Engineered Skin Substitutes in Dermatologic Surgery: A Systematic Review.

BACKGROUND: Artificial skin substitutes are a flexible alternative to autografting in Mohs micrographic surgery (MMS), but the characteristics and clinical outcomes of skin substitutes are not well defined. OBJECTIVE: Summarize clinical data of skin substitutes in MMS for cutaneous malignancy. METHODS: A MEDLINE/Embase/Web of Science search was conducted. Articles with original data on outcomes after skin substitute use in MMS for cutaneous malignancy were included. Articles not in English or without original data were excluded. Bias was assessed using the Oxford CEBM Levels of Evidence Table. Outcomes were synthesized using weighted averages. This study was prospectively registered in PROSPERO. RESULTS: Of 1,007 articles, 40 met eligibility for inclusion. In total, 898 patients who underwent MMS and received a skin substitute were included. Xenografts were most commonly used ( n = 613). Semi-synthetic grafts (∼$<1/cm 2 ) and xenografts (∼$10/cm 2 ) are most affordable. Overall, outcomes were excellent for all skin substitutes, with a small proportion of patients experiencing correctable complications. CONCLUSION: Skin substitutes are highly effective in MMS, with enormous potential. While the data demonstrate positive outcomes, they predominately draw from small, retrospective studies or case reports. There is also a scarcity of data comparing skin substitutes with each other or controls. Prospective studies are recommended.

Post-streptococcal small-vessel vasculitis in a 16-month-old with associated intussusception and hypertension: Henoch Schönlein purpura?

Henoch Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic small-vessel vasculitis typically occurring in children 3-15 years of age, with peak incidence at 4-6 years. It is characterized by a constellation of symptoms including palpable purpura, arthralgias or arthritis, abdominal pain including intussusception, and renal involvement. We report a patient with these clinical findings whose IgA immunofluorescence was negative but with a presumptive diagnosis of HSP at 16 months of age, significantly younger than the classic population. This condition rarely affects this age group, and we highlight the importance of considering vasculitis in children of all ages, as a failure to diagnose could lead to insufficient long-term monitoring, particularly regarding renal function.

No bilingual advantage in children's attentional disengagement: Congruency and sequential congruency effects in a large sample of monolingual and bilingual children.

According to recent accounts, bilingualism in childhood confers an advantage in a specific domain of executive functioning termed attentional disengagement. The current study tested this hypothesis in 492 children (245 boys; Mage = 10.98 years) from Canada, China, and Lebanon by testing for an association between language status and measures of attentional disengagement. Across the entire sample, monolinguals responded more quickly and accurately than bilinguals on a measure of attentional disengagement but differed in age, socioeconomic status, and general cognitive ability. Differences between monolinguals and bilinguals disappeared when the influence of these confounding variables was controlled using a matched samples analysis (ns = 105). Bayesian analyses further confirmed that the evidence was more likely under the null hypothesis than under the alternative hypothesis. In sum, there was little evidence of an association between language status and attentional disengagement in children.

Delayed diagnosis of DRESS syndrome in a patient with skin of color.

We describe a particularly severe case of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome with hemodynamic instability, erythroderma, profound eosinophilia, and severe organ dysfunction. We attribute the severity in part to a delay in diagnosis due to patient's skin of color, as the erythroderma was not noticed until a dermatologist was consulted. This case highlights how even severe skin disease can present less conspicuously in patients with darker skin types. We outline several strategies that can help clinicians to recognize DRESS and other skin disease phenotypes in patients of color, thereby avoiding delays in diagnosis as seen in this case.

Successful radical surgical resection of a giant neurofibroma.

Large neurofibromas often cause significant patient morbidity and present a unique challenge to dermatologists and surgeons. Radical resection offers the lowest rate of recurrence but is not often pursued due to the high risk of intraoperative hemorrhage and difficulty in repairing large defects. Subtotal resection and debulking are more frequently performed, leading to higher rates of recurrence. This case highlights a particularly large neurofibroma and demonstrates how surgical techniques like preoperative embolization and advancement flaps can improve outcomes in the radical resection of large neurofibromas.

