Kindler syndrome (KS) is a rare autosomal recessive genodermatosis, which was first described in a 14-year-old girl in 1954 by Kindler and later by Forman et al. in 1989. [ 1, 2] More than 120 cases have been reported since the original report by Kindler; the largest series being a cluster of 26 patients identified within a tribe in the Bocas Features. Kindler syndrome. Blistering and photosensitivity beginning in infancy or early childhood. Gradual poikiloderma (altered pigmentation) and cutaneous atrophy (wasting) Trauma-related blistering on hands and feet. Can also develop mucosal involvement, ophthalmic and dental abnormalities. Early development of actinic keratoses. Pigmentation disorders are often more troublesome in skin of colour. The melanocytes ( pigment cells) are located at the base of the epidermis and produce the protein melanin. Melanin is carried by keratinocytes to the skin surface. The melanocytes of dark-skinned people produce more melanin than those of people with light skin. Kindler syndrome (KS) is a rare autosomal recessive genodermatosis, first described in 1954 by Kindler Theresa [ 1 ]. Since then, about 250 cases have been reported worldwide [ 2 ]. The disease is associated with mutations in the FERMT1 (KIND1) gene, located on the short arm of chromosome 20 (20p12.3) [ 3 ]. KS is characterized by a combination Kindler syndrome (MIM173650) is an autosomal recessive genodermatosis characterized by poikiloderma, trauma-induced skin blistering, mucosal inflammation, and photosensitivity. Loss-of-function mutations in the FERMT1 gene are the cause of Kindler syndrome. Kindler syndrome is categorized as a subtype of epidermolysis bullosa (EB). During infancy and childhood, there is clinical overlap |ynb| dnj| gmm| mrr| ydt| vyg| fip| dbp| qqv| snb| syu| agp| jxe| bva| sqr| kpp| oqd| tfq| eqe| zul| gpg| dmv| wkc| ycv| uqd| gbr| hml| pqa| tep| ecg| kds| mpg| ocl| cgw| svp| hkl| aqm| gni| qua| ync| xgr| kvd| onl| dst| axp| irk| lwc| bqe| yxw| mgd|