Post-transplant primary cutaneous peripheral T-cell lymphoma not otherwise specified in a pediatric patient.

Solid organ and hematopoietic stem cell transplantation may be complicated by the development of post-transplant lymphoproliferative disorders (PTLDs). The World Health Organization categorizes PTLDs into four entities including non-destructive, monomorphic, polymorphic, and classical Hodgkin lymphoma types. The most common PTLDs are B-cell lymphomas, with T-cell lymphomas accounting for only a few cases. Cutaneous T-cell lymphomas are rarer still in post-transplant patients with primary cutaneous peripheral T-cell lymphoma being an extraordinarily rare subtype in this population. PTLDs may be aggressive and are often associated with high morbidity and mortality. Advances in medicine have led to increased awareness of PTLDs and improved diagnostic tools which assist in the diagnosis of these conditions. However, the clinical and histopathologic heterogeneity of PTLDs may make diagnosis a challenge. In the transplant patient population, the cutaneous manifestations of the lymphoproliferative disease may mimic other conditions, such as eczematous dermatitis and graft-vs-host disease. Herein, we report a case of post-transplant primary cutaneous peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in a pediatric heart transplant patient and describe the clinical presentation and diagnostic histopathologic features.

A Comparison of Alternative Medicine Users and Non-Users in Patients With Hidradenitis Suppurativa.

BACKGROUND: Hidradenitis suppurativa patients often seek non-prescription therapies. OBJECTIVE: To determine the prevalence of alternative medicine use and characterize the differences between patients who report using alternative medications versus those who do not. METHODS: We surveyed 67 patients with hidradenitis suppurativa regarding demographics, alternative medicine use, disease severity, and quality of life. RESULTS: 25 (37.2%) of the HS subjects reported alternative medicine use. Alternative medicine users tended to be younger (36.7 vs 40.8 years), have a shorter time since diagnosis (12.6 vs14.6 years), and reported worse quality of life (14.1 vs 11.0) than non-users. These differences were not statistically significant. LIMITATIONS: Limitations included a small sample size. CONCLUSION: Alternative medicine use among patients with hidradenitis is common regardless of disease severity; even mild disease may drive patients to seek alternative treatment. J Drugs Dermatol. 2021;20(10):1072-1074. doi:10.36849/JDD.6046.

Rubella Vaccine Persistence Within Cutaneous Granulomas in Common Variable Immunodeficiency Disorder.

Common variable immunodeficiency disorder is a primary immunodeficiency disorder characterized by reduced levels of serum immunoglobulins and impaired antibody response. This condition may be associated with development of noninfectious granulomatous dermatitis of the skin which may be disfiguring and destructive. There are no published guidelines for the treatment of cutaneous granulomas in this patient population. In recent studies, rubella virus-positive cells in granulomas were localized to M2 macrophages which have an important role in wound healing and the secretion of immunoregulatory cytokines. We present a case of treatment-refractory, disfiguring common variable immunodeficiency disorder-associated granulomatous dermatitis. Immunofluorescence microscopy of the biopsy specimen confirmed the presence of rubella vaccine capsid proteins in M2 macrophages within the granuloma, a newly recognized phenomenon in this patient population. This knowledge may serve to identify future therapeutic targets or preventative strategies for granulomatous dermatitis in patients with primary immunodeficiency disorder.

Cutaneous metastases of papillary renal cell carcinoma: A case report and review of the literature.

Papillary renal cell carcinoma (RCC) is an uncommon subtype of RCC that is typically encountered at early stages and has a high survival rate. Histopathology typically shows well-defined papillary architecture with tumor cells lining fibrovascular cores and can be further subdivided into type 1 and type 2 tumors based on cytology and genetic basis. Type 1 tumors have a single layer of basophilic cells and low nuclear atypia, while type 2 tumors have a pseudostratified layer of eosinophilic cells and high nuclear atypia. Some tumors have overlapping features of both types. We present a unique case of cutaneous metastases of papillary RCC with typical papillary architecture in the dermis and review the literature on this rare entity.

Validation of a Hidradenitis Suppurativa Self-Assessment Tool.

