Kindler syndrome.
نویسندگان
چکیده
Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma. The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome, Cockayne syndrome, dyskeratosis congenita, epidermolysis bullosa, Rothmund-Thomson syndrome and xeroderma pigmentosum. Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication.
منابع مشابه
سندرم Kindler و ارتباط آن با اپیدرمولیز بولوز سیمپلکس: گزارش سه مورد از بیمارستان رازی
Kindler syndrome is characterized by acral blister formation in infancy and childhood, poikiloderma and cutaneous atrophy. Undoubtedly, similarities of the clinical features exist between Kindler syndrome and Epidermolysis bullosa simplex with mottled pigmentation. In this article, we report 3 patients with Kindler syndrome. Until the Bullous component of Kindler syndrome is more completely und...
متن کاملKindler Syndrome: 2 Case Reports from India
Kindler syndrome (KS) is a rare autosomal recessive genodermatosis. We report two cases of KS with classical clinical presentations involving skin and mucus membranes. Clinically, both patients had four major features of KS in the form of acral skin blistering, photosensitivity, progressive poikiloderma, and diffuse cutaneous atrophy. Case 1 had associated features in the form of urethral...
متن کاملReport of a Case Report of Kindler Syndrome
A 13 years old boy with progressive poikiloderma, bullous lesions in the extremities and photosensitivity is reported. Physical examinations were otherwise normal, his physical development was normal, no other family member had a similar disease. Routin laboratory exams were in the normal limits.Histopathology exams from poikilodermatous skin showed atrophy of the epidermis, liquification degen...
متن کاملKindler Syndrome: A case Report from Iran
Kindler syndrome (KS) is a rare, autosomal recessive genodermatosis characterized by skin blistering and photosensitivity in infancy, progressive poikiloderma, and diffuse cutaneous atrophy. It affects the skin, mucous membranes, and oral cavity and is caused by mutations in the KIND1 gene on 20p12.3. The first case of KS associated with periodontitis was reported in 1996, and have been infrequ...
متن کاملClinical and microbiologic study of periodontitis associated with Kindler syndrome.
BACKGROUND Little is known about the onset and prevalence of periodontal disease in patients with the rare Kindler syndrome, a genodermatological disorder. This study investigated the level of clinical periodontal attachment in relation to age and presence of putative periodontopathogenic bacteria in individuals with Kindler syndrome. METHODS Eighteen individuals diagnosed with Kindler syndro...
متن کاملGastrointestinal Manifestation of Kindler Syndrome
1. Kindler T. congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy. Br J Dermatol 1954; 66: 104-11. 2. Yazdanfar A, Hashemi B. Kindler syndrome: report of three cases in a family and a brief review. Int J Dermatol 2009; 48:145-52. 3. Penagos H, Jaen M, Sancho MT, Saborio MR, Fallas VG, Siegel DH, et al. Kindler syndrome in Native Americans from Panama: report...
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ورودعنوان ژورنال:
- Indian journal of dermatology, venereology and leprology
دوره 71 5 شماره
صفحات -
تاریخ انتشار 1998