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.

Optimizing Palliative Focal Radiation Therapy Dose in Cutaneous T-Cell Lymphoma: How Low Can You Go?

PURPOSE: Primary cutaneous T-cell lymphomas (CTCLs) are radiosensitive tumors with variable and often relapsing courses. Local disease can be treated with low-dose focal palliative radiation therapy (RT), though little data supports the use of a specific dose. This study assesses clinical outcomes after focal RT to a total dose of 4 Gy, 8 Gy, or 12 Gy. METHODS AND MATERIALS: An International Review Board-approved, retrospective, single-institution study was performed of 225 lesions in 41 patients with primary CTCL treated with low-dose focal RT from 2015 to 2020. Patient, tumor, and treatment characteristics were reviewed. The primary outcome was freedom from treatment failure (FFTF), defined as time to requiring local retreatment, and secondary outcomes included response rates and toxicities. RESULTS: Of the 225 lesions, 90 received 4 Gy, 106 received 8 Gy, and 29 received 12 Gy. Lesions treated with 12 Gy (96%) or 8 Gy (92%) had a significantly higher 1-year FFTF compared with 4 Gy (77%) (P = .034). Overall response rate and complete response rate were not significantly different between different doses (P = .117), though there was a trend toward higher overall response rate at initial assessment with 8 Gy versus 4 Gy (91.5% vs 82.2%, P = .057). Toxicity was low, with 7.1% of lesions having grade 2 or higher radiation dermatitis. CONCLUSIONS: In primary CTCL lesions treated with focal palliative RT, a dose response was noted favoring 8 to 12 Gy, with 1-year FFTF rates over 90%. However, 4 Gy resulted in substantially better outcomes than previously reported, with 77% requiring no further treatment at 1 year and comparable response rates to higher doses. While our data substantiates 8 to 12 Gy as the standard of care, it also suggests that 4 Gy should be considered an acceptable alternative in situations with concern for radiation toxicities, such as with fragile or heavily pretreated skin.

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.

Chronic lymphocytic leukemia skin infiltrates presenting as eyebrow alopecia and erythematous pruritic papules refractory to treatment with topical steroids.

We describe a patient who presented with erythematous papules and hair loss solely limited to the eyebrows. Clinicians should be aware of this presentation of leukemia cutis and consider this diagnosis in a patient with a history of CLL.

Wrinkles, brown spots, and cancer: Relationship between appearance- and health-based knowledge and sunscreen use.

BACKGROUND: There is increasing understanding that sunscreen use is associated with reduced photoaging and that appearance-based messages are more effective than health-based messages in promoting daily sunscreen use. We hypothesized that persons with greater awareness of the effects of photoaging use sunscreen more consistently. METHODS: Cross-sectional survey of adults in Dallas, TX, assessing sunscreen-related knowledge, behavior, and information source. RESULTS: Each respondent averaged 2.5 ± 1.7 hours of self-reported sun exposure daily with the least reported time being 0.15 hours. Eighty-eight percent reported that sun exposure caused skin cancer "a lot." Eighty-nine percent "agreed" or "strongly agreed" that "sun exposure causes most signs of skin aging." Over 70% "agreed" or "strongly agreed" to an appearance-based reason for their sunscreen use. Sixty-six percent reported they would consider using sunscreen if anticipating sun exposure lasting 2 hours or less. Only 30% reported using sunscreen daily. Greater photoaging knowledge was positively associated with sunscreen adherence (at least P < 0.05 with each knowledge item). Those with darker complexions or skin less likely to burn reported less knowledge of photoaging (ρ = -0.219, P ≤ 0.001) and had lower sunscreen use (ρ = 0.301, P < 0.001). CONCLUSION: Despite most respondents reporting knowledge of health and appearance risks of sun exposure and a motivation to prevent them with sunscreen, most do not use sunscreen adequately. This discrepancy could be due to people perceiving risks of sun exposure based on duration of continuous exposure. There may be misconceptions that short-interval sun exposure that does not result in sunburn is less harmful than extended exposure.

Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.

The efficacy of alemtuzumab for the treatment of refractory Sézary syndrome (SS) versus other third-line agents such as pralatrexate and gemcitabine is poorly characterized. To elucidate the effectiveness of alemtuzumab versus other third-line options for the treatment of refractory SS, we conducted a meta-analysis of existing data. A systematic review was performed in March 2017 based on a search using Ovid-MEDLINE® and OVID-EMBASE® for articles evaluating single-agent alemtuzumab, gemcitabine, or pralatrexate for the treatment of SS and mycosis fungoides (MF). Twenty-two publications were identified that fulfilled all search criteria (total n = 323 patients), with six publications of lower quality being excluded from our analysis in order to decrease the risk of bias (final: n = 308 patients; 93 with SS and 147 with MF). Across all studies, alemtuzumab was significantly more effective in patients with SS (overall response rate [ORR]: 81%; complete response rate [CRR]: 38%) than patients with MF (ORR: 29%; CRR: 8%). However, gemcitabine was more effective than alemtuzumab or pralatrexate in treating MF. Alemtuzumab-treated patients had more frequent side effects, which were influenced by route of administration and dose. There was a lower incidence of lymphopenia and other serious adverse events in patients treated with subcutaneous (38%) compared to intravenous regimens (68%), and lower-dose (5%) compared to high-dose alemtuzumab regimens (54%). No significant differences were found in the effectiveness of different routes of administration or dosing regimens. Our review supports the use of low-dose subcutaneous alemtuzumab as a third-line treatment for SS.

The Sign of Leser-Trelát and Malignant Acanthosis Nigricans Associated With Fallopian Tube Carcinoma.

BACKGROUND: The sign of Leser-Trelát is controversial and rarely reported in gynecologic malignancies. It is characterized by rapid development of new or enlarging seborrheic keratoses. CASE: A 78-year-old woman presented with unintentional weight loss and new-onset erythematous patches and plaques with thickened, rugated skin and stuck-on brown waxy papules on the chest and back. Her symptoms were concerning for a paraneoplastic eruption; workup revealed an elevated CA 125 level and an ovarian mass on abdominal computed tomography scan. Exploratory laparotomy revealed a stage IIIC serous fallopian tube carcinoma and a synchronous low-grade endometrioid adenocarcinoma of the endometrium. CONCLUSION: The sign of Leser-Trelát can be associated with fallopian tube carcinoma. When recognized, paraneoplastic dermatoses can prompt clinicians to initiate a workup for occult malignancy.

A case of calciphylaxis and chronic myelomonocytic leukemia.

A 70-year-old woman presented for evaluation of symmetric necrotic ulcers of the lower extremities. Biopsy results revealed changes consistent with calciphylaxis. The predisposing factors in this patient included calcium supplementation, obesity, female gender, viscous blood, renal failure, and diabetes mellitus. To our knowledge, this is the first report of calciphylaxis occurring in the setting of chronic myelomonocytic leukemia. We discuss the history, clinical presentation, diagnosis, and treatment of calciphylaxis.

Preparedness of health care practitioners to screen women for domestic violence in a border community.

Health care professionals do not always screen patients for domestic abuse. In the absence of screening, the likelihood of misdiagnoses and prescriptions contraindicated for symptoms of abuse survivors is increased. Several factors may contribute to the lack of routine and uniform screening, including a lack of educational preparedness. The qualitative findings presented here are part of a larger quantitative study investigating the skills, beliefs, and expectations about screening for domestic abuse among physicians, dentists, and nurse practitioners from a border community in southwest Texas. Practitioners requested specific information to enhance educational preparedness and improve clinical practice for their abused patients.