Repair of Alar Defects Using the Alar Rotation Flap: Our Experience with 394 Patients.

BACKGROUND: The purpose of this study was for the authors to describe their patient selection, surgical technique, and results with the alar rotation flap for surgical defects of the nasal ala. METHODS: The authors performed a retrospective analysis of all alar rotation flaps performed between June of 2006 and February of 2019. Three hundred ninety-four patients were identified, and follow-up encounters were reviewed to assess for complications and need for revision procedures. RESULTS: The alar rotation flap was performed on 394 patients over a 13-year period. The mean defect size was 9.3 ± 2.8 mm by 7.2 ± 2.3 mm. Three hundred nineteen patients (81 percent) were evaluated postoperatively, with a mean average duration of follow-up of 2.3 years (range, 6 days to 11.9 years). Complications included hemorrhagic crust along the incision line [n = 9 (3 percent)], flap edema [n = 7 (2 percent)], internal nasal valve dysfunction [n = 3 (1 percent)], depressed surgical scar [n = 2 (1 percent)], hematoma [n = 1 (0.5 percent)], and paresthesia [n = 1 (0.5 percent)]. CONCLUSION: The alar rotation flap is a reliable one-stage flap for small- to medium-size partial-thickness defects of the nasal ala that can produce topographic restoration with minimal risk of aesthetic or functional complication. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Simulation: An Effective Method of Teaching Cosmetic Botulinum Toxin Injection Technique.

BACKGROUND: Learning to inject botulinum toxin for cosmetic purposes is difficult for beginners, given the nature of the procedure and patient population. Simulation training is an effective modality for medical professionals to acquire skills in an environment that provides low stress and ample opportunity for questions and correction of mistakes. OBJECTIVES: Compare posttraining comfort, knowledge, and practical botulinum toxin injection scores among trainees who underwent simulation vs video training only. METHODS: A total of 20 nurse practitioners, physician assistants, and resident physicians underwent cosmetic botulinum toxin injection training either through lecture and video, or lecture and hands-on simulation training. Comfort, knowledge, and practical test scores were recorded and compared between the groups. RESULTS: There was no evidence of a statistically significant difference in comfort or knowledge scores between simulation and video groups. The median (range) practical score was significantly higher in the simulation group compared to the video group (59.0 [31-60] vs 44.5 [27-57]; P < 0.01). CONCLUSIONS: Despite feeling similarly comfortable and having similar written knowledge test scores, the trainees who underwent simulation training had significantly higher hands-on practical test scores compared to trainees who underwent video training only for cosmetic botulinum toxin injections.

Bi-allelic Alterations in AEBP1 Lead to Defective Collagen Assembly and Connective Tissue Structure Resulting in a Variant of Ehlers-Danlos Syndrome.

AEBP1 encodes the aortic carboxypeptidase-like protein (ACLP) that associates with collagens in the extracellular matrix (ECM) and has several roles in development, tissue repair, and fibrosis. ACLP is expressed in bone, the vasculature, and dermal tissues and is involved in fibroblast proliferation and mesenchymal stem cell differentiation into collagen-producing cells. Aebp1-/- mice have abnormal, delayed wound repair correlating with defects in fibroblast proliferation. In this study, we describe four individuals from three unrelated families that presented with a unique constellation of clinical findings including joint laxity, redundant and hyperextensible skin, poor wound healing with abnormal scarring, osteoporosis, and other features reminiscent of Ehlers-Danlos syndrome (EDS). Analysis of skin biopsies revealed decreased dermal collagen with abnormal collagen fibrils that were ragged in appearance. Exome sequencing revealed compound heterozygous variants in AEBP1 (c.1470delC [p.Asn490_Met495delins(40)] and c.1743C>A [p.Cys581∗]) in the first individual, a homozygous variant (c.1320_1326del [p.Arg440Serfs∗3]) in the second individual, and a homozygous splice site variant (c.1630+1G>A) in two siblings from the third family. We show that ACLP enhances collagen polymerization and binds to several fibrillar collagens via its discoidin domain. These studies support the conclusion that bi-allelic pathogenic variants in AEBP1 are the cause of this autosomal-recessive EDS subtype.

Intraoperative Immunostaining for Cytokeratin-7 During Mohs Micrographic Surgery Demonstrates Low Local Recurrence Rates in Extramammary Paget's Disease.

BACKGROUND: Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy with high recurrence rates following standard surgical treatments, ranging from 22% to 60% in large retrospective reviews. OBJECTIVE: To evaluate the local recurrence rate of Mohs micrographic surgery (MMS) supplemented with intraoperative immunohistochemistry for cytokeratin-7 (MMS + CK-7) for primary and recurrent EMPD. MATERIALS AND METHODS: Retrospective, multi-center, cross-sectional study of patients treated using MMS + CK-7. Demographic, clinicopathologic, treatment, and follow-up data were obtained by chart review. RESULTS: The observed local recurrence rate for MMS + CK-7 is 3.3% (2/61 tumors) with a mean follow-up of 43.5 months (1-120 months). Local recurrence occurred in 2.3% (1/43) of primary tumors and 5.6% (1/18) of recurrent tumors. Kaplan-Meier 5-year tumor-free rates are 94.6% overall, 97.1% for primary tumors, and 80.0% for recurrent tumors. The Kaplan-Meier 5-year tumor-free rates for all EMPD tumors treated with MMS + CK-7 versus a historical cohort of MMS alone are 94.6% versus 72.0% (p = .012). CONCLUSION: MMS + CK-7 is an effective treatment for EMPD, demonstrating improved outcomes compared with historical controls.

