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188宝金博页面版: Vitiligo-like depigmentation with perifollicular pigment retention in systemic sclerosis treated successfully with suplatast tos

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内容提示: 110 EJD, vol. 26, n ? 1, January-February 2016In 1992, a 60-year-old man was referred to the dermatol-ogy unit at Barts Health. Other urology teams had knownhim since birth due to congenital hypospadias, which hadledtorepeatedreconstructionsurgeriesandacircumcision.Histology from circumcisional surgery was never elicited.In later adult life, he developed anterior urethral strictures.His urologists diagnosed LS of the (residual) penile skinin 1989 and he was subsequently managed topically withemollients an...

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110 EJD, vol. 26, n ? 1, January-February 2016In 1992, a 60-year-old man was referred to the dermatol-ogy unit at Barts Health. Other urology teams had knownhim since birth due to congenital hypospadias, which hadledtorepeatedreconstructionsurgeriesandacircumcision.Histology from circumcisional surgery was never elicited.In later adult life, he developed anterior urethral strictures.His urologists diagnosed LS of the (residual) penile skinin 1989 and he was subsequently managed topically withemollients and moderately potent topical steroids. He wasreferred to the dermatology department at Barts Health in1992 and was managed with super-potent topical steroidsto the LS involving the penile skin. This led to the resolu-tion of symptoms but residual post inf l ammatory atrophyof the penile skin. No further biopsies of the penile skinwere taken, so although the LS was considered ‘burnedout’, this was unvalidated by biopsy. At this stage, therewas no LS involving the perineal skin. However in 2003,the patient developed symptoms and signs of LS aroundhis perineal urostomy site and his scrotum. Again, this wasmanaged with super-potent topical steroids and emollientsand his symptoms (itching and burning of the skin) set-tled and the disease appeared not to advance in surfacearea affected. In 2006, areas of hyperkeratosis were notedat his urethrostomy site and neoplasia was suspected. Aperi-urethrostromy punch biopsy in 2007 conf i rmed thediagnosis of scrotal LS with hyperkeratosis but no fea-turesofneoplasia.By2008,ourpatientwasbeingmanagedfor persistent urinary incontinence around the periurethralmeatus and advancing signs of LS together with furtherclinical signs of hyperkeratosis around the peri-urethralorif i ce, and signs of LS involving the left posterior cruralfold (f i gure 1A). Intra-epithelial malignancy of the peri-urethral skin was considered but a repeat punch biopsy(f i gures 1B, C) showed hyperkeratosis, parakeratosis andhydropic degeneration of basal cells, along with subepider-mal oedema and homogenization of upper dermal collage,consistent with the diagnosis of LS, but no evidence ofdysplasia or malignancy. It was not until 2010, when thepatient underwent suprapubic urostomy for his urinaryincontinence, that this led to signif i cant improvement in hisLS control, with resolution of the once active lesions andresolution of the perineal hyperkeratosis which mimickedneoplastic change.LS is known in men particularly to affect the glans penisand foreskin and only rarely affects the ano-perineal areas(as opposed to women, where ano-crural LS is much morecommon). Our patient had LS of the penile and (probably)A BCFigure 1. A) Hyperkeratosis around the peri-urethral orif i ceand signs of LS involving the left posterior crural fold. Low(B) and high (C) power magnif i cation, Hematoxylin and eosinstain.prepucial skin when his external urethral meatus was nearthe glans penis. Following a repair of his hypospadias andthe formation of a perineal urethrostomy, his LS symptomssettledontheresidualglansanddevelopedaroundthesurgi-cally reconstructed perineal urethral opening. The repeatedareas of hyperkeratosis were clinically diff i cult to differ-entiate from white sclerotic skin and neoplasia and were aconstantclinicalworry.Punchbiopsiesrepeatedlyruledoutmalignant changes. Importantly, our patient’s perineal andcruralLS(togetherwiththeclinicallyconcerninghyperker-atosis and white sclerotic skin) settled following formationof a suprapubic urostomy. We believe that our patient’sclinical history supports the theory [4] that it was urinaryincontinencewhichactedasanirritantandtriggeredhisLS.Finally, our patients LS was only better controlled whenhe had a suprapubic urostomy formed, and the perinealincontinence was resolved.Our case demonstrates further evidence that it is chronicurinary irritation of skin which may lead to the develop-ment of male ano-genital LS. Also, it is well recognizedthat LS is associated with the development of skin malig-nancy. Our report indicates that persistent urinary irritationmay lead to clinically worrying hyperkeratosis. Finally,our case shows that, whilst persistent incontinence exists,treatment with even super-potent topical steroids can bediff i cult. ?Disclosure. Financial support: none. Conf l ict of interest:none.1 Department of Dermatology,2 Department of CutaneousMedicine & Surgery, Barts HealthNHS Trust, Aneurin Bevan House,81 Commercial Road,London E11RD,UK<Anthony.Bewley@bartshealth.nhs.uk>Bernard CHI-LOK HO 1Rino CERIO 2Anthony BEWLEY 11. Fistarol S, Itin P. Diagnosis and treatment of lichen sclerosus: anupdate. Am J Clin Dermatol 2013;14:27-47.2. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Der-matologists’ guidelines for the management of lichen sclerosus 2010.Br J Dermatol 2010;163:672-82.3. Shim TN, Andrich DE, Mundy AR, Bunker CB. Lichen sclerosusassociated with perineal urethrostomy. Br J Dermatol 2013:169.4. Bunker CB, Shim TN. Male genital lichen sclerosus. Indian J Der-matol 2015;60:111-7.doi:10.1684/ejd.2015.2683Vitiligo-like depigmentation with peri-follicular pigment retention in systemicsclerosistreatedsuccessfullywithsuplatasttosilateA 26-year-old woman noticed swollen f i ngers and depig-mentation on her chest and back three months previously

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