Management of relapsed cutaneous T-Cell lymphoma following allogeneic hematopoietic stem cell transplantation: Review with representative patient case.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative treatment option for patients with refractory cutaneous T-cell lymphoma (CTCL) through replacement of the bone marrow responsible for lymphoma cells and possibly induction of a graft-versus-lymphoma effect. However, allo-HSCT is not always curative; relapse of CTCL occurs in about half of patients post-transplant. Treatment of relapsed CTCL after allo-HSCT is challenging because post-transplant patients are at high risk of graft-versus-host disease, and this condition may be precipitated or exacerbated by standard CTCL therapies. The benefit of each potential therapy must therefore be weighed against its risk of graft versus host disease (GVHD). In this article, we review the management of relapsed CTCL after allo-HSCT. We begin with an exemplative patient whose relapsed Sezary syndrome was successfully treated without development of GVHD. We also report high-throughput T-cell receptor sequencing data obtained during the patient's disease relapse and remission. We then review general guidelines for management of relapsed CTCL and summarize all reported cases and outcomes of relapsed CTCL after transplant. We conclude by reviewing the current CTCL therapies and their risk of GVHD.

Resiquimod: a new topical immune-response modifier for the treatment of actinic keratosis.

Resiquimod is a Toll-like receptor (TLR)-7 and TLR-8 agonist that, like imiquimod, belongs to the class of imidazoquinolines, small organic molecules with potent antiviral and anticancer activity. Resiquimod activates myeloid and plasmacytoid dendritic cells while also promoting release of cytokines as interleukin-6, tumor necrosis factor-α and interferon-γ more effectively than imiquimod. Given these immunologic effects, resiquimod is emerging as a new topical pharmacotherapy for actinic keratosis (AK). In the pivotal trial by Stockfleth et al. complete clinical clearance in all the resiquimod-treated arms was more significant than placebo varying from 56% to 74%. Partial clinical clearance, or disappearance of >75% of AKs, was also significantly higher in the resiquimod arms varying from 75% to 87%. Overall, resiquimod is a new molecule that remains a promising therapy for AK, although additional studies are needed to further evaluate its efficacy.

Interleukin-31, a Potent Pruritus-Inducing Cytokine and Its Role in Inflammatory Disease and in the Tumor Microenvironment.

Substantial new information has emerged supporting the fundamental role of the cytokine interleukin-31 (IL-31) in the genesis of chronic pruritus in a broad array of clinical conditions. These include inflammatory conditions, such as atopic dermatitis and chronic urticaria, to autoimmune conditions such as dermatomyositis and bullous pemphigoid, to the lymphoproliferative disorders of Hodgkin's disease and cutaneous T-cell lymphoma. IL-31 is produced in greatest quantity by T-helper type 2 (Th2) cells and upon release, interacts with a cascade of other cytokines and chemokines to lead to pruritus and to a proinflammatory environment, particularly within the skin. Antibodies which neutralize IL-31 or which block the IL-31 receptor may reduce or eliminate pruritus and may diminish the manifestations of chronic cutaneous conditions associated with elevated IL-31. The role of IL-31 in these various conditions will be reviewed.

The efficacy of omalizumab in Cutaneous Mastocytosis: A case series.

BACKGROUND: Mastocytosis describes a heterogeneous group of disorders arising from a clonal proliferation of mast cells. Given the lack of curative treatments for the cutaneous form, there is a significant need for superior therapies. Omalizumab is a recombinant DNA-derived humanized IgG monoclonal antibody that selectively binds to human immunoglobulin E (IgE). It represents a potential treatment for the management of cutaneous mastocytosis, which currently has no standard treatment. METHODS: Two patients were treated with subcutaneous omalizumab 300 mg every 4 weeks. DISCUSSION: Patient 1 experienced 50% reduction in cutaneous infiltration and moderate improvement in pruritus. Patient 2 underwent 90% complete clearance of cutaneous lesions and reported full resolution of pruritus. The median duration of treatment was 24 weeks and time to response was 8 weeks. No significant changes in tryptase levels were observed. Both patients experienced injection site reactions. CONCLUSION: We provide evidence from two cases supporting the efficacy of IgE-mediated therapy in the treatment of cutaneous mastocytosis. Even at a higher-than-standard dose (300 mg vs. 150 mg), the drug was well-tolerated. As we await the results of pivotal clinical trials, omalizumab appears to be a promising treatment option in patients with cutaneous mastocytosis unresponsive to traditional therapies.