[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases].

[kindler syndrome]

Kindler 's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia . We report three new sibling cases of this rare syndrome .The condition was seen in three sisters aged 12 , 16 and 20 years , born of a first -degree consanguineous marriage with no family history of Kindler 's syndrome . The three patients presented spontaneously regressive bullous eruptions , poikilodermia of gradual onset , major cutaneous atrophy on the back of the hands and the feet , photosensitivity and gingival hypertrophy . Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage .Diagnosis of Kindler 's syndrome is based upon clinical evidence . Kidler 's syndrome is a well defined clinical entity . Ultra -structural studies show intraepidermal , junctional , and dermal cleavage . This syndrome must be differentiated from congenital epidermolysis bullosa , Weary 's syndrome , and other bullous hereditary poikilodermas .