Introduction: Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome.
Cases Reports: The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage.
Discussion: Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas.
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http://dx.doi.org/10.1016/j.annpat.2011.05.009 | DOI Listing |
BJPsych Open
December 2024
University Hospital of Child and Adolescent Psychiatry and Psychotherapy Bern, University of Bern, Switzerland.
Orphanet J Rare Dis
September 2024
Faculty of Health Sciences, University of Castilla-La Mancha, Talavera de la Reina, 45600, Spain.
Front Pediatr
September 2024
Laboratory of Genetic Metabolism Center, Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.
Front Oral Health
September 2024
Department of Oral and Maxillofacial Surgery, A B Shetty Memorial Institute of Dental Sciences, Nitte (Deemed to be University), Deralakatte, India.
Eur Heart J
July 2024
Cardiology Department, AP-HP, Hôpital Bichat, 46 Rue Henri Huchard, 75018 Paris, France.
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