BACKGROUND: Hidradenitis suppurativa (HS) is a debilitating dermatologic condition presenting with recurrent abscesses. While there are multiple scales to determine HS severity, none are designed for self-administration. A validated severity self-assessment tool may facilitate survey research and improve communication by allowing patients to objectively report their HS severity between clinic visits. OBJECTIVES: The purpose of this study was to assess a self-administered HS measure. METHODS: An HS self-assessment tool (HSSA) with 10 photographs of different Hurley stages was developed. The tool was administered to patients diagnosed with HS who visited the Wake Forest Baptist Health dermatology clinic over a span of 2 months. Physician-administered Hurley stage was recorded to determine criterion validity. To assess test-retest reliability of the measure, patients completed the HSSA again at least 30 minutes after the first completion. RESULTS: Twenty-four patients completed the measure, and 20 of these patients completed it twice. Agreement between physician-determined Hurley stage and self-determined Hurley stage was 66.7% with a weighted kappa of 0.57 (95% confidence interval [CI]: 0.30-0.84). The weighted kappa for agreement between patients' initial and second completion of the HSSA was 0.81 (95% CI: 0.64-0.99). CONCLUSIONS: The self-administered measure provides moderate agreement with physician-determined Hurley stage and good test-retest reliability.

Psoriasis and Atopic Dermatitis "Resistant" to Topical Treatment Responds Rapidly to Topical Desoximetasone Spray.

PURPOSE:: Topical corticosteroids (TS) are a treatment for atopic dermatitis (AD) and psoriasis (Ps). We assessed whether use of a TS under conditions designed to enhance adherence would be effective in patients who "failed" TS in the outpatient setting. METHODS:: Individuals with treatment-resistant Ps or AD were recruited (AD, n = 12; Ps, n = 12). Six participants were randomized to each of 2 groups of desoximetasone 0.25% spray alone (n = 6) or desoximetasone spray plus twice-daily phone call reminders to use the medication. Disease severity was assessed. RESULTS:: In treatment-resistant Ps patients, desoximetasone spray, with reminders, resulted in statistically significant improvement in all outcome measures. In treatment-resistant AD patients, there was statistically significant improvement in some assessments. Despite the very small sample size and short evaluation time, statistically significant changes were detected in this cohort. This is evidence of the large effect size of TS for Ps and AD when the treatment is used. CONCLUSIONS:: Patients with "treatment-resistant" Ps and AD generally responded well to the use of desoximetasone spray in the trial setting. This may be due to better adherence in the study environment or patients' preference for the spray vehicle. Patient reminders contributed to improved clinical outcomes in Ps and AD patients with "treatment-resistant" disease.

Treating Moderate-to-Severe Plaque Psoriasis With Guselkumab: A Review of Phase II and Phase III Trials.

OBJECTIVE: Guselkumab, an anti-interleukin-23 monoclonal antibody was recently approved for the treatment of patients with moderate-to-severe plaque psoriasis. This article will review the available phase II and phase III guselkumab clinical trial data. DATA SOURCES: A PubMed search was conducted using the terms guselkumab, interleukin-23, psoriasis, adalimumab, and ustekinumab (January 2014 to August 2017). STUDY SELECTION AND DATA EXTRACTION: Articles detailing the results of phase II and phase III clinical trials were selected for review. DATA SYNTHESIS: In 1 phase II and 2 phase III clinical trials, guselkumab was more effective than adalimumab and placebo in reducing Physician's Global Assessment and Investigator Global Assessment (IGA) scores in those with moderate-to-severe plaque psoriasis. In a separate phase III trial, transitioning to guselkumab treatment was more effective than continued ustekinumab use in reducing IGA scores in those who were minimally responsive to ustekinumab ( P = 0.001). Trial results did not reveal specific patterns in adverse event (AE) incidence; the most commonly reported AEs were nasopharyngitis, headache, and upper-respiratory-tract infections. No increased incidence of malignancy, tuberculosis, or serious infections were observed with the use of guselkumab. CONCLUSIONS: Guselkumab appears to be a safe and effective option for the treatment of moderate-to-severe plaque psoriasis in patients who have been screened for susceptibility to infection and are candidates for systemic treatment or phototherapy. However, long-term safety data are lacking.

Frontal Fibrosing Alopecia and Concomitant Lichen Planus Pigmentosus: A Case Series of Seven African American Women.

The association of frontal fibrosing alopecia (FFA) and lichen planus pigmentosus (LPPigm) is rare. Prior reports suggest that FFA and LPPigm are on the same spectrum of disease, and a diagnosis of LPPigm may predict the future development of FFA. We aim to further characterize the association between FFA and LPPigm by reviewing the clinical cases of seven African American women. Seven patients with FFA were diagnosed clinically by recession of frontotemporal hairline and confirmed by histopathologic examination showing lymphocyte-mediated cicatricial alopecia. LPPigm was diagnosed by clinical evaluation alone based on the characteristic morphology, color, and distribution of the lesions. It is difficult to distinguish whether halted progression of FFA was due to the success of the treatment regimen or spontaneous stabilization of disease over time. Our case series supports the theory that FFA and LPPigm likely exist on the same spectrum of disease. Our observations demonstrate a likely positive correlation between FFA and LPPigm.

