[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
Validation

Diseases presenting "renal failure" symptom

cholangiocarcinoma

congenital diaphragmatic hernia

cystinuria

erdheim-chester disease

fabry disease

heparin-induced thrombocytopenia

homocystinuria without methylmalonic aciduria

junctional epidermolysis bullosa

kindler syndrome

legionellosis

oculocutaneous albinism

primary hyperoxaluria type 1

scrub typhus

systemic capillary leak syndrome

This symptom has already been validated