Neutrophilic panniculitis associated with myelodysplastic syndrome/myeloproliferative neoplasm: a case report and literature review.

Introduction: Neutrophilic panniculitis (NP) is a rare subtype of neutrophilic dermatosis, a group of neutrophil-rich inflammatory skin disorders that can present in association with myeloid neoplasms. NP is defined by the presence of a neutrophilic infiltrate in the fat lobules of the subcutis in the absence of either infection or vasculitis. We herein describe a 65-year-old woman with a recent diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome (MDS/MPN) who abruptly developed painful, pruritic nodules consistent with NP. Case presentation: A 65-year-old woman with MDS/MPN presented for evaluation of painful and pruritic nodules on her upper and lower extremities. A biopsy revealed a lobular neutrophilic infiltrate in the subcutis without evidence of microorganisms or vasculitis. The patient was diagnosed with NP and treated with oral prednisone. Within 1 month of treatment, she reported complete resolution of the nodules. Discussion: Similar to other neutrophilic dermatoses, NP may arise in association with hematologic malignancies of myeloid origin, such as MDS/MPN. A literature review revealed that most cases of NP associated with MDS occur after the onset of MDS and respond to systemic corticosteroids, not antibiotics. Infection should be ruled out before initiating treatment with systemic steroids. Conclusion: Although the mechanism is still unknown, it is important for clinicians to be aware that NP is associated with MDS; thus, hematological malignancies should be investigated upon diagnosis of NP. Once diagnosed, NP is easily treated and has an excellent response to systemic corticosteroids.

Combination cryotherapy and intralesional corticosteroid versus steroid monotherapy in the treatment of keloids.

BACKGROUND: Keloids are common and have significant negative effects on quality of life. There is a need for more effective treatment approaches for keloids. AIMS: We investigated treatment outcomes of intralesional triamcinolone acetonide (IL TAC) compared with combination IL TAC and cryotherapy, including changes in pruritus, pain, and keloid size. PATIENTS/METHODS: We performed a prospective study of patients referred to one provider who treated patients with combination therapy and compared them to a historic control cohort treated with IL TAC alone. All patients were seen at Thomas Jefferson University between 2019 and 2021. Patient demographics, location of keloids, and inciting events were recorded. Pruritus and pain scores were self-reported by patients using a 10-point Likert scale administered as standard of care. Changes in keloid size were denoted as "No change," "up to 50% decrease," "more than 50% decrease," and "completely flattened." RESULTS: While both treatments produced a significant reduction in mean pruritus and pain scores, there was no difference between the two treatment groups (p = 0.3933 and p = 0.2123, respectively). A greater percentage of keloids in the combination therapy group had a post-treatment size difference greater than 50% compared with those in the IL TAC only treatment group (p = 0.0021). In the subgroup of pubic keloids, all lesions treated with combination IL TAC and cryotherapy responded remarkably well to treatment. CONCLUSIONS: While both IL TAC and IL TAC with cryotherapy were effective at reducing pruritus and pain, combination therapy was more effective in reducing keloid size, specifically for pubic keloids.

Keloid Disorder: Genetic Basis, Gene Expression Profiles, and Immunological Modulation of the Fibrotic Processes in the Skin.

Keloid disorder, a group of fibroproliferative skin disorders, is clinically comprised of keloids, hypertrophic scars, keloidalis nuchae, and acne keloidalis. The prototype of these disorders is keloids, which manifest as cutaneous lesions with excessive deposition of collagen following an initiating trauma of varying degrees. The principal cell type responsible for collagen accumulation is the myofibroblast, and its gene expression is modulated by a network of regulatory factors, including cytokines, growth factors, and noncoding RNA species. In addition, keloids harbor a number of inflammatory cells, including macrophages and mast cells, that interact with fibroblastic cells by direct contact or by paracrine actions. Transforming growth factor-ß1/Smad signaling regulates the expression of genes encoding extracellular matrix proteins and also controls cell proliferation and apoptosis. A key profibrotic molecule is the fibronectin splice variant cellular fibronectin extracellular domain A (cFN-EDA), which interacts with a number of cell-surface integrins and TLR4, contributing to the modulation of gene expression by lesional fibroblasts. Collectively, these complex cellular interactions result in accumulation of collagen with clinical development and growth of keloid lesions. Understanding of the precise pathomechanistic details of keloid formation will provide targets for pharmacological interference toward treatment of the keloid disorder, a group of currently difficult to treat skin diseases.

Omaveloxolone attenuates squamous cell carcinoma growth and disease severity in an Epidermolysis Bullosa mouse model.

