Paul Curtiss

An Update on Kaposi’s Sarcoma: Epidemiology, Pathogenesis and Treatment

Kaposi’s sarcoma is an angioproliferative neoplasm which has undergone considerable epidemiologic change since the original description by Moritz Kaposi in the late 1800s. This opportunistic neoplasm gained widespread notoriety within the US during the height of the AIDS epidemic, where it was frequently found co-occurring with opportunistic infections. With the advent of modern antiretroviral therapies, as well as an increasing number of individuals on immunosuppression for autoimmune disease or organ transplantation, the landscape of the immunocompromised individual has changed. It is now important for clinicians to be mindful of Kaposi’s sarcoma manifesting in a growing variety of clinical contexts.

A systematic review and meta-analysis of the effects of topical nitrates in the treatment of primary and secondary Raynaud's phenomenon

Background: Multiple placebo‐controlled trials have assessed locally applied topical nitrate preparations in treating Raynauds phenomenon (RP). Objectives: The objective of this meta‐analysis was to assess the effects of local topical nitrates in primary and secondary RP with respect to a combined end point integrating parameters of digital blood flow and clinical severity. Methods: A systematic review was performed using MEDLINE, Embase, and the Cochrane library. Only trials comparing locally applied topical nitrates with placebo comparators were included. Studies were appraised for bias by 2 independent reviewers. Results: A total of 7 placebo‐controlled trials including 346 patients were used in the meta‐analysis; 4 trials used nitroglycerin ointments, 2 used the nitroglycerin gel vehicle MQX‐503, and 1 used compounded nitrite. The meta‐analysis results supported a moderate‐to‐large treatment effect in RP (standardized mean difference [SMD] = 0.70; 95% CI, 0.35–1.05; P < .0001). Subgroup analyses showed a large treatment effect in secondary RP (SMD = 0.95; 95% CI, 0.25–1.65; P = .008) and moderate effect in primary RP (SMD = 0.45; 95% CI, 0.05–0.85; P = .03). Limitations: Limitations include the inclusion of multiple topical nitrate preparations and integration of different outcomes assessments. Conclusion: Local topical nitrates have significant efficacy in the treatment of both primary and secondary RP.

The readability, suitability, and content features of eczema action plans in the United States

Little is known about the reading grade level (readability), appropriateness of design (suitability), and content variability of written eczema action plans (EAPs), which can impact the effectiveness of these patient education tools. Here, we assess the readability, suitability, and content of EAPs currently used by pediatric dermatologists in the United States.

The clinical effects of l-arginine and asymmetric dimethylarginine: implications for treatment in secondary Raynaud's phenomenon

Secondary Raynauds phenomenon (RP) is often the sentinel clinical finding in systemic sclerosis and may precede systemic disease by several years. Altered nitric oxide metabolism plays a critical role in both fibrosis and severe secondary RP phenotypes in these patients. Increased flux through inducible nitric oxide synthase (iNOS) drives cutaneous fibrosis. Failure of flux through endothelial nitric oxide synthase (eNOS) contributes to increased vasoconstriction and decreased vasorelaxation. The underproduction of nitric oxide by eNOS is in part due to increased levels of asymmetric dimethylarginine (ADMA), an endogenous competitive inhibitor of nitric oxide synthase. The inhibitory effects of increased ADMA levels may be counteracted increasing serum l_arginine, which is often an effective treatment strategy in these patients. As such, l_arginine_based therapies should be considered in managing secondary RP, particularly given their favourable safety and tolerability profile. While there is no established dosing regimen, studies of oral l_arginine in secondary RP suggest that divided dosing may begin at 1_2 g/day and may be titrated up to 10 g/day. Conversely, primary RP is not associated with increased ADMA production which likely accounts for the failure of l_arginine trials to show benefit in primary RP.

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody–associated vasculitis: An emerging overlap syndrome with cutaneous manifestations

AAV: antineutrophil cytoplasmic antibodyeassociated vasculitis ACR: American College of Rheumatology ANA: antinuclear antibody ANCA: antineutrophil cytoplasmic antibody anti-MPO: antimyeloperoxidase MCTD: mixed connective tissue disease p-ANCA: perinuclear antineutrophil cytoplasmic antibody RNP: ribonucleoprotein SLE/AAV: systemic lupus erythematosus antineutrophil cytoplasmic antibodyeassociated vasculitis overlap syndrome INTRODUCTION Systemic lupus erythematosus and antineutrophil cytoplasmic antibody (ANCA)eassociated vasculitis (SLE/AAV) overlap syndrome is a rare disease originally described in 2008. With only a few reports in the literature, this condition is characterized by aggressive crescentic glomerulonephritis, arthritis, cutaneous involvement, and both antinuclear antibody (ANA) and ANCA. Dermatologic manifestations are common and may occur at initial presentation. Although skin lesions may vary in morphology, the most commonly reported include cutaneous nodules and ecchymoses. Few reports have characterized the cutaneous findings seen in this disorder. We report a young woman with SLE/ AAV overlap syndrome who presented with a macular eruption and histopathology findings demonstrating interface dermatitis.

Genital ulcers in an immunocompromised man

A 72-year-old man with a history of type 2 diabetes, renal transplant, long-term immunosuppression with prednisone and tacrolimus, and a recent diagnosis of posttransplant lymphoproliferative disorder, presented with a 2-week history of worsening headaches and blurry vision. Physical examination was notable for a left facial droop and 4 genital ulcerations: 2 on the left medial thigh and 2 on the left lateral scrotum. The ulcerations had well-defined pink raised borders and a thin yellowmembrane overlying the base (Fig 1). Results of a punch biopsy are shown (Fig 2).

Bullous id eruption in the setting of orf

O-fucosyltransferase 1, cause generalized Dowling-Degos disease. Am J Hum Genet 2013; 92:895–903. 3 McMillan BJ, Zimmerman B, Egan ED et al. Structure of human POFUT1, its requirement in ligand-independent oncogenic Notch signaling, and functional effects of Dowling-Degos mutations. Glycobiology 2017. https://doi.org/10.1093/glycob/cwx020 4 Li CR, Brooks YS, Jia WX et al. Pathogenicity of POFUT1 mutations in two Chinese families with Dowling-Degos disease. J Eur Acad Dermatol Venereol 2016; 30:79–81.