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Kindler syndrome

Kindler syndrome is a rare type of epidermolysis bullosa, a group of inherited conditions that cause fragile and blistering skin

Prevalence

Unknown

N/A

US Estimated

N/A

Europe Estimated

Age of Onset

ICD-10

Q81.8

Inheritance

Autosomal dominant

Autosomal recessive

Mitochondrial/Multigenic

X-linked dominant

X-linked recessive

5 Facts you should know

FACT

1

Kindler syndrome causes skin to blister beginning in early infancy

FACT

2

Infants and young children with Kindler syndrome have a tendency to blister with minor trauma

 

FACT

3

Other symptoms may include photosensitivity, poikiloderma, hyperkeratosis

FACT

4

The condition can also affect the moist lining of the mouth, eyes, esophagus, intestines, genitals, and urinary system

FACT

5

Patients with Kindler syndrome have an increased risk for squamous cell carcinoma

Interest over time

Kindler syndrome is also known as...

Kindler syndrome is also known as:

  • Poikiloderma, hereditary acrokeratotic
  • Bullous acrokeratotic poikiloderma of kindler and weary
  • Poikiloderma, congenital, with bullae, weary type
 

What’s your Rare IQ?

Almost one-fourth of documented cases of Kindler syndrome, a very rare type of epidermolysis bullosa, were found in native tribes of which country?

Common signs & symptoms

Abnormal blistering of the skin

Aplasia/Hypoplasia of the skin

Cheilitis

Cutaneous photosensitivity

Erythema

Palmoplantar keratoderma

Poikiloderma

Current treatments

Currently, there is no cure or established treatment for Kindler syndrome. Therefore, the goals of management are to treat the symptoms and prevent complications.

Blister care

Avoid skin trauma

Avoid sun exposure

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