OnabotulinumtoxinA improves oral aperture in patients with scleroderma: A small clinical trial.

BACKGROUND: Reduced oral aperture (ROA), resulting from systemic sclerosis (SSc), is a debilitating condition with limited treatment options. Improvement in oral function has been reported with perioral administration of botulinum toxin type A. OBJECTIVE: To prospectively evaluate the efficacy of onabotulinumtoxinA (onabotA) injection in improving oral opening and quality of life in SSc patients with ROA. METHODS: Seventeen women with SSc and ROA were treated with 16 units of onabotA in 8 different sites around the cutaneous lips. Measurements of maximum mouth opening were taken before treatment, at 2 weeks posttreatment, and at 3 months posttreatment. Function and quality of life were also assessed via surveys. RESULTS: Interincisor and interlabial distances were significantly increased 2 weeks after treatment with onabotA (P < .001) but not 3 months after. Subjective improvement in quality of life was noted. LIMITATIONS: This single-institution study enrolled 17 patients and did not have a placebo control group. CONCLUSION: OnabotA appears to have a strong short-term symptomatic benefit in patients with ROA due to SSc, with possible benefit to quality of life.

Novel use of poly-L-lactic acid filler for the treatment of facial cutaneous atrophy in patients with connective tissue disease.

BACKGROUND: Patients with connective tissue disease (CTD) often suffer from facial cutaneous defects and resultant facial asymmetry. Unfortunately, these issues have been known to be difficult-to-treat, and concern exists regarding the use of cosmetic procedures in this patient population due to the theoretical risk of disease flare-up or reactivation. Injectable poly-L-lactic acid (PLLA) is one type of filler that has been used to treat skin atrophy in patients with morphea and lupus erythematous panniculitis. However, overall, there is a dearth in literature regarding the safety and efficacy of PLLA filler in patients with CTDs. AIMS: This case series intends to evaluate the safety and efficacy of PLLA filler in treating facial atrophy in patients with CTDs. PATIENTS/METHODS: Three patients underwent various treatment courses involving the use of PLLA filler to treat facial atrophy. RESULTS: Two patients demonstrated significant improvement in facial atrophy following their treatment course. No patient experienced reactivation or exacerbation of their CTD following PLLA injection. CONCLUSION: PLLA filler appears to have good viability as a safe and potentially effective treatment for facial atrophy in patients with CTDs.

Safety and Efficacy of Dermal Fillers in Patients With Connective Tissue Disease: A Review.

BACKGROUND: Dermal fillers such as hyaluronic acid, poly-l-lactic acid, and polymethyl-methacrylate are often used to treat cutaneous defects such as lipoatrophy. However, concern exists regarding their use in patients with a connective tissue disease (CTD) because of a theoretical risk of disease reactivation or exacerbation. Evidence regarding their use in patients with CTD also remains limited. OBJECTIVE: This review intends to summarize and evaluate the available literature regarding the use of dermal fillers in patients with CTD. MATERIALS AND METHODS: A literature search until May 2020 was conducted through PubMed, Ovid MEDLINE, and Ovid Embase to identify articles discussing the treatment of cutaneous defects secondary to CTD. Articles discussing the use of autologous fat transfer alone were excluded. RESULTS: Twenty-three articles were reviewed. The amount of available evidence varies between the type of CTD and type of filler with morphea having the most published evidence out of the CTDs discussed and hyaluronic acid having the most published evidence out of the fillers discussed. Most studies demonstrated positive results with no report of disease reactivation or exacerbation. CONCLUSION: Despite limited available evidence, dermal fillers seem to be safe as an adjunctive treatment for cutaneous defects in patients with CTD.

Review of the Current Medical and Surgical Treatment Options for Microstomia in Patients With Scleroderma.

