Kindler syndrome: A rare case report

Kindler Syndrome (KS) is a rare hereditary disorder characterized by acral blistering of infancy and childhood, photosensitivity, progressive poikiloderma, and cutaneous atrophy. We report this case of KS in a 4 year old female child on account of its rarity Keywords: Kindler syndrome; acral blistering; pokiloderma; photosensitivity

Treatment of striae distensae: recent conceptions

Stretch marks (striae distensae) are linear areas of cutaneous atrophy that appear in regions of greatest stretch of the skin. Their formation is a complex multifactorial process, its etiopathogenesis has not been fully understood yet. Aesthetic correction of stretch marks is an urgent problem of modern dermatocosmetology due to their widespread prevalence and pronounced negative impact on the patient’s quality of life. The aim of this paper was to provide a review of the literature on current reported treatment options for stretch marks. The principles of the effect of various methods of treatment, their classification and data from clinical trials are considered.

DENTAL MANAGEMENT OF A PEDIATRIC PATIENT WITH KINDLER SYNDROME- A REVIEW

Kindler syndrome is a rare autosomal recessive genetic disorder which is a form of Epidermolysis Bullosa. It is characterized by skin fragility and acral blister formation beginning at birth. We hereby present a case of Kindler syndrome. The case is being reported for its rarity and a number of general features like blistering, cutaneous atrophy, and/or poikilodermatous skin changes and oral features such as gingival swelling, advanced periodontal bone loss, mild-to-severe gingivitis, dental caries, and leukokeratosis of buccal mucosa along with its management.

Kindler Syndrome : Clinical Particularities about Two Cases Reports in Two Siblings

The Kindler syndrome, the fourth major type of hereditary epidermolysis bullosa (HEB), is a rare autosomal recessive genodermatoses, characterized by trauma-induced blistering, cutaneous atrophy, and progressive poikiloderma, in association with mucosal inflammation. We report tow new sibling cases of this rare syndrome.

The Assessment of Polysaccharides, Mucosubstances and Colagen IV in Kindler Syndrome

Kindler syndrome is a rare autosomal recessive genodermatosis characterized by particular fragility of the skin and mucosa, manifesting in the appearance of bullae in minor traumas, photosensitivity, diffuse cutaneous atrophy and progressive poikiloderma. It is caused by mutations in the FERMT1 gene encoding kindlin-1. We present the case of a 12-year-old girl diagnosed with Kindler syndrome showing the classical clinical features affecting the skin, in addition to nail involvement, gingivitis, periodontitis, growth retardation, mental retardation, esophageal and anal stenosis. The histopathological examination of a cutaneous biopsy revealed the presence specific features of Kindler syndrome. Keywords: Kindler syndrome, photosensitivity, esophageal and anal stenosis, skin biopsy

Cutis marmorata telangiectatica congenita: a literature review

Background Cutis marmorata telangiectatica congenita (CMTC) is a rare capillary malformation characterised by persistent reticulated marbled erythema. It tends to be associated with cutaneous atrophy, ulcerations and body asymmetry. CMTC is usually reported to be a benign condition; however, associated anomalies are not rare. Here, we have compiled information on published CMTC patients with the aim to evaluate the proposed diagnostic criteria by Kienast et al. and address the clinical manifestations, associated anomalies, differential diagnoses, management and prognosis. Our review is based on a search of the PubMed database which retrieved studies between 1922 and April 2019. The search yielded 148 original articles with a total of 485 patients. Results Of the identified patients, 24.5% had generalised CMTC, 66.8% had localised and 8.7% had a non-specified distribution of CMTC. Associated anomalies were observed in 42.5% of patients, predominantly body asymmetry and neurological defects like seizure and developmental delay. Fewer patients (10.1%) had ophthalmological defects, usually glaucoma. The major criterium “absence of venectasia” was not met in 20.4% of patients. Conclusion We suggest that children with CMTC should be referred to an ophthalmologist for regular follow-up, and children with CMTC affecting the legs should be monitored for leg length discrepancy throughout the growth period. Furthermore, we suggest reconsideration of the major criterium “absence of venectasia” from the proposed diagnostic criteria, and instead include body asymmetry.