Author: Jane Widdowson et al, DEBRA New Zealand, February 2016.
Epidermolysis bullosa (EB) is a group of inherited diseases that are characterised by blistering lesions on the skin and mucous membranes. These may occur anywhere on the body but most commonly appear at sites of friction and minor trauma such as the feet and hands. In some subtypes, blisters may also occur on internal organs, such as the oesophagus, stomach and respiratory tract, without any apparent friction.
Kindler syndrome is a form of epidermolysis bullosa in which there is a mixed pattern of blistering on multiple levels within and/or beneath the basement membrane zone.
Kindler syndrome is inherited as an autosomal recessive disorder. This means that an abnormal gene must be inherited from each parent. On average, one-in-four children in a family are affected, and the familial nature of the disorder may be unnoticed.
In the dominant subtypes of EB, where an informative family tree is known, it is often acceptable for a clinical diagnosis (based on the presenting signs) to be made by a specialist dermatologist.
See treatment of epidermolysis bullosa – general.
The blistering associated with Kindler syndrome reduces with age. Skin changes (poikiloderma) and photosensitivity persist.
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