BACKGROUND: Most patients with scleroderma suffer from microstomia, which can have debilitating consequences on their quality of life. Unfortunately, treatment options remain limited. No specific guidelines exist; hence, microstomia remains a challenge to treat in this patient population. OBJECTIVE: This review aims to evaluate the different medical and surgical treatment modalities currently available for microstomia in patients with scleroderma and make recommendations for future research. MATERIALS AND METHODS: A search of PubMed, Ovid MEDLINE, and Ovid Embase was conducted to identify articles discussing the treatment of microstomia in scleroderma. Twenty articles discussing surgical therapy and one article discussing medical therapy were reviewed. RESULTS: Mostly because of a scarcity of high-level evidence, no individual therapy has documented long-term efficacy. Some treatments demonstrate positive results and warrant further research. CONCLUSION: Given the variability of results, specific recommendations for the treatment of microstomia in patients with scleroderma are difficult to establish. A multifaceted approach that includes surgical and medical therapy is likely the best option to improve oral aperture in this patient population. Surgical treatments such as neurotoxins, autologous fat grafting, and ultraviolet A1 phototherapy may hold the most potential for improvement.

Scented lotions may cause scaring and premature fading of tattoos.

Although tattoo artists provide tattoo aftercare instructions to their clients, recommendations are often not cost-effective or supported by evidence. A 22-year-old man developed a pruritic red rash over his healing tattoo one week after receiving the tattoo. Although multiple queries were negative, the patient did note use of a scented lotion before the eruption. We determined that allergic contact dermatitis from the scented lotion caused scarring and premature fading of the new tattoo. Tattoo artists should recommend avoidance of scented lotions and instruct clients to care for their new tattoo like a wound in their aftercare instructions.

Aftercare Instructions in the Tattoo Community: An Opportunity to Educate on Sun Protection and Increase Skin Cancer Awareness.

Tattoo studios provide a unique venue for primary and secondary skin cancer prevention. Most tattoo artists promote sun protection for new tattoos in the form of aftercare instructions. Unfortunately, most tattoo artists are not well-informed on comprehensive sun safety recommendations, such as applying sunscreen prior to sun exposure with a sun protective factor (SPF) of 30 or higher, reapplying sunscreen when outdoors for more than two hours, using clothing that physically blocks ultraviolet rays, wearing wide-brimmed hats that shade the head, or seeking shade when available. However, recent evidence suggests that tattoo artists are open to learning about comprehensive sun safety recommendations and secondary skin cancer prevention methods. Tattoo studios also offer an opportunity to reach younger adults who may not be exposed to public health information or have access to health care. In addition to providing aftercare instructions to their clients, tattoo artists can disseminate sun safety information through social media and tattoo studio websites. Tattoo studios might serve as a an effective intervention environment to provide comprehensive full-body skin cancer prevention recommendations. Current recommendations to protect new tattoos from the sun should be compatible with the current sun safety guidelines from the American Academy of Dermatology.

Hispanic Tattoo Artists Could Provide Skin Cancer Prevention via Aftercare Instructions and Social Media

BACKGROUND: The incidence of melanoma and non-melanoma skin cancer in the Hispanic population has increased. Hispanics are more likely to present with advance-staged melanoma and worse overall prognosis. Thus, public health campaigns are necessary to target the underrepresented Hispanic population. OBJECTIVE: To explore Hispanic tattoo artists' skin cancer knowledge, sun safety recommendations, and their willingness to implement primary and secondary skin cancer prevention in their daily work routines. METHODS: We conducted an in-depth semi-structured interview study with ten Hispanic tattoo artists. Interviews were conducted at multiple tattoo studios in Salt Lake City, Utah.Data was coded by a third-party. Thematic analysis identified recurrent sub-themes from the transcript. RESULTS: Majority of Hispanic tattoo artists had a high percentage of Hispanic clientele (mean: 51%, range: 25-93%) and repeat customers (mean: 73%, range: 50-90%). All tattoo artists had suboptimal skin cancer knowledge. Most Hispanic tattoo artists provide inadequate sun protective information in their aftercare instructions including a specific Sun Protection Factor, sunscreen reapplication, and protective clothing. However, all tattoo artists were willing to provide sun protective information on their social media profiles and undergo primary and secondary skin cancer prevention training. CONCLUSION: Hispanic tattoo artists could serve as public health allies and influence early detection of skin cancers in the Hispanic population by implementing preventative skin cancer behaviors in their daily work routines and providing comprehensive sun safety information through aftercare instructions and social media. J Drugs Dermatol. 2019;18(12):1237-1243.

Histopathologic vasculitis from the periulcer edge: A retrospective cohort study.

