Report of two rare cases of Kindlers syndrome in siblings

Kindler’s syndrome is a rare autosomal recessive disorder. It is charac-terized by trauma-induced blistering, photosensitivity, poikiloderma and mucosal inflammation. It occurs due to mutation on chromosome 20p. This report describes two siblings with history of blistering and photosensitivity in childhood and later developed poikiloderma with histopathology of effected skin showing features of poikiloderma.

[1]  J. McGrath,et al.  Kindler syndrome: a focal adhesion genodermatosis , 2009, The British journal of dermatology.

[2]  E. Bauer,et al.  The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. , 2008, Journal of the American Academy of Dermatology.

[3]  C. Herz,et al.  Novel and recurrent KIND1 mutations in two patients with Kindler syndrome and severe mucosal involvement. , 2007, Acta dermato-venereologica.

[4]  R. Phelps,et al.  Aggressive squamous cell carcinoma in Kindler syndrome. , 2006, Skinmed.

[5]  D. Siegel,et al.  Kindler syndrome in native Americans from Panama: report of 26 cases. , 2004, Archives of dermatology.

[6]  H. Feiler,et al.  Loss of kindlin-1, a human homolog of the Caenorhabditis elegans actin-extracellular-matrix linker protein UNC-112, causes Kindler syndrome. , 2003, American journal of human genetics.

[7]  J. Weissenbach,et al.  Identification of mutations in a new gene encoding a FERM family protein with a pleckstrin homology domain in Kindler syndrome. , 2003, Human molecular genetics.

[8]  M. Wygoda,et al.  Kindler syndrome complicated by squamous cell carcinoma of the hard palate: successful treatment with high‐dose radiation therapy and granulocyte–macrophage colony‐stimulating factor , 2001, The British journal of dermatology.

[9]  H. Larjava,et al.  Early-onset periodontitis associated with Weary-Kindler syndrome: a case report. , 1996, Journal of periodontology.

[10]  A. Hovnanian,et al.  Poikiloderma of Theresa Kindler: Report of a Case with Ultrastrectural Study, and Review of the Literature , 1989, Pediatric dermatology.

[11]  T. Kindler CONGENITAL POIKILODERMA WITH TRAUMATIC BULLA FOKMATION AND PROGRESSIVE CUTANEOUS ATROPHY. , 1954, The British journal of dermatology.