Kindler syndrome (hereditary acrokeratotic poikiloderma)

Epidemiology

• rare
• infants, children, may persist into adulthood

Genetics

• mutation in the KIND1 gene

Clinical manifestations

expression variable, 4 patterns suggested:

• vesicopustule formation remains confined to the hands & feet
  • begins 1-3 months of age, resolves in late childhood;
• widespread eczematoid dermatitis resembling atopic eczema
  • starts 3-6 months of age, completely resolves by age 5
• gradual appearance of diffuse poikiloderma with striate & reticulate atrophy
  • spares ace, scalp, & ears
  • persists into adulthood
• development of keratotic papules on the hands, feet, elbows, & knees
  • lesions first appear at varying times before 5 years of age & persist indefinitely.

More general terms

• genetic syndrome (multisystem disorder)
• poikiloderma

Additional terms

• KIND1 gene

Database

• OMIM: https://mirror.omim.org/entry/173650