BACKGROUND: Histopathologic vasculitis is often reported in periulcer specimens, but the frequency and clinical significance of this finding have not been evaluated. OBJECTIVE: We evaluated the sensitivity, specificity, negative predictive value, and positive predictive value of histopathologic vasculitis from the periulcer edge for detecting ulcers due to cutaneous vasculitis. METHODS: We performed a retrospective chart review of patients with leg ulcers at a tertiary hospital between 2009 and 2016. Histopathologic slides were evaluated by 2 dermatopathologists who were blinded to the etiology of ulcer. Focal vasculitis was defined as involvement of fewer than 3 vessels. RESULTS: Vasculitis at the periulcer edge was seen in 51.6% of the specimens (32 of 62). Of the specimens with histopathologic vasculitis, focal vasculitis was seen in the majority of specimens (71.9% [23 of 32]), whereas diffuse vasculitis was observed in 28.1% (9 of 32). Periulcer vasculitis yielded a high sensitivity (100% [95% confidence interval, 29%-100%]). Furthermore, the specificity was low (50.9% [95% confidence interval, 38.1%-63.6%]) for detecting vasculitis-induced ulcers. LIMITATIONS: Small number of vasculitis-induced ulcers. CONCLUSION: Focal vasculitis from the periulcer edge is a nonspecific finding and provides little diagnostic value in determining the etiology of lower leg ulcers. Emphasis should be placed on the combination of clinical history and examination, histology, and laboratory findings when diagnosing ulcers.

Localization and age distribution of telangiectases in children and adolescents with hereditary hemorrhagic telangiectasia: A retrospective cohort study.

BACKGROUND: The location of telangiectases in hereditary hemorrhagic telangiectasia (HHT), as set forth in the consensus diagnostic (Curaçao) criteria, is based primarily on adults. OBJECTIVE: Document the locations and numbers of telangiectases in a cohort of pediatric patients with HHT. METHODS: A retrospective chart review using a standardized data collection form for site and number of telangiectases was performed for pediatric patients with HHT (age, 0-18 years) from 2005 to 2016. RESULTS: Of 90 pediatric patients with HHT, 71% had one or more telangiectases. Of all the telangiectases counted (N = 319), cutaneous telangiectases were more common (73%) than oral telangiectases (27%). The hands were the most frequent site, accounting for 33% of all telangiectases. Adolescents were more likely than children to have cutaneous telangiectases (85% vs 50% [Q = 0.005]). The most frequent sites in children younger than 10 years were the hands excluding the fingers (27%), fingers (25%), and face (23%). Only 23% of subjects (21 of 90) presented with multiple (≥3) telangiectases at locations considered characteristic for the current consensus diagnosis guidelines (lips, oral cavity, and fingers). LIMITATIONS: Ascertainment bias based on recruitment. CONCLUSIONS: In this pediatric population, telangiectases at sites not included as "characteristic" by the Curaçao diagnostic criteria were common. The Curaçao criteria in regard to both number and location of telangiectases may be inadequate in the pediatric HHT population.

Epistaxis in children and adolescents with hereditary hemorrhagic telangiectasia.

OBJECTIVES/HYPOTHESIS: Our objective was to describe epistaxis onset and severity in pediatric hereditary hemorrhagic telangiectasia (HHT) patients and study the cumulative incidence of epistaxis by age of onset within each genetic subtype. STUDY DESIGN: Retrospective cohort chart review. METHODS: Charts were reviewed of patients age 0 to 18 years with a clinical or genetic diagnosis of HHT who were evaluated at a tertiary multidisciplinary HHT clinic from January 2010 to June 2016. The epistaxis severity score (ESS), a validated tool for assessing epistaxis severity, was used to assess epistaxis. Statistical analyses were conducted on the full HHT cohort as well as subgroups stratified by the HHT causative gene (HHT1 = ENG and HHT2 = ACVRL1). RESULTS: Sixty-nine pediatric subjects were identified; 60 had HHT confirmed by genetic testing, and nine (from families with known mutations) met published clinical diagnostic criteria alone. Fifty-nine (85%) had onset of epistaxis. The median age of onset of epistaxis was 5 years (interquartile range [IQR]: 2-9 years). The median ESS for the entire cohort was 1.6 (IQR: 0-2.6). The median ESS was higher in HHT1 versus HHT2 (2.3 vs. 1.1, P = .002), and age of epistaxis onset was earlier in HHT1 (3 vs. 5 years, P = .03). Sex and age were not associated with ESS. CONCLUSIONS: Epistaxis may present early in HHT, but is typically mild in the pediatric period. Severity in the pediatric population is worse in patients with HHT1. By recognizing the significance of even mild, infrequent epistaxis in a child with a family history of HHT, and understanding that not all HHT patients have epistaxis during childhood, community providers and otolaryngologist can assist in the early detection of HHT. LEVEL OF EVIDENCE: 4 Laryngoscope, 128:1714-1719, 2018.

