A case is reported of a 28 year old man referring the appearance of swollen blisters due to insignificant trauma since birth. Later he noticed progressive changes of the skin as teleangectasies, atrophic spots, sensivity to sun, dystrohic fingermails and webbing between fingers.The patient was hospitalized several times for an appropriate diagnosis and asked for the permission to undergo an operation of plastic surgery in Paris to correct webbing of the hands.
The rare association of two congenital diseases, epidermolysis bullosa dystrophica and poikiloderma, leads to the diagnosis of a Kindler syndrome.
The Kindler Syndrome described by Theresa Kindler in 1954 is probably a variant of hereditary acrokeratotic Poikiloderma in which Poikiloderma is preceded by a tendency to blistering following traumatic blisters.
There are not many ultrastructural studies on this syndrome. The ultrastructural level of blister formation has not been well characterized. The cutis of the hand back of our patient has been examined at the electronic microscope and it shows a thinned epidermis with a normal keratinization and a compact corneous ortokeratosic layer. A dermo epidermic flaking is noticed in several points and at different levels.Since the first description in 1954 approximately 70 cases have been reported worldwide.
KINDLER SYNDROME (Abstract)
Prof. Camillo O. DI CICCO, M.D.
Presented to VIth Congress of the European Society for Pediatric Dermatology.
Courtesy of "DermAtlas-Johns Hopkins University"
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