Unique characteristics of anti-MDA-5 associated dermatomyositis in Southern California with a large Hispanic population.

OBJECTIVES: There is little to no data about the presentation and clinical course of anti-melanoma differentiation-associated gene-5 antibody (anti-MDA-5) dermatomyositis in a primarily U.S. Hispanic population. We describe the clinical course of anti-MDA-5 dermatomyositis in our majority Hispanic population. METHODS: This is a multicenter, retrospective case series of anti-MDA-5 dermatomyositis. Patients diagnosed with anti-MDA-5 dermatomyositis from June 2015 to March 2023 at four medical centers in Los Angeles, California, were included. Demographics and clinical characteristics were obtained. Descriptive statistics, Pearson's chi-squared, Fisher's exact, Wilcoxon rank sum, and Kruskal-Wallis tests were performed as applicable. RESULTS: Thirty anti-MDA-5 dermatomyositis patients were included. Twenty-two (73 %) were Hispanic. Twenty-one patients (70 %) were female, with a median age of 40.5 years. Hispanic patients were diagnosed with anti-MDA-5 dermatomyositis at a younger age than non-Hispanic patients (p = 0.025). Inflammatory arthritis was prominent; more males were affected than females (p = 0.027). Thirteen patients (43 %) were amyopathic. Twenty-five patients (83.3 %) had evidence of interstitial lung disease (ILD), and a higher ferritin level was associated with ILD (p = 0.049). There were six deaths (20 %); five (17 %) were ascribed to rapidly progressive ILD. CONCLUSION: ILD was the most common presentation of anti-MDA-5 dermatomyositis in our cohort and was associated with higher ferritin levels. Hispanic patients had a younger age of diagnosis than non-Hispanic patients. Necrotic skin lesions and inflammatory arthritis were frequently seen. This is the first study looking at clinical phenotypes and outcomes of anti-MDA-5 dermatomyositis in a primarily Hispanic U.S. POPULATION: Future studies are needed to better understand the clinical manifestations (to promptly recognize and treat) of this population of anti-MDA-5 dermatomyositis.

Psychiatric comorbidities on an inpatient dermatology consultation service: A cross-sectional analysis.

Despite the high prevalence of psychiatric illness in hospitalised dermatology patients, characterisation of psychiatric comorbidities on an inpatient dermatology consultation service in the United States has yet to be performed. To fill this gap in knowledge, we investigated the prevalence of and factors associated with psychiatric illness on the inpatient dermatology consultation service at the University of Southern California. Of the 429 patients seen by the dermatology consultation service between June 2021 to July 2022, 147 (34%) had psychiatric illness (defined as having at least 1 psychiatric diagnosis). Increasing age was associated with a decreased likelihood of psychiatric illness, while housing instability, chronic dermatologic disease, drug reaction, and pruritus without rash were associated with an increased likelihood of psychiatric illness. The high prevalence of psychiatric illness observed in hospitalised dermatology patients emphasises the importance of collaboration between consultant dermatologists and mental health specialists, particularly when specific sociodemographic or disease factors are present.

Treatments for Dissecting Cellulitis of the Scalp: A Systematic Review and Treatment Algorithm.

BACKGROUND: Dissecting cellulitis of the scalp (DCS) is a chronic inflammatory skin condition characterized by abscesses, nodules, fistulas, and scarring alopecia. Management of this oftentimes debilitating dermatosis can be challenging due to its recalcitrant nature. There is limited data regarding the efficacy of treatment options for DCS. OBJECTIVE: The aim of this study was to conduct a systematic review of the literature to explore the efficacy and safety of reported DCS treatments. METHODS: In October 2022, MEDLINE and EMBASE databases were searched for articles on treatments for DCS. Studies that contained outcome efficacy data for DCS treatments were included. Reviews, conference abstracts, meta-analyses, commentaries, non-relevant articles, and articles with no full-text available were excluded. Data extraction was performed by two independent reviewers. RESULTS: A total of 110 relevant articles with 417 patients were identified. A majority of studies (86.4%) were case reports or series. Treatment options included systemic antibiotics, oral retinoids, biologics, procedural treatments, combination agents, and topical treatments. Oral retinoids and photodynamic therapy were the most extensively studied medical and procedural interventions, respectively. CONCLUSION: Overall, randomized controlled trials are needed to evaluate various treatment regimens for DCS and provide patients with a robust, evidence-based approach to therapy.

Refining the Classification of Field Cancerization.

BACKGROUND: Field cancerization is a commonly encountered but poorly defined entity in dermatology. Lack of a consensus definition makes characterizing and studying this condition difficult. OBJECTIVE: Propose a framework for defining and managing field cancerization patients. METHODS: Clinical experience and available data in the literature was used to develop a proposed field cancerization disease classification system and chemoprevention algorithm. RESULTS: The author group developed a field cancerization classification and treatment algorithm that stratifies patients into 5 different classes based on the extent of their field cancerization. They have started to recruit and study the highest risk cohort (Category 4 or 5) and have recruited 57 patients with 5 or more lifetime keratinocyte carcinomas for a prospective study evaluating chemoprevention strategies and disease burden. In these cohorts, the average number of skin cancers was greater than 12; however, less than 39% of these patients had used any chemoprevention in the prior 6 months. CONCLUSION: A meaningful and clinically relevant disease stratification framework with chemoprevention guidance has the potential to highly impact the specialty and patients.

