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The original version of this article, published on 04 June 2018, unfortunately contained a mistake.

The pathophysiology of generalized essential telangiectasia is not well understood. Generalized essential telangiectasia is an uncommon disorder in which widespread telangiectasias of unknown cause develop without associated systemic or antecedent dermatologic disease. We report a case of generalized essential telangiectasia in an otherwise healthy 49-year-old man.

Kaposi sarcoma (KS) is a vascular neoplasm that is one of the most common human immunodeficiency virus (HIV)-related malignancies. We present the case of a 42-year-old man with a new diagnosis of HIV and acquired immune deficiency syndrome (AIDS)-related epidemic KS.

INTRODUCTION/BACKGROUND:Dermatomyositis (DM) is an idiopathic inflammatory myopathy involving severe debilitation in need of diagnostics. We evaluated the proximal lower extremity musculature with diffusion tensor imaging (DTI), intravoxel incoherent motion (IVIM) and dynamic DTI in DM patients and controls and compared with standard clinical workup.  METHODS: In this IRB-approved, HIPAA-compliant study with written informed consent, anatomical, Dixon fat/water and diffusion imaging were collected in bilateral thigh MRI of 22 controls and 27 DM patients in a 3T scanner. Compartments were scored on T1/T2 scales. Single voxel dynamic DTI metrics in quadriceps before and after 3-min leg exercise were measured. Spearman rank correlation and mixed model analysis of variance/covariance (ANOVA/ANCOVA) were used to correlate with T1 and T2 scores and to compare patients with controls. RESULTS:DM patients showed significantly lower pseudo-diffusion and volume in quadriceps than controls. All subjects showed significant correlation between T1 score and signal-weighted fat fraction; tissue diffusion and pseudo-diffusion varied significantly with T1 and T2 score in patients. Radial and mean diffusion exercise response in patients was significantly higher than controls. CONCLUSION/CONCLUSIONS:Static and dynamic diffusion imaging metrics show correlation with conventional imaging scores, reveal spatial heterogeneity, and provide means to differentiate dermatomyositis patients from controls. KEY POINTS/CONCLUSIONS:• Diffusion imaging shows regional differences between thigh muscles of dermatomyositis patients and controls. • Signal-weighted fat fraction and diffusion metrics correlate with T1/T2 scores of disease severity. • Dermatomyositis patients show significantly higher radial diffusion exercise response than controls.

BACKGROUND:Malignant angiomyolipoma is an uncommon tumor of the class of perivasciular epithelioid cell neoplasms (PEComas). These tumors are characteristically driven by deleterious mutations in the tumor suppressors TSC1 and TSC2, whose gene products typically act to inhibit mTOR. There are several cases of malignant angiomyolipoma which exhibit transient responses to mTOR inhibitors, forming the basis of current practice guidelines in malignant PEComa. However the tumors ultimately acquire resistance, and there is no well-established second-line option. Despite the increasing prevalence of immunotherapy across a wide range of solid tumors, little is known about the immune infiltrate and PD-L1 expression of angiomyolipoma. Furthermore, there is no reported case on the treatment of malignant angiomyolipoma with an immune checkpoint inhibitor. CASE PRESENTATION/METHODS:A 38 year-old man presented with gross hematuria and was diagnosed with renal epithelioid angiomyolipoma. Despite surgical resection, the tumor recurred and metastasized. Targeted genomic sequencing revealed a deleterious mutation in TSC2, and the patient was treated with the mTOR inihbitor everolimus. The patient went on to have a partial response but ultimately progressed. He was then treated with the anti-PD-1 immune checkpoint inhibitor nivolumab, and achieved a durable near-complete response which is ongoing after two years of treatment. Immunohistochemical staining of tumor tissue revealed strong PD-L1 expression and a brisk T-cell infiltrate. CONCLUSIONS:We report on the first durable systemic treatment of malignant epithelioid angiomyolipoima with the use of PD-1 antibody nivolumab. Given the absence of prospective clinical trials in this exceedingly rare disease, particularly in the second-line setting, immune checkpoint inhibitors like nivolumab should be considered.

Background: Lichen Planus (LP) is a chronic idiopathic disorder of the skin, nails, and mucous membranes with a varied clinical presentation. This study describes the epidemiology and treatment of LP patients treated in a large tertiary medical center.
Method(s): This retrospective chart review identified 294 patients with biopsy-proven LP seen within the dermatology department at NYU Langone Medical Center from January 1, 2005, September 5, 2016. Patients were identified using ICD code-based queries (ICD-9 697.0, ICD-10 L43.0-L43.9). Demographic, physical examination, pathologic, laboratory, and treatment data were extracted.
Result(s): Of 294 participants, 65% were female. Fifty-five percent were white, 16% black, 12% South Asian, 10% Hispanic, and 6% Asian. Mean age at symptom-onset, presentation, and diagnosis was 46.7, 51, and 49.9 years, respectively. Initial misdiagnosis occurred in 15.6% of patients. Of individuals for whom physical examination was recorded (n = 282), 35.1% had documented mucosal involvement (59.6% oral only, 19.2% genital only, and 21.2% both oral and genital). Cutaneous involvement was seen in 69.5% of patients with mucosal involvement of only the oral cavity, compared with 36.8% with only genital involvement. Pruritus was the most common symptom (57.3%). Hepatitis C testing was performed in 38.3% of patients; 9.7% of those tested were positive. Sixty-seven percent were treated with topicals only and 27.5% received systemic therapy. In total, 20 systemic agents were used; prednisone was used most commonly, followed by cyclosporine and hydroxychloroquine. Of patients on systemic therapies, 28.4% required 2 or more systemic agents during their course.
Conclusion(s): This is the largest study to date to describe the epidemiology and treatment of lichen planus. Lichen planus is most common among women, has a mean age of onset of 46.7 years, and most commonly presents with pruritus. Mucosal involvement is present in 35.1% of individuals, most commonly involving the oral cavity. Patients with only oral involvement had a higher rate of cutaneous involvement than subjects with only genital involvement. The majority of patients did not receive hepatitis C testing, but 9.7% of those with test results were positive. Topical monotherapy was often insufficient, and 27.5% of patients required systemic therapy. Systemic therapy most commonly included the use of corticosteroids, although many required therapy with multiple systemic agents.
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