What causes linear IgA disease?

The exact cause of Linear IgA Disease is unknown however it is considered to be an “autoimmune” disease. The body’s immune system produces antibodies known as Immunoglobulin A (IgA) which are incorrectly deposited in the top layer of the skin in a line – hence the term “linear”.

What is linear dermatitis?

Linear immunoglobulin A (IgA) dermatosis (LAD) is an autoimmune subepidermal vesiculobullous disease that may be idiopathic or drug-induced. Children and adults are affected, with disease of the former historically referred to as chronic bullous dermatosis of childhood.

Does Linear IgA go away?

Treatment suppresses Linear IgA disease but does not cure it. However, in the majority of patients the disease tends to eventually resolve. Skin lesions tend to heal without leaving permanent scars. However, ulcers in the eyes, mouth or genital region can leave scars or cause permanent damage.

Is Linear IgA bullous dermatosis contagious?

Linear IgA Bullous Dermatosis (LABD) is an autoimmune disease that affects the skin. The main symptoms are painful blisters and rashes. The disease is also sometimes called “chronic bullous” or “linear IgA dermatosis.” The skin disease is not hereditary and it is also not contagious.

Is Linear IgA disease itchy?

What are the symptoms of Linear IgA disease? These range from mild itching to a severe burning sensation.

How long does linear IgA last?

The mean duration of idiopathic linear IgA dermatosis of childhood is 3.9 years, ranging from 2.1-7.9 years. Remission has been reported to occur in 64% of children, in most cases within 2 years. Disease of adults is more protracted, with a mean duration of 5.6 years, lasting anywhere from 1-15 years.

What is chronic bullous disease?

Chronic bullous disease of childhood is a rare autoimmune skin condition which results in clusters of blisters developing in rings often on the face or genitals. In autoimmune disorders, antibodies – which usually seek and destroy foreign invaders to the body, such as viruses – attack the body’s own cells instead.

Are lesions cancerous?

Lesions can be categorized according to whether or not they are caused by cancer. A benign lesion is non-cancerous whereas a malignant lesion is cancerous. For example, a biopsy of a skin lesion may prove it to be benign or malignant, or evolving into a malignant lesion (called a premalignant lesion).

What does sarcoidosis look like on the skin?

Smooth bumps or growths Mostly painless, these bumps and growths tend to develop on the face or neck, and often appear around the eyes. You may see lesions that are skin-colored, red, reddish-brown, violet, or another color. When touched, most bumps and growths tend to feel hard.

What is EBA disease?

Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Current treatment options rely on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment.

What are bullous changes in lungs?

Bullous lung disease is characterized by the development of bullae within the lung parenchyma. A bulla is a permanent, air-filled space within the lung parenchyma that is at least 1 cm in size and has a thin or poorly defined wall; it is bordered only by remnants of alveolar septae and/or pleura.

Which is the best example of a linear disease?

Herpes zoster is the best-known example for a skin disease usually confined to certain dermatomes. Based on own observations of 170 patients with epidermal nevi, in 1901 the German dermatologist Alfred Blaschko (1858–1922) was the first to describe a specific linear pattern of genetic skin diseases manifesting themselves on the trunk.

What causes linear skin defects ( MLS ) syndrome?

MLS syndrome is typically caused by either a deletion of certain genetic material on the p (short) arm of the X chromosome or by a mutation in the HCCS gene. In some cases, it may be caused by mutations in the COX7B and NDUFB11 genes, (also located on the X chromosome).

What are the two types of linear IgA disease?

Linear IgA disease has two main clinical variants—bullous disease of childhood and adult linear IgA disease. Although they vary clinically in minor ways, their immunofluorescence patterns are identical. The IgA autoantibodies target several antigens within the dermal–epidermal junction.

What are linear skin defects on the head?

People with this condition usually also have what is described as linear skin defects on the head and neck. These are characteristic skin markings that follow the paths along which cells migrate as the skin develops before birth. These paths are known as the lines of Blaschko.