Cardiovascular Risk Management in Patients Treated with JAK Inhibitors.

ABSTRACT: The Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway plays a critical role in the pathogenesis of many immune-mediated inflammatory diseases (IMIDs). Although Janus kinase inhibitors (JAKi) are an effective treatment for several IMIDs, they have come under scrutiny as a class due to a potential risk of venous thromboembolism (VTE) and cardiovascular (CV) events, specifically noted with the oral JAKi, tofacitinib, as reported in the ORAL Surveillance Trial of a high CV risk rheumatoid arthritis population. This trial resulted in a black box warning from the Food and Drug Administration and European Medicines Agency regarding risk of VTE and CV events that was extended across several types of JAKi (including topical ruxolitinib) when treating IMIDs, leading to considerable controversy. Included is an up-to-date review of the current and rapidly evolving literature on CV risk in patients with IMIDs on JAKi therapy, including identification of potential risk factors for future VTE and CV events on JAKi therapy. We suggest a comprehensive, multimodal, and systematic approach for evaluation of CV risk in patients considering taking JAKi and emphasize that cardiologists play an important role in risk stratification and mitigation for patients with high CV risk factors or on long-term JAKi therapies.

Experience of a Single Academic Center Using IL-1 Inhibition for Rare Dermatologic Conditions.

Evidence-based literature regarding management of rare and severe dermatologic disease is limited. Canakinumab and anakinra, two therapeutics used for inhibiting IL-1 pathways, have seen increased utilization for treatment of refractory dermatoses. We sought to better characterize the breadth of dermatologic conditions for which these medications could be utilized.

Use of teledermatology by dermatology hospitalists is effective in the diagnosis and management of inpatient disease.

BACKGROUND: Patient outcomes are improved when dermatologists provide inpatient consultations. Inpatient access to dermatologists is limited, illustrating an opportunity to use teledermatology. Little is known about the ability of dermatologists to accurately diagnose disease and manage inpatients with teledermatology, particularly when using nondermatologist-generated clinical data. METHODS: This prospective study assessed the ability of teledermatology to diagnose disease and manage 41 dermatology consultations from a large urban tertiary care center, using internal medicine referral documentation and photographs. Twenty-seven dermatology hospitalists were surveyed. Interrater agreement was assessed by the κ statistic. RESULTS: There was substantial agreement between in-person and teledermatology assessment of the diagnosis with differential diagnosis (median κ = 0.83), substantial agreement in laboratory evaluation decisions (median κ = 0.67), almost perfect agreement in imaging decisions (median κ = 1.0), and moderate agreement in biopsy decisions (median κ = 0.43). There was almost perfect agreement in treatment (median κ = 1.0), but no agreement in follow-up planning (median κ = 0.0). There was no association between raw photograph quality and the primary plus differential diagnosis or primary diagnosis alone. LIMITATIONS: Selection bias and single-center nature. CONCLUSIONS: Teledermatology may be effective in the inpatient setting, with concordant diagnosis, evaluation, and management decisions.

A narrative review of therapies for scalp dermatomyositis.

Cutaneous involvement of the scalp is a common manifestation of dermatomyositis (DM), occurring in up to 82% of adults with DM. Scalp DM predominantly affects women and is characterized by dermatitis, alopecia, pruritus, and/or burning. While cutaneous DM negatively impacts quality-of-life, scalp symptoms in particular are often severe, debilitating, and recalcitrant to standard DM therapies. Currently, there is a paucity of guidelines to inform management of scalp symptoms in patients with cutaneous DM. In this narrative review, we summarize the treatments utilized to manage scalp DM and highlight potential areas for future research. We identified eight studies that reported on 27 treatments focused on cutaneous DM and described outcomes on scalp symptoms. A majority of the treatments were standard therapies for cutaneous DM and resulted in no or minimal improvement in scalp symptoms. Five therapies did result in complete resolution of scalp symptoms and were recommended as potential areas of future research. These included low-dose naltrexone and platelet-rich plasma, as well as two frequent and one less common therapy for cutaneous DM respectively: intravenous immunoglobulin, rituximab, and apremilast. Though the literature was not systematically assessed in this review, these findings illustrate not only that strategies for refractory scalp DM are lacking, but also that those demonstrating potential efficacy are limited by low levels of evidence. Additional studies, especially randomized controlled trials, are needed to better inform management of scalp DM.

