the first dose.
2. Diseases such as typhoid, brucella, and tuberculosis have also been associated with LAD.  
4. Malignancies such as Hodgkin’s disease, non-Hodgkin lymphoma, and chronic lymphocytic leukemia (CLL) have also been associated with LAD though the association has not been proven.
5. Idiopathic or cases which arise due to unknown causes.

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