Polarized transilluminating dermoscopy: Bedside trichoscopic diagnosis of trichothiodystrophy.

Trichothiodystrophy is a rare autosomal recessive disorder resulting in a broad range of systemic abnormalities. Polarizing microscopy of the hair reveals the pathognomic "tiger tail" of alternating light and dark bands, but the need for a microscope prevents rapid bedside diagnosis. We describe a new technique for the bedside diagnosis of trichothiodystrophy using a handheld polarizing dermatoscope, precluding the need for microscopic examination.

Reducing unnecessary testing: an intervention to improve resident ordering practices.

PURPOSE OF THE STUDY: To reduce the number of unnecessary laboratory tests ordered through a measurement of effects of education and cost awareness on laboratory ordering behaviour by internal medicine residents for common tests, including complete blood cell count (CBC) and renal profile (RP), and to evaluate effects of cost awareness on hospitalisation, 30-day readmission rate and mortality rate. STUDY DESIGN: 567 patients admitted during February, March and April 2014 were reviewed as the control group. Total CBC, CBC with differential and RP tests were counted, along with readmission and mortality rates. Interventions were education and visual cost reminders. The same tests were reassessed for 629 patients treated during 12 months after intervention in 2015. RESULTS: Data showed a significant increase in CBCs ordered after the intervention (mean number per hospitalisation changed from 1.7 to 2.3 (p<0.001)), a decrease in CBCs with differential (mean number changed from 1.7 to 1.2 (p<0.001)) and no change in RPs ordered (mean number, 3.7 both before and after intervention (p=0.23)). No change was found in mortality rate, but the decrease in the readmission rate was significant (p=0.008). CONCLUSIONS: Education in the form of cost reminders did not significantly reduce the overall ordering of the most common daily laboratory testing in our academic teaching service. We believe further research is needed to fully evaluate the effectiveness of other education forms on the redundant ordering of tests in the hospital setting.

Clinical features of shiitake dermatitis: a systematic review.

Shiitake dermatitis is a rare cutaneous reaction to lentinan, a polysaccharide component in the cell walls of shiitake mushrooms (Lentinula edodes). Herein, we systematically review the case report and case series English-language literature on shiitake dermatitis, which refers to a total of 50 patients (38 males, 12 females; mean age: 44.58 years). The majority of cases occurred after the consumption of raw mushrooms, whereas 22% of cases were caused by the eating of lightly or undercooked mushrooms. The most common clinical presentations, localized symptoms, and systemic findings include linear flagellated dermatitis (98%), pruritus (78%), and fever, diarrhea, and mucosal ulcers, respectively. The diagnosis of this entity continues to be based on clinical findings as laboratory abnormalities, and the findings of skin biopsies and patch/prick tests are nonspecific and inconsistent. The condition is self-limiting, resolving in approximately 12.5 d without treatment. Based on the included case reports, it appears that medical treatment may slightly shorten the course of disease (to 9-11 d, varying by therapy) but should be considered on an individual patient basis. However, the treatment of symptoms, reassurance, and the avoidance of re-exposure are sufficient treatment recommendations for this condition.

The changing face of complicated infantile hemangioma treatment.

Infantile hemangiomas are the most common vascular tumors of infancy. A multidisciplinary approach including dermatologists, otolaryngologists, plastic surgeons, hematologists/oncologists and interventional/diagnostic radiologists is crucial for appropriate management of children with complicated infantile hemangiomas. Since its unforeseen discovery in 2008, propranolol has become the first-line treatment for infantile hemangiomas, eclipsing systemic corticosteroids and radiologic intervention. There are still, however, uncommon indications for more aggressive interventional management. We review the 2014-updated International Society for the Study of Vascular Anomalies (ISSVA) classification for vascular anomalies. Additionally, we suggest management algorithms for complicated lesions, including recommendations for radiologic and surgical intervention.

Pediatric melanomas often mimic benign skin lesions: A retrospective study.