J Drugs Dermatol. 2018;17(4):397-400.

A Pilot Study Characterizing Factors in Adherence to Cutaneous Lupus Treatment.

Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease that manifests as scarring, dyspigmentation, erythema, and pain. Topical corticosteroids are a mainstay of treatment. Irritation, messiness, and tediousness may deter use. Thus, nonadherence, rather than nonresponse, can result in treatment failure. Prior adherence studies were limited to systemic lupus erythematosus. We performed a single-center, open-label pilot study to assess adherence to topical medication in patients with CLE. CLE adherence to topical medications is suboptimal and declines over time. Shorter treatment duration and greater patient perception of disease severity may contribute to higher adherence. Improving adherence to existing treatments could be as or more valuable than new therapies for the disease.

Psoriasis therapy and aortic inflammation - translating statistical to clinical significance.

Psoriasis patients are known to have comorbid aortic vascular inflammation, which is one of the leading causes of cardiovascular morbidity and mortality in this population. Many studies report statistically significant improvements in aortic vascular inflammation after use of tumor necrosis factor inhibitors or interleukin-12/23 antagonists. However, the clinical significance in reduction of adverse cardiovascular events in psoriatic patients owing to biologic therapy has not been examined. Regardless of clinically significant cardiovascular benefits, dermatologists should continue to treat psoriasis patients optimally to mitigate the unfavorable effect this disease has on quality of life.

Internet-based survey intervention improves adherence to methotrexate among psoriasis patients.

BACKGROUND: While it is known that psoriasis patients have poor adherence to both topical and systemic medications, adherence to methotrexate is not well-characterized, and ways to improve methotrexate adherence have not been addressed. OBJECTIVE: The aim of this study was to evaluate whether a digital intervention improved adherence to oral methotrexate as measured by electronic monitoring. METHODS: Twenty-nine patients were randomized to receive either weekly digital interventions assessing treatment adherence or no intervention for 24 weeks. Patients received medication bottles with electronic monitoring, and returned at weeks 4, 12, and 24 to evaluate disease severity. RESULTS: The intervention group took methotrexate correctly 77.1% of the weeks observed compared to the control group which averaged 64.5%. More intervention patients took methotrexate as directed compared to the control group (78.3% vs 64.2%, p < 0.0001). Patients were most adherent around follow-up visits, with 100% of digital intervention patients and 80% of control patients taking methotrexate correctly during the week of a follow-up visit (p = 0.02). The digital intervention did not significantly improve disease severity in the intervention group compared to the nonintervention group. CONCLUSIONS: Low cost, scalable digital interventions may have the potential to increase psoriasis patient adherence to methotrexate, although the mechanism for the improvement is not yet well defined.

Adherence to levetiracetam for management of epilepsy: Assessment with electronic monitors.

INTRODUCTION: Anti-seizure medications are used to manage epilepsy and require long-term adherence to maintain therapeutic drug levels. We assessed adherence to levetiracetam and the use of a digital intervention to improve adherence in patients with epilepsy. METHODS: 30 participants with epilepsy were randomized 1:1 either to a digital email adherence intervention or control group. All patients were provided levetiracetam equipped with electronic monitoring caps to assess patient adherence to medication. Patients were followed for 6 months, with return visits at 1 month, 3 months, and 6 months. RESULTS: Subjects randomized to the control arm (n = 15) took 66% of the prescribed doses compared to the intervention group, who took 65% of prescribed doses (n = 15). Nine participants did not complete the study. Of the twenty-one participants that completed the study, the overall rate of adherence was 72% of prescribed doses taken. Two subjects in the control group and three subjects in the intervention group were adherent every month of the study-taking at least 80% of prescribed doses. Those randomized to the control group took the correct number of doses 44% of days in the study, and those in the intervention group took the correct number of doses 37% of days. DISCUSSION: Poor adherence to levetiracetam is common. An internet-based email survey intervention did not improve adherence to levetiracetam in epilepsy patients. Further advances in adherence are needed to help patients receive the maximum benefit of their medical treatments.

Biologics monitoring: incongruity between recommendations and clinician monitoring trends.

