04Jun 2018

Lymphoedème primaire et psoriasis: similitude étiopathogènique et espoirs thérapeutiques

  • Service de dermatologie-v?n?rologie, H?pital d?instruction Mohammed V, Rabat-Maroc.
  • Abstract
  • Keywords
  • References
  • Cite This Article as
  • Corresponding Author

Lymphedema is a chronic and debilitating pathology due to lymphatic stasis, responsible for an increase in the volume of the affected limb. Primary and secondary lymphedemas are distinguished. The former are due to a primitive anomaly of the lymphatic vessels (hereditary or not), the latter to their obstruction or destruction. Primary lymphedemas are classified as congenital, early and late by age of onset. Apart from functional and psychological repercussions, erysipelas remains the main complication, aggravating lymphatic circulatory deficit. The treatment is based on physical methods, resection surgery or reconstruction, and drugs (diuretic type, benzopyrones, coumarinic and flavonoids). These treatments are sometimes heavy or insufficient, and the therapeutic hope lies in gene therapy. We report here the case of psoriasis occurring in a patient presenting a primary lymphedema.


  1. Rockson, S.G., and Rivera, K.K. (2008). Estimating the population burden of lymphedema. Ann. N Y Acad. Sci. 1131, 147?154
  2. Ferrell, R.E., Levinson, K.L., Esman, J.H., Kimak, M.A., Lawrence, E.C., Barmada, M.M., and Finegold, D.N. (1998). Hereditary lymphedema: evidence for linkage and genetic heterogeneity. Hum. Mol. Genet. 7, 2073?2078.
  3. Karkkainen, M.J., Ferrell, R.E., Lawrence, E.C., Kimak, M.A., Levinson, K.L., McTigue, M.A., Alitalo, K., and Finegold, D.N. (2000). Missense mutations interfere with VEGFR-3 signalling in primary lymphoedema. Nat. Genet. 25, 153?159.
  4. Fang, J., Dagenais, S.L., Erickson, R.P., Arlt, M.F., Glynn, M.W., Gorski, J.L., Seaver, L.H., and Glover, T.W. (2000). Mutations in FOXC2 (MFH-1), a forkhead family transcription factor, are responsible for the hereditary lymphedema-distichiasis syndrome. Am. J. Hum. Genet. 67, 1382?1388.
  5. Finegold, D.N., Kimak, M.A., Lawrence, E.C., Levinson, K.L., Cherniske, E.M., Pober, B.R., Dunlap, J.W., and Ferrell, R.E. (2001). Truncating mutations in FOXC2 cause multiple lymphedema syndromes. Hum. Mol. Genet. 10, 1185 1189
  6. Irrthum, A., Devriendt, K., Chitayat, D., Matthijs, G., Glade, C., Steijlen, P.M., Fryns, J.P., Van Steensel, M.A., and Vikkula, M. (2003). Mutations in the transcription factor gene SOX18 underlie recessive and dominant forms of hypotrichosis-lymphedema-telangiectasia. Am. J. Hum. Genet. 72, 1470?1478.
  7. Finegold, D.N., Schacht, V., Kimak, M.A., Lawrence, E.C., Foeldi, E., Karlsson, J.M., Baty, C.J., and Ferrell, R.E. (2008). HGF and MET mutations in primary and secondary lymphedema. Lymphat. Res. Biol. 6, 65?68
  8. Alders, M., Hogan, B.M., Gjini, E., Salehi, F., Al-Gazali, L., Hennekam, E.A., Holmberg, E.E., Mannens, M.M., Mulder, M.F., Offerhaus, G.J., et al. (2009). Mutations in CCBE1 cause generalized lymph vessel dysplasia in humans. Nat. Genet. 41, 1272?1274.
  9. Connell, F., Kalidas, K., Ostergaard, P., Brice, G., Homfray, T., Roberts, L., Bunyan, D.J., Mitton, S., Mansour, S., Mortimer, P., and Jeffery, S.; Lymphoedema Consortium. (2010). Linkage and sequence analysis indicate that CCBE1 is mutated in recessively inherited generalised lymphatic dysplasia. Hum. Genet. 127, 231?241
  10. Partanen TA, Arola J, Saaristo A, Jussila L, Ora A, Miettinen M, et al. VEGF-C and VEGF-D expression in neuroendocrine cells and their receptor, VEGFR-3 in fenestrated blood vessels in human tissues. FASEB J 2000 ; 14 : 2087-96. 2000 ; 14 : 2087-96.
  11. Valtola R, Salven P, Heikkila P, Taipale J, Joensuu H, Rehn M, et al. VEGFR-3 and its ligand VEGF-C are associated with angiogenesis in breast cancer. Am J Pathol 1999 ; 154 : 1381- 90.
  12. Capon M.Munro J.Barker R.Trembath: Searching for the major histocompatibility complex psoriasis susceptibility gene. J Invest Dermatol1182002745751.
  13. Trembath RC, Clough RL, Rosbotham JL, Jones AB, Camp RD, Frodsham A, et al. Identification of a major susceptibility locus on chromosome 6p and evidence for further disease loci revealed by a two stage genome-wide search in psoriasis. Hum Mol Genet 1997;6:813?20.
  14. Bowcock AM, Krueger JG. Getting under the skin: the immunogenetics of psoriasis. Nat Rev Immunol 2005;5:699?711
  15. Viac J, Palacio S, Schmitt D, Claudy A. Expression of vascular endothelial growth factor in normal epidermis, epithelial tumors and cultured keratinocytes. Arch Dermatol Res 1997;289:158?63
  16. Karkkainen MJ, Saaristo A, Jussila L, Karila KA, Lawrence EC, Pajusola K, et al. A model for gene therapy of human hereditary lymphedema. Proc NatlAcad Sci USA2001 ; 98 : 12677- 82.

[Jawad El-azhari, Naoufal Hjira and Mohammed Boui. (2018); Lymphoedème primaire et psoriasis: similitude étiopathogènique et espoirs thérapeutiques Int. J. of Adv. Res. 6 (Jun). 18-22] (ISSN 2320-5407). www.journalijar.com


El-azhari jawad, Naoufal Hjira, Mohammed Boui
Service de dermatologie-vénérologie, Hôpital d’instruction Mohammed V, Rabat-Maroc

DOI:


Article DOI: 10.21474/IJAR01/7182      
DOI URL: https://dx.doi.org/10.21474/IJAR01/7182