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"
- PHYSICAL SCIENCES
- EARTH SCIENCES
- LIFE SCIENCES
- SOCIAL SCIENCES
Subscribe to the newsletter
Stay in touch with the scientific world!
Know Science And Want To Write?
- Quantum mechanics in 1834?
- Why This New "Planet X" Is No Threat To Earth :).
- Would New Planet X Clear Its Orbit? - And Any Better Name Than "Planet Nine"?
- Top Secret: On Confidentiality On Scientific Issues, Across The Ring And Across The Bedroom
- The Greenhouse Effect Fallacy
- Double Dose Of Bad Earthquake News
- From The Great Wall To The Great Collider
- "There are over a hundred cancers and since the primary cause is mutation, managing it is the best..."
- "I'm not sure whether we have less cancer than ever, but yes, it is much more manageable these days..."
- "My sloppiness. That’s a good point; it should read net energy retention in the system. Thanks...."
- "Agree with most of what you say, except for the phraseIt’s things like that which cause the confusion..."
- "The percentage of CO2 in the atm is irrelevant; it is the total mass of a given gas in the atm..."
- It’s Fat Tuesday! Have a paczki!
- Giving C-Section Babies Mom’s Germs to Improve Immunity
- California to Follow UK Lead on Embryonic Gene Editing
- (Most) Docs Listening To Task Force Recs Against PSAs: Not Urologists Though
- Spice of Life Can, Literally, Lead to Longer Life
- Keeping Babies Safe from ‘Tourniquet’ Hair
- Children with special health care needs and their families have high food insecurity risk
- Rare bumble bee may be making a comeback in Pacific northwest
- Paper: Homeownership a 'dream deferred' for millennial generation
- NASA data reveals tropical cyclone forming near Madagascar
- Mysterious menominee crack is unusual geological pop-up feature