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What is cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. It is also called benign mucous membrane pemphigoid or oral pemphigoid. Areas commonly involved are the oral mucosa (lining of the mouth) and conjunctiva (mucous membrane that coats the inner surface of the eyelids and the outer surface of the eye). Other areas that may be affected include the nostrils, oesophagus, trachea and genitals. Sometimes the skin may also be involved where blistering lesions can be found on the face, neck and scalp.

Brunsting Perry cicatricial pemphigoid is a rare variant in which localised crops of recurrent blisters arise within urticarial plaques, usually on the head and neck. The blisters may burst resulting in blood-crusted plaques and scars.

Who gets cicatricial pemphigoid?

Cicatricial pemphigoid is predominantly a disease of the elderly with a peak incidence at around 70 years. However, childhood cases have been reported. It appears to be twice as common in women than men.

What are the signs and symptoms of cicatricial pemphigoid?

Site Features
Eye
  • Sensation of grittiness or pain
  • Conjunctivitis
  • Lesions form, erode and heal to leave scar tissue
  • May lead to impaired vision or blindness
Mouth
  • Blisters form first on the gums near the teeth
  • Palate, tongue, lips, buccal mucosa, floor of the mouth and throat may be affected
  • Painful and make it difficult to eat
  • Lesions occurring in the throat (oesophagus, trachea and larynx) can become life-threatening
Skin
  • Blisters on the skin develop in 25-30% of patients
  • May be itchy
  • Bleeding may occur if traumatised
Nose
  • Nose bleeds after blowing the nose
  • Crusting causing discomfort
Genitals
  • Painful blisters and erosions on the clitoris, labia, shaft of the penis, perianal area

What causes cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune blistering disease, which basically means that an individual’s immune systems starts reacting against his or her own tissue. In this particular instance autoantibodies react with proteins found in mucous membranes and skin tissue resulting in blistering lesions. The binding site appears to be within the anchoring filaments that help the epidermis (outside layer of skin) stick to the dermis (inner layer of skin).

Full article from DermNet NZ

http://www.dermnetnz.org/immune/cicatricial-pemphigoid.html

 

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An Epidemic of Absence: A New Way of Understanding Allergies and Autoimmune Diseases could be co-marketed with the Thomas Rockwell’s children’s classic How to Eat Fried Worms. It begins with the author, Moises Velasquez-Manoff, recounting his border-crossing to Tijuana to infect himself with Necator americanus—hookworms—in an attempt to cure the asthma, hay fever, food allergies, and alopecia that had plagued him since childhood. In the next three hundred pages, the author very cogently explains the idea that led him to willingly infect himself with a parasite known to cause severe diarrhea, anemia, and mental retardation in children.

Velasquez-Manoff marshals the reams of evidence researchers have accumulated to support said concept: the hygiene hypothesis, but with an updated, parasitic twist. The ideas he presents haven’t been accepted by many in the medical community, and there’s little high-quality evidence, in the form of well controlled trials, that exposure to parasites could have positive effects on human health. So, even if the author is thorough, it’s important to keep in mind that the evidence he’s presenting is primarily in the form of correlations.

The Hygiene Hypothesis

A simplistic view of the hygiene hypothesis is that in the absence of something dangerous to fight against—the cholera toxin, for example—immune cells get confused, or bored, and fight against harmless stimuli like dust mites and peanuts instead. But there is a more nuanced view. Our immune systems co-evolved with an enormous community of microbes, and were in fact shaped by them. Many became established, long-term, and vital residents in our guts; the importance, and in fact the very existence, of these commensals has only recently been realized.

Constant exposure to all of these bugs, as a unit, enhanced the regulatory arm of the immune system, modulating responses so that we could tolerate the filthy environment in which we lived while at the same time (hopefully) fighting off those pathogens that posed a mortal threat and not destroying our own bodies in that process. In the martial analogy that is inevitable in discussing immunology, ancient human immune cells that were always surrounded by microbes were like battle-hardened old soldiers who have learned the ability to watch warily when encountering something new, waiting to see whether or not it is dangerous; modern immune cells raised in our hyper-sanitized environment are like new recruits just given their first gun, testy and jumpy at the first hint of a threat and liable to blow up their surroundings in inappropriately directed and outsized force. Experience has not taught them moderation.

Seeing worms everywhere

Yes, he includes autism in the list of modern diseases caused by our out-of-whack immune systems. Along with other cases where immune dysfunction hasn’t been established, like obesity, cardiovascular disease, type 2 diabetes, and cancer.

There are some serious problems with blaming all of these on immune dysfunction, but we’ll focus on a single example: autism. Just as the absence of worms’ mediating effects on our immune system causes some people to have an allergic response to harmless ingested proteins and others to attack their own tissues, the argument goes, chronic inflammation in the womb generates fetuses with autism.

The rest of this article can be read here: http://arstechnica.com/science/2012/10/book-review-an-epidemic-of-absence-takes-on-the-worms-youre-missing/