Patients with epidermolysis bullosa (EB) are susceptible to development of squamous cell carcinomas (SCC) at sites of chronic inflammation and fibrosis. While triterpenoids such as RTA 408 (Omaveloxolone) have been shown to reduce inflammation and inhibit tumour growth in various cancer models, the utility of this class of drugs in the treatment of SCC has not been investigated. Given the dual anti-inflammatory and anti-neoplastic properties of triterpenoids, we hypothesized RTA 408 would be an effective treatment for SCCs that arise in the chronic inflammatory setting in EB. We tested the effects of topical RTA 408 on a mouse model of non-Herlitz, junctional EB. RTA 408 significantly reduced phenotypic severity in the affected ears of Lamc2jeb mice. In cultures, RTA 408 reduced cell viability in EB-associated SCC cell lines and normal human epidermal keratinocytes. When administered in vivo, RTA 408 inhibited SCC tumour growth in mice without cutaneous or systemic toxicity. These results suggest that RTA 408 can be a promising new therapy to reduce inflammation and inhibit SCC growth in patients with EB.

Mepivacaine versus Bupivacaine Spinal Anesthesia for Early Postoperative Ambulation.

BACKGROUND: Early ambulation after total hip arthroplasty predicts early discharge. Spinal anesthesia is preferred by many practices but can delay ambulation, especially with bupivacaine. Mepivacaine, an intermediate-acting local anesthetic, could enable earlier ambulation than bupivacaine. This study was designed to test the hypothesis that patients who received mepivacaine would ambulate earlier than those who received hyperbaric or isobaric bupivacaine for primary total hip arthroplasty. METHODS: This randomized controlled trial included American Society of Anesthesiologists Physical Status I to III patients undergoing primary total hip arthroplasty. The patients were randomized 1:1:1 to 52.5 mg of mepivacaine, 11.25 mg of hyperbaric bupivacaine, or 12.5 mg of isobaric bupivacaine for spinal anesthesia. The primary outcome was ambulation between 3 and 3.5 h. Secondary outcomes included return of motor and sensory function, postoperative pain, opioid consumption, transient neurologic symptoms, urinary retention, intraoperative hypotension, intraoperative muscle tension, same-day discharge, length of stay, and 30-day readmissions. RESULTS: Of 154 patients, 50 received mepivacaine, 53 received hyperbaric bupivacaine, and 51 received isobaric bupivacaine. Patient characteristics were similar among groups. For ambulation at 3 to 3.5 h, 35 of 50 (70.0%) of patients met this endpoint in the mepivacaine group, followed by 20 of 53 (37.7%) in the hyperbaric bupivacaine group, and 9 of 51 (17.6%) in the isobaric bupivacaine group (P < 0.001). Return of motor function occurred earlier with mepivacaine. Pain and opioid consumption were higher for mepivacaine patients in the early postoperative period only. For ambulatory status, 23 of 50 (46.0%) of mepivacaine, 13 of 53 (24.5%) of hyperbaric bupivacaine, and 11 of 51 (21.5%) of isobaric bupivacaine patients had same-day discharge (P = 0.014). Length of stay was shortest in mepivacaine patients. There were no differences in transient neurologic symptoms, urinary retention, hypotension, muscle tension, or dizziness. CONCLUSIONS: Mepivacaine patients ambulated earlier and were more likely to be discharged the same day than both hyperbaric bupivacaine and isobaric bupivacaine patients. Mepivacaine could be beneficial for outpatient total hip arthroplasty candidates if spinal is the preferred anesthesia type.

A review of principles in design and usability testing of tactile technology for individuals with visual impairments.

To lay the groundwork for devising, improving, and implementing new technologies to meet the needs of individuals with visual impairments, a systematic literature review was conducted to: a) describe hardware platforms used in assistive devices, b) identify their various applications, and c) summarize practices in user testing conducted with these devices. A search in relevant EBSCO databases for articles published between 1980 and 2014 with terminology related to visual impairment, technology, and tactile sensory adaptation yielded 62 articles that met the inclusion criteria for final review. It was found that while earlier hardware development focused on pin matrices, the emphasis then shifted toward force feedback haptics and accessible touch screens. The inclusion of interactive and multimodal features has become increasingly prevalent. The quantity and consistency of research on navigation, education, and computer accessibility suggest that these are pertinent areas of need for the visually impaired community. Methodologies for usability testing ranged from case studies to larger cross-sectional studies. Many studies used blindfolded sighted users to draw conclusions about design principles and usability. Altogether, the findings presented in this review provide insight on effective design strategies and user testing methodologies for future research on assistive technology for individuals with visual impairments.