Kindler syndrome

Kindler syndrome is a rare genodermatosis characterized by acral bullae and photosensitivity

• photosensitivity improves with advancing age and results in progressive poikiloderma and cutaneous atrophy
• classical features include acral blistering and photosensitivity in childhood, and subsequent development of poikiloderma, leukokeratosis of oro-ano-genital mucosae, phimosis and meatal stenosis
• additional features noted in two case reports include:
  • in one patient additional ophthalmic features of chronic simple conjunctivitis caused by persistent irritation, multiple stromal nebular corneal opacities and thickened corneal nerves
  • in one patient skeletal changes, namely a dome-shaped skull (turri-cephaly), bifid fourth rib, missing fifth rib, short fourth and fifth metacarpals and mandibular abnormalitie

Reference:

1. Sharma JC et al. Kindler syndrome. Int J Dermatol. 2003 Sep;42(9):727-32