Use of Spreader Flaps Without Dorsal Hump Reduction and the Effect on Nasal Function.

IMPORTANCE: Internal nasal valve (INV) collapse is a common cause of nasal obstruction, and spreader grafts are the established standard of treatment. Recently, spreader flaps have gained attention in the treatment of nasal valve stenosis when performed in conjunction with dorsal hump reduction. To date, the efficacy of the spreader flap technique without dorsal hump reduction has not been previously described. OBJECTIVE: To determine whether spreader flaps are equivalent to spreader grafts in correcting INV collapse in the absence of simultaneous dorsal hump reduction. DESIGN, SETTING, AND PARTICIPANTS: This retrospective medical record review included 26 patients with nasal obstruction and INV collapse who underwent correction with spreader flaps or spreader grafts concurrently with septoplasty and inferior turbinate reduction but without dorsal hump reduction. The type of graft placed was based on surgeon preference and patient anatomy. Patients were treated at a tertiary academic medical center from September 1, 2012, through August 31, 2014, and had follow-up of at least 6 months. Follow-up was completed for this study on August 12, 2016. MAIN OUTCOMES AND MEASURES: All patients completed the Nasal Obstruction Symptom Evaluation (NOSE) questionnaire preoperatively and at 1, 3, and 6 months postoperatively. The latest postoperative NOSE score was compared with the preoperative score. RESULTS: Among the 26 patients included (12 men and 14 women; mean age, 38.4 years [range, 18-64 years]), 13 underwent spreader flap placement and 13 underwent spreader graft placement. No difference was found between patient demographic characteristics or mean (SD) preoperative NOSE score (spreader flap group, 81.9 [15.8]; range, 72.4-91.4; spreader graft group, 75.4 [19.3]; range, 63.7-87.1) between groups. In addition, no difference was found in mean (SD) postoperative NOSE score (spreader flap group, 18.5 [21.6]; range, 5.4-31.5; spreader graft group, 16.9 [16.4]; range, 7.0-26.8), total NOSE score improvement (spreader flap group, 63.5 [23.5]; range, 49.3-77.7; spreader graft group, 58.5 [27.8]; range, 41.7-75.3), or percentage of improvement in NOSE score (spreader flap group, 78.0% [23.8%]; range, 63.6%-92.4%; spreader graft group, 76.02% [26.31%]; range, 60.1%-91.9%) between groups. CONCLUSIONS AND RELEVANCE: In appropriately selected patients, spreader flaps are equivalent to spreader grafts in correcting nasal obstruction secondary to INV collapse. LEVEL OF EVIDENCE: 3.

Long-term survivors of childhood bone and soft tissue sarcomas are at risk of hospitalization.

BACKGROUND: Childhood cancer survivors can have a high burden of chronic conditions related to cancer treatment, some of which are debilitating or potentially life-threatening. Much remains to be learned about late effects in bone and soft tissue sarcoma survivors. PROCEDURES: The Utah Cancer Registry was used to identify survivors of bone (N = 71) and soft tissue sarcomas (N = 98) who were diagnosed at ages 0-20 years between 1973 and 2007 and were alive at least 5 years after diagnosis. We selected an age-sex-matched comparison cohort (N = 934). Hospitalizations from 1996 to 2012 were extracted from the Utah Department of Health statewide inpatient hospitalization discharge records. Cox, Poisson, and Gamma regressions were used to evaluate the risk of hospitalization, rate of admission, and length of stay for survivors versus the comparison cohort. Primary ICD-9 codes defined the most common reasons for hospitalizations. RESULTS: The hazard ratio (HR) of any hospitalization was higher for survivors in reference to the comparison cohort (HR = 2.12, 95% confidence interval [CI] 1.51-2.97). Survivors experienced more hospital admissions (rate ratio [RR] = 4.58, 95% CI 3.92-5.35) and longer length of stay (RR = 1.28, 95% CI 1.12-1.46) compared with the comparison cohort. Survivors treated with any chemotherapy were at three-fold higher risk (HR = 3.37, 95% CI 1.94-5.83) of hospitalization compared with survivors who received surgery and/or radiation alone. Among hospitalized survivors, the most common reason was injury for bone tumor (26.8%) and neoplasm for soft tissue sarcoma (12.2%). CONCLUSION: Childhood survivors of bone tumor and soft tissue sarcoma face ongoing risk of hospitalization for years after diagnosis.