Efficacy of Etanercept in the Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

It has been suggested that the use of etanercept for treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) might provide improved mortality benefit and decreased skin healing times. This retrospective study compared the use of single-dose subcutaneous etanercept to intravenous immunoglobulin (IVIG) and supportive care alone. Thirteen patients were treated with a single dose (50 mg) of subcutaneous etanercept. Results of this study support the use of etanercept as a potentially beneficial agent in the treatment of SJS/TEN.

Overlap between hemophagocytic lymphohistiocytosis and drug reaction and eosinophilia with systemic symptoms: a review.

Drug reaction and eosinophilia with systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome (DIHS), shares features with hemophagocytic lymphohistiocytosis (HLH), most notably fever, rash, and internal organ involvement. However, there is increasing recognition of drug-induced (secondary) HLH and biopsy-proven hemophagocytosis in DRESS, suggesting that HLH and DRESS not only overlap but also may be diseases on the same spectrum of immune dysfunction. To characterize existing literature on HLH/DRESS overlap, we queried the PubMed/MEDLINE database for 23 cases of HLH-DRESS codiagnosis. Average time-to-onset of rash after exposure to inciting drug was 2.7 weeks. Fourteen cases (61%) clinically worsened despite initial therapy, prompting a workup with diagnosis of HLH on average 2.3 weeks after diagnosing DRESS. Nine cases met HLH diagnostic criteria and had a RegiSCAR score ≥4. Nine cases met one set of criteria with a presentation suggestive of the other. Five cases met neither criteria. A patient presenting with fever, generalized rash, bicytopenia, and internal organ involvement after drug exposure was most predictive of meeting diagnostic criteria for both HLH and DRESS. Treatment was highly variable, although most initiated systemic corticosteroids with/without IVIG, plasmapheresis, or etoposide. Patients with poor outcomes in this review were treated using steroid monotherapy and had viral reactivation. Dermatologists should consider the possibility of HLH in any patient presenting with fever, rash, internal organ involvement, and cytopenia. Additional studies will be necessary to further characterize HLH and DRESS overlap and determine optimal management.

Nodular secondary syphilis in three HIV-positive patients: a case series.

Nodular secondary syphilis is an uncommon variant of secondary syphilis. We identified three cases of nodular secondary syphilis at our institution. The first patient presented with a diffuse nodular rash that included his scrotum and penis. The second patient had disseminated skin-colored nodules with serosanguinous crust on his face, trunk, and extremities. The third patient had a pruritic papular and nodular rash with overlying crust. All three patients had a reactive rapid plasma reagin and tested positive for fluorescent treponemal antibody absorption. All were eventually confirmed to be human immunodeficiency virus-positive. Histopathological examination demonstrated inflammatory infiltrate in the dermis composed of lymphocytes, histiocytes, and plasma cells, and treponemal staining highlighted spirochetes in the dermis. The patients were successfully treated with intramuscular penicillin benzathine G. Physicians should be aware of nodular syphilis as a less common cutaneous manifestation of secondary syphilis. Prompt diagnosis of secondary syphilis can expedite resolution of the infection and avoid progression to tertiary syphilis.

Disseminated cutaneous gout: a rare manifestation of a common disease.

Disseminated cutaneous gout is a rare atypical cutaneous manifestation of gout in which widespread dermal and subcutaneous tophi develop at extra-articular body sites. Given the lack of joint involvement that is typically a feature in tophaceous gout, the diagnosis may not be initially suspected. We present the case of a 50-year-old Hispanic man with poorly controlled gout who was evaluated for several years of firm papulonodules over the trunk and upper and lower extremities, sparing the joints; histopathology confirmed, the diagnosis of disseminated cutaneous gout. Per our literature review, disseminated cutaneous gout presents with polymorphous papules and nodules that can mimic other, more common cutaneous diseases. There is a preponderance of cases in males, Asians, and patients with longstanding gout. The lower extremities are involved in nearly all reports. Uric acid-lowering therapy with allopurinol has been reported to decrease the size and number of lesions in a minority of treated patients.

Sorafenib Toxicity Mimicking Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) Syndrome

Sorafenib is an oral multikinase inhibitor approved by the United States Food and Drug Administration for the treatment of advanced hepatocellular and renal cell carcinoma. Cases of sorafenib-induced Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome have been reported in the literature. DRESS syndrome is a potentially fatal, drug-induced hypersensitivity reaction that occurs 2-8 weeks after drug exposure. DRESS syndrome presents with generalized morbilliform eruption, facial edema, eosinophilia, and end-organ damage. We present the first reported case of sorafenib toxicity mimicking DRESS syndrome in a patient with metastatic adrenocortical carcinoma presenting with fever, morbilliform rash, and transaminitis in the absence of eosinophilia three days following initiation of sorafenib therapy. It is critical that clinicians are equipped to accurately diagnose DRESS syndrome due to its high mortality rate and the morbidity associated with prolonged steroid therapy. J Drugs Dermatol. 2019;18(5):468-469.

Patient satisfaction and quality of care: a prospective study at outpatient dermatology clinics.

Patient satisfaction often is used as a proxy for quality of care, with physicians evaluated and reimbursed based on patient satisfaction scores. As a specialty, dermatology is lagging in quality improvement studies. To fill this gap, we conducted a prospective study of targeted interventions administered at outpatient dermatology clinics to determine if they resulted in statistically significant increases in patient satisfaction measures, particularly among Spanish-speaking patients. This study, along with the existing body of research, suggests the need for continued work to maximize patient satisfaction in dermatology.