Association of cutis laxa and genital prolapse: a case report.
Cutis laxa (CL) is an extremely inherited or acquired connective tissue disorder characterised by a markedly reduced systemic elastin content. Genital abnormalities in patients with CL have been rarely reported. We report such a case in a 48-year-old CL patient affected by genital prolapse, focusing on immunohistological and molecular biology assessment of elastin and collagen type I, III, VI content in the main uterine ligaments. The woman was referred to our department for the onset of a rapidly progressing genital prolapse and urinary incontinence. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and sacrocolpopexy. Punch biopsies from both cardinal and uterosacral ligaments revealed a dramatic reduction in elastin and an increase in collagen type VI content. The present report seems to underline the central role exerted primarily by elastin in the supportive connective tissue and might contribute to the knowledge of extracellular matrix abnormalities at the basis of genital abnormalities in CL patients.
Paladini
Dario
D
Department of Gynecology and Obstetrics and Pathophysiology of Human Reproduction, University of Naples Federico II, Via Pansini 5, Naples, Italy.
Di Spiezio Sardo
Attilio
A
Mandato
Vincenzo Dario
VD
Guerra
Germano
G
Bifulco
Giuseppe
G
Mauriello
Silvana
S
Nappi
Carmine
C
D001706:Biopsy; D003483:Cutis Laxa / Q000150:complications* / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008875:Middle Aged; D014596:Uterine Prolapse / Q000150:complications* / Q000378:metabolism / Q000473:pathology* / Q000601:surgery