Kindler syndrome

Author: Jane Widdowson et al, DEBRA New Zealand, February 2016.

What is epidermolysis bullosa?

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.

What is Kindler syndrome?

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.

Who gets Kindler syndrome?

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.

What are the clinical features of Kindler syndrome?

Kindler syndromeFeatures

Kindler syndrome

  • Blistering and photosensitivity beginning in infancy or early childhood
  • Gradual poikiloderma (altered pigmentation) and cutaneous atrophy (wasting)
  • Trauma related blistering on hands and feet
  • Can also develop mucosal involvement, ophthalmic and dental abnormalities
  • Early development of actinic keratoses

How is Kindler syndrome diagnosed?

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.

  • Diagnostic tests are also available in some countries and include skin biopsy of a newly induced blister which undergoes immunofluorescence antigen mapping (IFM) and/or transmission electron microscopy (EM). Mutational analysis (blood testing of genes), although not currently considered the first-line diagnostic test, is also available in some countries.

What is the treatment for Kindler syndrome?

See treatment of epidermolysis bullosa – general.

What is the outcome for patients with Kindler syndrome?

The blistering associated with Kindler syndrome reduces with age. Skin changes (poikiloderma) and photosensitivity persist.

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