Comparison between organismal staining on histology and tissue culture in the diagnosis of cutaneous infection: A retrospective study.

BACKGROUND: In instances of suspected cutaneous infection, the standard of care includes obtaining skin biopsy specimens for histology and tissue culture. Few studies have compared the clinical utility of each test. OBJECTIVE: To assess the concordance of results between tissue culture and histology, as well as the clinicopathologic features that may influence the diagnostic yield of each test. METHODS: A retrospective review of all patients who underwent skin biopsy for histology and tissue culture at New York University from 2013 through 2018. RESULTS: Of 179 patients, 10% had positive concordance, 21% had positive tissue culture only, and 7% had positive histology only. We calculated a kappa correlation coefficient of 0.25 between histology and tissue culture (reference, 0.21-0.39 indicates minimal agreement). Histology exhibited higher sensitivity in detecting fungi, whereas tissue culture was more sensitive in identifying Gram-negative bacteria. Antimicrobial use before biopsy led to significantly fewer positive cultures (37.5% vs 71%; P = .023) in patients ultimately diagnosed with infection. LIMITATIONS: This study was conducted at a single institution, thereby restricting its broad applicability. The lack of a validated criterion standard to diagnose infection also limits interpretation of the results. CONCLUSION: Tissue culture and histopathology often yield discordant results. Dermatologists should recognize specific limitations, yet high clinical utility in special circumstances, of tests when approaching cases of suspected infection.

Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. Participants were administered 9-point Likert scale questionnaires regarding 135 statements. The RAND/UCLA Appropriateness Method was used to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5 to 9 and a disagreement index of ≤1 were included in the guideline. For the final round, the guidelines were appraised by all of the participants. Included are an evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN.

Generalized essential telangiectasia.

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.

Acquired immune deficiency syndrome-related epidemic Kaposi sarcoma.

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.

Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment.

PURPOSE OF REVIEW: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF. RECENT FINDINGS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.

Generalized hyperhidrosis secondary to presumed eccrine gland dysfunction with possible apocrine metaplasia.

We present a 57 year-old man presented with generalized hyperhidrosis and widespread, smooth, flesh colored papules on the torso and extremities.Histological examination from multiple biopsies demonstrated morphologic alteration of the eccrine glands with an apocrine phenotype, suggesting eitherapocrine metaplasia or the presence of "apoeccrine glands." The morphologic similarities between eccrine, apocrine, and apoeccrine as they relate to ourpatient's histologic findings are discussed. We consider secondary causes of generalized hyperhidrosis, which may also play a role in this patient's presentation. Treatment and further workup are discussed, whilemanagement of this patient remains in progress.

Epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is a rare, acquired subepidermal blistering disease. EBA is characterized by autoantibodies to collagen VII,which serves to link the epidermis to the dermis. The two most common presentations of EBA are classical noninflammatory EBA and bullous pemphigoid-like EBA. Diagnosis of EBA can be challenging as it sharesclinical and histopathologic features with other blistering diseases. Treatment is often recalcitrant and will often necessitate multiple therapies. We presenta case of a thirty-six-year-old Chinese man with EBA and review the literature.

Primary anetoderma with undifferentiated connective tissue disease.

Anetoderma is a rare benign elastolytic disorder that is characterized by focal loss of elastin fibers on histopathology and is often recalcitrant to treatment. We present a case of a patient with a 20-year history of pruritic and painful hyperpigmented atrophic papules clustered on the neck, axillae, inframammary folds, and right medial thigh. Although the histopathologyof her axillary lesions was consistent with anetoderma, her clinical presentation is unusual given the extent of involvement, reported pain and pruritus, and sharp demarcation of the distribution. The diagnosticuncertainty of this case led to added difficulty in management of a disease that is already notoriously difficult to treat and may significantly impact patient's quality of life.