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"
- Clinical Phase IIb Study Of ATH008 In Hand-Foot Syndrome (Palmar-Plantar Erythrodysesthesia Syndrome)
- Birth Hormone May Control Expression Of Autism In Animals
- Unique Aspects Of Down Syndrome
- The Genetic Determinants Of Symptoms In A Rare Chromosomal Deletion Disorder
- Study: Seasonal Weight Changes Increase Odds For Metabolic Syndrome