The Functional and Structural Outcomes of Arthroscopic Iliopsoas Release.

BACKGROUND: Arthroscopic release of the iliopsoas tendon may alleviate pain associated with internal snapping hip, but previous reports of physical function, hip strength, and muscle atrophy after surgery are mixed. HYPOTHESIS: The hips of patients who underwent arthroscopic iliopsoas release would demonstrate significantly reduced hip flexion strength and iliopsoas muscle volume when compared with their contralateral hips and the hips of patients who underwent hip arthroscopy without psoas release. STUDY DESIGN: Cohort study; Level of evidence, 3. METHODS: Eighteen patients who underwent hip arthroscopy with iliopsoas release for symptomatic internal snapping hip and concomitant femoroacetabular impingement (FAI) and/or chondrolabral damage (release group) and 18 patients who underwent arthroscopy for FAI and/or chondrolabral damage without iliopsoas release (control group) were evaluated at a mean of 21 months (range, 16-30 months) postoperatively. Magnetic resonance images were performed and segmented to calculate iliopsoas volume. Isometric hip flexion strength was evaluated in the supine and seated positions with a custom testing apparatus. Differences between groups and differences between the operative and nonoperative limbs within groups were compared with unpaired and paired t tests, respectively. RESULTS: In the release group, the iliopsoas muscle of the surgical limb was significantly smaller (288 ± 98 vs 384 ± 113 cm(3), P < .001) and weaker in the seated position (13 ± 4.7 vs 17 ± 5.8 kg, P < .001) than the contralateral limb. Compared with the control group, the release group demonstrated a greater percentage decrease in iliopsoas volume on magnetic resonance imaging (-25% ± 9.1% vs -0.6% ± 4.6%, P < .001) and seated hip flexion strength (-19% ± 16% vs -3.9% ± 20%, P = .018) between the operative and contralateral limbs. There were no significant differences in supine strength between limbs or groups (all P > .168). CONCLUSION: Arthroscopic iliopsoas release results in iliopsoas atrophy with a 25% volume loss and a 19% reduction in seated hip flexion strength.

Complex nontuberculous mycobacterial cervicofacial lymphadenitis: What is the optimal approach?

OBJECTIVES/HYPOTHESIS: Assess the role of combined antimicrobial and surgical therapy for difficult-to-treat nontuberculous mycobacteria (NTM) lesions of the head and neck in children. STUDY DESIGN: Retrospective cohort. METHODS: Retrospective review of pediatric head and neck NTM lesions at a tertiary children's hospital from 1999 to 2012. RESULTS: Seventy-one children were diagnosed with NTM lesions. Age of presentation ranged between 7 and 204 months of age. Most patients (62%) had multiple lesions. Treatments included incision and drainage, curettage, antibiotics, excision, and any combination of surgery and antibiotics. Upon initial presentation, the most common treatment was surgical excision alone (n = 34) with a high complication rate (50%). In 18 cases, patients were initially treated with a combination of antibiotics and surgical excision due to the extent or location of the lesion(s). Complication rate in these patients was also high (67%). The most common complications in surgically excised NTM lesions included temporary or persistent facial nerve dysfunction (24.6%), poor wound healing/scarring (10.8%), and Frey's syndrome (6.2%). CONCLUSIONS: Surgical excision with or without medical therapy for NTM cervicofacial lymphadenitis in high-risk regions commonly resulted in marginal mandibular nerve dysfunction (24.6%). Postoperative facial nerve weakness generally resolved within a year. High rates of complications and a lack of proven best approaches suggest tailoring the approach to address the potential risks in that particular patient based on location and severity. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:1677-1680, 2016.