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
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