BACKGROUND: Childhood melanoma can be misdiagnosed because of its rarity and atypical presentation. OBJECTIVE: We sought to correlate the clinical appearance of pediatric melanomas with Breslow depth and clinical behavior, and to identify diagnostic errors made by dermatologists and nondermatologist physicians. METHODS: This was a retrospective review of Mayo Clinic records of children and young adults 21 years of age or younger with a diagnosis of primary cutaneous melanoma between January 2000 and January 2015. RESULTS: Pediatric melanomas that mimicked benign skin lesions were more often deeper (>1 mm; odds ratio 5.48; P = .002) and had a higher T stage (odds ratio [T2, T3, or T4] 6.28; P = .001) than melanomas with a clinically malignant appearance. Of pediatric melanomas, 66% originally diagnosed as benign melanocytic lesions exhibited changes in size, shape, and color. LIMITATIONS: Sample size and retrospective design are limitations. CONCLUSIONS: Benign-appearing pediatric skin lesions with a history of evolution, bleeding, or ulceration should raise suspicion for melanoma. Melanomas demonstrating these features are associated with a higher Breslow depth and T stage. Although biopsy of all lesions that exhibit change in children is not practical, safe, or desired, close monitoring is recommended.

Steroid hormone influence on melanomagenesis.

Disparities in the prognosis and incidence of melanoma between male and female patients have led clinicians to explore the influence of steroid hormones on the development and progression of this malignancy. A better understanding of the disparities of melanoma behavior between sexes and ages could lead to improved prevention and treatment options. There are multiple themes in the literature that unify the physiologic functions of estrogen and androgen receptors; herein we discuss and map their pathways. Overall, it is important to understand that the differences in melanoma behavior between the sexes are multifactorial and likely involve interactions between the immune system, endocrine system, and environment, namely UV-radiation. Melanoma deserves a spot among hormone-sensitive tumors, and if tamoxifen is re-introduced for future therapy, tissue ratios of estrogen receptors should be obtained beforehand to assess their therapeutic predictive value. Because androgens, estrogens, and their receptors are involved in signaling of commonly mutated melanoma pathways, potential synergistic properties of the recently developed molecular kinase inhibitors that target those pathways may exist.

Triggering Receptor Expressed on Myeloid Cells in Cutaneous Melanoma.

The tumor microenvironment plays an important role in the progression of melanoma, the prototypical immunologic cutaneous malignancy. The triggering receptor expressed on myeloid cells (TREM) family of innate immune receptors modulates inflammatory and innate immune signaling. It has been investigated in various neoplastic diseases, but not in melanoma. This study examines the expression of TREM-1 (a proinflammatory amplifier) and TREM-2 (an anti-inflammatory modulator and phagocytic promoter) in human cutaneous melanoma and surrounding tissue. Indirect immunofluorescence staining was performed on skin biopsies from 10 melanoma patients and staining intensity was semiquantitatively scored. Expression of TREM-1 and TREM-2 was higher in keratinocytes than melanoma tissue (TREM-1: p < 0.01; TREM-2: p < 0.01). Whereas TREM-2 was the dominant isoform expressed in normal keratinocytes, TREM-1 expression predominated in melanoma tissue (TREM-1 to TREM-2 ratio: keratinocytes = 0.78; melanoma = 2.08; p < 0.01). The increased TREM ratio in melanoma tissue could give rise to a proinflammatory and protumor state of the microenvironment. This evidence may be suggestive of a TREM-1/TREM-2 paradigm in which relative levels dictate inflammatory and immune states, rather than absolute expression of one or the other. Further investigation regarding this paradigm is warranted and could carry prognostic or therapeutic value in treatment for melanoma.

Methamphetamine use is an independent predictor of poor outcome after aneurysmal subarachnoid hemorrhage.

BACKGROUND: Clinical outcomes of methamphetamine users with aneurysmal subarachnoid hemorrhage (aSAH) are unknown. OBJECTIVE: To analyze differences in presentation, in-hospital morbidity, and outcomes between methamphetamine users and non-users. METHODS: All 472 patients included in the Barrow Ruptured Aneurysm Trial from 2003 to 2007 were reviewed. Patients with 1- and 3-year follow-up were included in this analysis (n=398). Methamphetamine users were identified as patients who provided a history of methamphetamine use on admission or tested positive on urine toxicology testing. Methamphetamine users were compared with non-users using univariate analysis. Outcomes were then analyzed using multivariate logistic regression models for demographic characteristics, medical comorbidities, radiographic and clinical presentation, and vasospasm. RESULTS: Thirty-one patients (7.8%) were identified as methamphetamine users in this cohort. Methamphetamine users were younger than non-users (mean age 42.8 vs 55 years, p<0.001). In multivariate logistic regression models, methamphetamine use was an independent predictor of poor Glasgow Outcome Scale score at both 1 year (OR=5.02; 95% CI 1.03 to 24.48; p<0.05) and 3 years (OR=7.18; 95% CI 1.73 to 29.87; p=0.007). Other independent predictors in this model included older age, clinical vasospasm, diabetes, and aneurysm size. Cocaine and tobacco use were not significantly associated with poor outcome in our cohort. Methamphetamine use was not significantly associated with vasospasm, higher Fisher or Hunt and Hess grade, or intraparenchymal hemorrhage/intraventricular hemorrhage. CONCLUSIONS: Methamphetamine users have significantly worse outcomes at 1 and 3 years following aSAH. Further analysis is necessary to understand the pathological response associated with methamphetamine use in this setting.