Cutis
laxa is a rare disease, related to loss of skin elasticity, which can be
hereditary or acquired, with or without associated visceral damage. It is
marked by great psychological and social repercussions. The purpose of this
paper is to highlight a particular cause of neonatal occlusion: cutis laxa. We
report a new observation about a case of cutis laxa hospitalized in the
pediatric department at Mohammed V hospital in Tangier, admitted right after
birth for the management of macrosomia with wrinkled and inelastic skin,
suggesting the diagnosis of cutis laxa. The evolution that followed was marked
by the occurrence of several occlusive episodes of a functional nature.
Conclusion: visceral involvement in the cutis laxa is reported in several reviews.
In our patient the neonatal occlusion was most likely related to her disease.
The management of the case must be multidisciplinary.
References
[1]
Tassabehji, M., Metcalfe, K., Hurst, J., Aschcroft, G.S., Kielty, C., Wilmot, C., et al. (1998) An Elastin Gene Mutation Producing Abnormal Tropoelastin and Abnormal Elastic Fibres in a Patient with Autosomal Dominant Cutis Laxa. Human Molecular Genetics, 7, 1021-1028. https://doi.org/10.1093/hmg/7.6.1021
[2]
Hucthagowder, V., Sausgruber, N., Kim, K.H., Angle, B., Marmorstein, L.Y. and Urban, Z. (2006) Fibulin-4: A Novel Gene for an Autosomal Recessive Cutis Laxa Syndrome. The American Journal of Human Genetics, 78, 1075-1080.
https://doi.org/10.1086/504304
[3]
Marshall, J., Hey, T. and Weber, H.W. (1966) Postinflammatory Elastolysis and Cutis Laxa: A Report on a New Variety of This Phenomenon and a Discussion of Some Syndromes Characterized by Elastolysis. South African Medical Journal, 40, 1016.
[4]
Wolff, K., Goldsmith, L.A., Katz, S.I., Gilchrest, B.A., Paller, A. and Leffell, D.J. (2008) Heritable Disorders of Connective Tissue with Skin Changes. In Fitzpatrick’s Dermatology in General Medicine. 7th Edition, Mc Graw Hill, New York, pp. 1308-1310.
[5]
Rybojad, M., Baumann, C., Godeau, C., Moraillon, I., Prigent, F., Morel, P., et al. (1999) Cutis laxa gé né ralisé e congé nitale: Cinq cas. Annales de Dermatologie et de Vénéréologie, 126, 317-319.
[6]
Kardelen, F., Akcurin, G., Ertu, H., Tacoy, S. and Ciftcioglu, A. (2002) Supravalvular Aortic Stenosis in Cutis Laxa Syndrome: A Case Report. International Journal of Cardiology, 83, 167-168. https://doi.org/10.1016/S0167-5273(02)00028-1
[7]
Reed, W.B., Horowitz, R.E. and Beighton, P. (1971) Acquired Cutis Laxa. Archives of Dermatology, 103, 661-669.
https://doi.org/10.1001/archderm.1971.04000180087012
[8]
Elahi, E., Kalhor, R., Banihosseini, S.S., Torabi, N., Pour-Jafari, H., Houshmand, M., et al. (2006) Homozygous Missense Mutation in Fibulin-5 in an Iranian Autosomal Recessive Cutis Laxa Pedigree and Associated Haplotype. Journal of Investigative Dermatology, 126, 1506-1509. https://doi.org/10.1038/sj.jid.5700247
[9]
Benjemaa, H., Cheurier, S., Georgien, N., Pailheret, J.P. and Watier, E. (2002) Post Inflammatory Cutis Laxa. A Case Report. Annales de Chirurgie Plastique Esthétique, 47, 647-650. https://doi.org/10.1016/S0294-1260(02)00158-9
[10]
Beighton, P., Bull, J.C. and Edgerton, M.T. (1970) Plastic Surgery in Cutis Laxa. British Journal of Plastic Surgery, 23, 285-290.
https://doi.org/10.1016/S0007-1226(70)80057-1
[11]
Yedomon, H.G. and Do Ango-Padonou, F. (1990) Cutis laxa acquis postinflammatoire. Annales de Dermatologie et de Vénéréologie, 117, 547-548.
