[Kindler syndrome: three cases reports in three siblings].

[kindler syndrome]

Kindler syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia . We report three new cases of this rare syndrome .This condition was seen in two brothers aged 4 and 14 years and in their sister aged 6 years , born of a first -degree consanguineous marriage in a family with Kindler syndrome of varying degrees of severity . The three patients presented spontaneously regressive bullous eruptions , poikilodermia of gradual onset , major cutaneous atrophy on the back of the hands and the feet , pseudo-syndactyly and photosensitivity . The older brother presented a severe form complicated by chronic terminal renal failure with hydronephrosis secondary to urethral stenosis and hypertension as well as oesophageal stenosis requiring dilatation sessions . Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and proliferation of the basal layer . Electron microscopy examination of rubbed poikilodermic skin from the other two siblings showed cleavage at both the superficial and deep intra-epidermal levels .Diagnosis of Kindler syndrome is based upon clinical evidence . Electron microscopic examination is used in particular to rule out congenital bullous epidermolysis . Detachment of layers at two or three different levels in relation to the dermal-epidermal junction described in the literature forms a specific but inconsistent feature of Kindler syndrome .

Diseases
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Diseases presenting "major cutaneous atrophy on the back of the hands" symptom

kindler syndrome

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