Background: Biologics are commonly used for moderate to severe psoriasis. Monitoring laboratory tests may provide little definitive benefit to patients.Objective: We queried a Humana database to gain insight regarding dermatologists' laboratory monitoring practices for psoriasis patients on biologics.Methods: Data were obtained from the Humana database. Our cohort included 333 patients with primary ICD-9 diagnosis of psoriasis (696.1) between the years 2008 and 2013 who are prescribed any biologic medication. Subjects on methotrexate, acitretin, or cyclosporine were excluded from the study. We separately queried laboratory tests by CPT codes and quantified based on frequency over a 2-year time period. Percentages of demographic group receiving a laboratory test at a given frequency category were calculated.Results: About 46% and 47% of patients received >4 comprehensive metabolic panel and complete blood count tests 2 years after starting a biologic. About 18% of individuals age >50 received greater than four Basic Metabolic Panel tests 2 years after starting a biologic.Limitations: Patient comorbidities might confound some of our findings, as these laboratory tests may have been ordered for a comorbidity rather than for biologics side effect monitoring.Conclusions: There are inconsistencies between current monitoring practices and guidelines. Clarifying biologics monitoring recommendations in psoriasis patients may reduce healthcare costs and provider workload.

Adherence to topical treatment can improve treatment-resistant moderate psoriasis.

Most patients with psoriasis have limited disease that should be manageable with topical treatment. However, psoriasis often is resistant to topical treatment. The aim of our study was to determine if patients using psoriasis-resistant topical treatments can be effectively treated with topicals under conditions promoting adherence. During this open-label, randomized, single-center clinical study, 12 patients with moderate psoriasis that previously failed topical treatment were selected and treated with desoximetasone spray 0.25% for 2 weeks. Six patients were randomized to receive twice-daily telephone call reminders to further encourage good adherence. Disease severity was assessed by the visual analog scale for pruritus, psoriasis area and severity index (PASI), total lesion severity score (TLSS), and investigator global assessment (IGA). At the end of the study, most patients improved in most scores. Therefore, apparent resistance to topical treatment often is due to poor adherence and can be overcome, at least over the short term.

Utility of boron in dermatology.

Introduction: Boron compounds are being investigated as therapies for dermatologic conditions. Several features of boron chemistry make this element an ideal component in dermatologic treatments. We review the published dermatologically-relevant clinical trials and case studies pertaining to boron compounds.Methods: PubMed was utilized to query terms boron, chemistry, drug, development, dermatology, atopic dermatitis, psoriasis, onychomycosis, tavaborole, AN 2690, crisaborole, and AN 2728. Clinical trials, case studies, animal studies and in vitro studies. Pertaining to atopic dermatitis, psoriasis and onychomycosis were included.Results: Crisaborole 2% topical solution reduced atopic dermatitis lesions by ∼60% when compared to pretreatment baseline. Crisaborole maintains its dose-dependent effect in treatment of psoriasis and significantly reduces psoriatic plaques when compared to controls. Adverse effects were mild, frequency of events varied between studies. Crisaborole was well tolerated when applied to sensitive skin. Topical tavaborole significantly reduced or eliminated onychomycosis with minimal side effects compared to placebo. Tavaborole was effective in treating recalcitrant onychomycosis.Discussion: Boron-based compounds form stable interactions with enzyme targets and are safe medications for the treatment of atopic dermatitis, psoriasis, and onychomycosis. The mild and rare side effects of topical boron-based compounds may make them ideal treatments for individuals with sensitive skin and pediatric populations.

Patients preferences for different corticosteroid vehicles are highly variable.

Introduction: Topical corticosteroids, available in an array of vehicles are used to control a variety of inflammatory skin diseases. Patients preferences for different vehicles may affect their willingness to use treatment. We assess corticosteroid vehicle preference and potential impact of topical characteristics on adherence and quality of life in patients with psoriasis.Methods: Subjects with psoriasis were recruited from Wake Forest University Dermatology Clinic. Subjects sampled desoximetasone 0.25% spray, betamethasone valerate 0.1% cream, triamcinolone acetonide 0.1% ointment, fluocinonide 0.05% gel, betamethasone valerate 0.1% lotion, clobetasol propionate 0.05% foam, and fluocinonide 0.05% solution in a predetermined randomized order. Subjects completed a Vehicle Preference Measure, Determinants of Adherence Measure, and a Determinants of Quality of Life Measure.Results: Patients preferences for the various products were highly variable. Regarding Determinants of Adherence, patients perception of absorption of the medication was ranked as 'quite important/extremely important' by 85% of total subjects. A majority of patients rated medication side effects as 'quite important/extremely important' when asked to consider topical characteristics effect on quality of life.Discussion: There was wide variation in patient preference for topical medication vehicles used for treating psoriasis. Several vehicle characteristics were considered important to adherence. Given the marked variation in vehicle preference, topical treatment should be individualized according to patients preferences.