Kindler syndrome.

[kindler syndrome]

Kindler syndrome is a rare genodermatosis characterized by acral bullae and photosensitivity . The photosensitivity improves with advancing age and results in progressive poikiloderma and cutaneous atrophy , and many additional features have also been described . This report describes two male Kindler syndrome patients with classical features of acral blistering and photosensitivity in childhood , and subsequent development of poikiloderma , leukokeratosis of oro-ano-genital mucosae , phimosis and meatal stenosis . The first patient had additional ophthalmic features of chronic simple conjunctivitis caused by persistent irritation , multiple stromal nebular corneal opacities and thickened corneal nerves . The second patient showed skeletal changes , namely a dome-shaped skull (turri-cephaly ), bifid fourth rib , missing fifth rib , short fourth and fifth metacarpals and mandibular abnormalities . This is the first report of such ophthalmic and skeletal features of Kindler syndrome .