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5.
Pediatr Dermatol ; 34(3): 352-355, 2017 May.
Article in English | MEDLINE | ID: mdl-28523882

ABSTRACT

Phacomatosis pigmentokeratotica (PPK) is a rare epidermal nevus syndrome characterized by the co-occurrence of a nevus sebaceous arranged along the lines of Blaschko with a speckled lentiginous nevus (SLN). We report a novel KRAS mutation in a patient with a large nevus sebaceous and an SLN who subsequently developed a vaginal botryoid rhabdomyosarcoma, an association not previously reported in the literature. This case expands our knowledge of the genetic basis for phacomatosis, in which mutations in HRAS have been previously described, although this report provides evidence that activating mutations in KRAS or HRAS may cause PPK. This report confirms that PPK is a mosaic RASopathy with malignant potential and raises the question of whether screening for other RAS-associated malignancies should be performed for all children with PPK.


Subject(s)
Nevus, Pigmented/diagnosis , Proto-Oncogene Proteins p21(ras)/genetics , Skin Neoplasms/diagnosis , Female , Humans , Infant , Mosaicism , Mutation , Nevus, Pigmented/genetics , Skin Neoplasms/genetics
6.
J Am Acad Dermatol ; 75(1): 169-76, 2016 Jul.
Article in English | MEDLINE | ID: mdl-26944598

ABSTRACT

BACKGROUND: Full-thickness skin grafts (FTSGs) are a common repair option on the external ear, but there are few large case series examining graft sublocations, dimensions, and outcomes. OBJECTIVE: We sought to report our experience with FTSGs for repair of postsurgical defects of the external ear. METHODS: We conducted a retrospective review of all FTSGs on the ear performed by 2 surgeons (J. C., 2000-2014; B. C. L., 2007-2014) after clearance by Mohs micrographic surgery at a single institution. RESULTS: A total of 1519 FTSGs on the ear were performed between June 2000 and March 2014. The most common sublocations were the superior helix (38.8%), the crura of the antihelix or scapha (18.9%), and the back of ear/back of helix (15.4%). The overall complication rate was 1.6%, and the most common complication was graft failure (1.2%). LIMITATIONS: Data were collected retrospectively from a single institution. Follow-up beyond 3 months was limited. A standardized assessment tool for aesthetic outcomes was not performed. CONCLUSION: By taking advantage of predictable "pincushioning" and combining with local flaps or cartilage grafts, FTSGs can provide more volumetric replacement than previously described. They reliably preserve the height and complex topography of the ear with a low complication rate.


Subject(s)
Ear Auricle/surgery , Ear Deformities, Acquired/surgery , Ear Neoplasms/surgery , Skin Neoplasms/surgery , Skin Transplantation , Cartilage/transplantation , Dermatologic Surgical Procedures/adverse effects , Dermatologic Surgical Procedures/methods , Humans , Medical Illustration , Mohs Surgery , Photography , Retrospective Studies , Skin Transplantation/adverse effects
7.
Dermatol Surg ; 41(6): 667-76, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25984901

ABSTRACT

BACKGROUND: Antiseptics are chemical agents used to reduce the microbial population on the surface of the skin and are used in nearly every surgical procedure today. Despite this, there are currently no definitive guidelines on surgical preoperative antisepsis that indicate a specific regimen based on demonstration of superior efficacy. OBJECTIVE: This review serves to examine preoperative antisepsis, including cutaneous bacteriology, preoperative hair removal, preoperative decolonization, surgical attire, and the antiseptic agents themselves. MATERIALS AND METHODS: A review of the literature on surgical antiseptics was performed. RESULTS: Although numerous studies have demonstrated differences in bacterial colonization rates, few well-controlled investigations have demonstrated superiority of a given regimen. The alcohol-based iodophor and chlorhexidine products seem to exhibit greater efficacy than their aqueous counterparts. CONCLUSION: More randomized controlled trials will be needed to determine if any specific regimen is most effective. At this point in time, product usage should be based on specific attributes relating to the products, such as iodophors around the eyes and/or ears to avoid irritation and aqueous-based solutions in hair bearing areas because of concern for flammability. Ultimately, it is up to the individual surgeon to tailor the optimal antiseptic regimen for their specific scope of practice.


Subject(s)
Alcohols/therapeutic use , Anti-Infective Agents, Local/therapeutic use , Chlorhexidine/analogs & derivatives , Iodophors/therapeutic use , Preoperative Care , Surgical Wound Infection/prevention & control , Chlorhexidine/therapeutic use , Hair Removal , Humans , Skin/microbiology , Surgical Attire
8.
Radiat Prot Dosimetry ; 148(4): 444-51, 2012 Mar.
Article in English | MEDLINE | ID: mdl-21561946

ABSTRACT

The purpose of this study was to investigate how the choice of the scanned region affects organ doses in CT. ImPACT CT Patient Dosimetry Calculator (version 1.0) was used to compute absorbed doses to eight organs of interest in medical radiation dosimetry. For 13 dosimetry data sets, the authors calculated the maximum organ dose (D(max)) as well as the corresponding organ dose for a scan with selected length D(L). These data permitted the relative dose (D(r) = D(L)/D(max)) to be determined for varying scan lengths. Computations were performed for a nominal X-ray tube current of 100 mA, a rotation time of 1 s and a CT pitch of 1. The authors also determined values of D(max)/CTDI(vol), where CTDI(vol) is obtained in a 32-cm diameter CT dosimetry phantom using the same radiographic techniques. For each organ, D(r) was independent of the type of scanner, and increased monotonically to unity with increasing scan length. Relative doses for a scan restricted to the organ length ranged from 0.65 D(max) for the bladder to 0.86 D(max) for the lungs. There was good correlation (r = 0.64) between relative organ dose and the corresponding organ length. At 120 kV, the lowest value of D(max)/CTDI(vol) was 1.23 for the breast and the highest was 2.22 for the thyroid. Varying the X-ray tube voltage between 100 and 130 kV results in changes in D(max)/CTDI(vol) of no more than 4 %. CT scans limited to the direct irradiation of an average-sized organ results in an absorbed dose of ~0.75 D(max).


Subject(s)
Radiometry/methods , Tomography, X-Ray Computed/methods , Anthropometry/methods , Computer Simulation , Female , Humans , Male , Monte Carlo Method , Phantoms, Imaging , Radiometry/instrumentation , Risk , Software , Tissue Distribution , X-Rays
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