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Autoimmune Disorders In Celiac Disease

Each antibody binds to a specific antigen; an interaction similar to a lock and key. Courtesy Wikipedia.
Each antibody binds to a specific antigen; an interaction similar to a lock and key. Courtesy Wikipedia.

What Are Autoimmune Disorders?

[dropcap]A[/dropcap]utoimmune disorders refer to those conditions that involve an abnormal immune attack on the body’s own tissues perpetuated by the production of autoantibodies directed against the body, or “self.” Auto means self.

Q: Why does the immune system attack the body?

A: The exact answer is not yet known why the immune system turns against body tissue or “self.” 

Normally, the immune system protects the body from harmful substances and pathogens and produces antibodies against the offending foreign substances, called antigens, to get rid of them. The immune system (humoral) thereafter remembers all antigens and is ready for the next encounter should it happen.

Production of autoimmune antibodies is catastrophic because there is no turning off the readiness to attack a remembered threat (antigen) which is unfortunately “self.” 

Yes, steroids and anti-inflammatory drugs can control symptoms, but nothing can undo the memory programmed into the immune system to produce autoantibodies. There is an enormous research effort ongoing for the answer. 

Autoimmune disorders cover a wide range of diseases that may target only a particular organ, such as autoimmune hepatitis (liver), while others are systemic because the autoantibodies target a particular tissue that is part of more than one organ, such as scleroderma (connective tissue).

Autoimmune diseases as a group affect approximately 8.5% of people worldwide.

What Are Autoimmune Disorders In Celiac Disease and/or Gluten Sensitivity?

Chronic Bullous Dermatosis of Childhood or Linear IgA Dermatosis

chronic bullous dermatosis of childhoodWhat Is Chronic Bullous Dermatosis Of Childhood?

[dropcap]C[/dropcap]hronic bullous dermatosis of childhood, also termed linear IgA dermatosis, is the most common acquired autoimmune blistering disorder of childhood and is characterized by itchy, urticated papules and plaques as well as polycyclic lesions (merged circles) with blisters at the edge, located on normal looking skin around the mouth and perineum in young children. In children over 7 years, other parts of the body may rather be affected.

Q: What tissue is targeted in chronic bullous dermatosis of childhood?

A: In chronic bullous dermatosis of childhood, there is an autoimmune attack on structural proteins, usually proteolytic fragments of collagen XVII, which renders the dermal-epidermal junction prone to blistering.

The dermal-epidermal junction is where the surface skin layer, or epidermis, meets the lower layer, or dermis. Diagnosis is confirmed by characteristic histology and direct immunofluorescence showing linear IgA (immunoglobulin A antibody) staining of the basement membrane zone.1

The incidence of chronic bullous disease of childhood is rare. Age of onset is typically before 5 years of age and is seen in all ethnic groups.

What Is Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Mintz EM, Morel KD.Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin. 2011 Jul;29(3):459-62, ix. doi: 10.1016/j.det.2011.03.022. [↩]