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[Congenital cutis laxa: a case study]. | LitMetric

[Congenital cutis laxa: a case study].

Pan Afr Med J

Service de Pédiatrie, CHU Mohammed VI, Université Mohammed I, Oujda, Maroc.

Published: August 2020

"Cutis laxa" (CL) are rare elastic tissue disorders characterized by loose, sagging skin. They can be a congenital or acquired condition. Inherited cutis laxa is a heterogeneous group of disorders characterized by the severity of their visceral involvement and by their mode of transmission. Three groups have been identified on the basis of their genetic transmission: autosomal dominant, recessive autosomal, X-linked recessive. The severity of the visceral involvement affects the prognosis of inherited CL which is potentially fatal in the short term in patients with cardiac or pulmonary involvement. This study aims to remind clinicians of this rare affection through direct observation of an infant being followed-up for respiratory distress sixteenth days after birth.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7060907PMC
http://dx.doi.org/10.11604/pamj.2019.34.195.17110DOI Listing

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Cutis laxa is a genetically heterogeneous disorder characterized primarily by loose, redundant skin with abnormal wrinkling and elasticity. It is an exceptionally rare condition, with an estimated prevalence of < 1 in 1,000,000 individuals. In addition to the distinctive cutaneous manifestations, cutis laxa can present with a constellation of other features, including progeroid appearance, growth retardation, and developmental delays.

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