Mortality and Autopsy Findings in Patients with Pyoderma Gangrenosum: A Multi-Institutional Series.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with an increased risk of mortality compared to the general population. The causes of this increased risk are not well understood. Misdiagnosis is common in PG, and many studies are limited by the inclusion of misdiagnosed cases. The goal of this study was to review autopsy findings, identify causes of death, and identify factors that may worsen outcomes among deceased patients confirmed to have PG. METHODS: Data was retrospectively reviewed from the electronic medical records at five academic hospitals. A search was conducted for deceased patients with a diagnosis of PG who had an autopsy performed between 2010 and 2020. We report a descriptive analysis of 11 patients and their clinical characteristics, causes of death, and autopsy findings. RESULTS: The average age of death was 62.9 years. Seven patients had at least one underlying condition known to be associated with PG including inflammatory bowel disease, inflammatory arthritis, or a hematologic disorder. The most common cause of death was infection (n = 6, 54.5%), followed by pulmonary embolism (n = 3, 27.3%), and myelodysplastic syndrome (n = 2, 18.2%). Six patients (54.5%) were taking systemic steroids at the time of death. CONCLUSION: The development of PG may shorten life expectancy among those with underlying conditions associated with PG, and common treatments for PG may contribute to the risk of fatal complications. Awareness of the risk of infection, thrombosis, and malignancy among those with PG is necessary for proper management. Further research is needed to explore the relationship between PG and thromboembolism.

Efficacy and safety of repository corticotropin injection for refractory cutaneous dermatomyositis: a prospective, open-label study.

OBJECTIVES: Cutaneous dermatomyositis (DM) is often refractory to multiple medications. Repository corticotropin injection (RCI) is FDA-approved for DM, but little is known about its efficacy and safety for treating cutaneous DM. We conducted a prospective, open-label trial assessing efficacy and safety of RCI for treating refractory cutaneous DM. METHODS: DM patients with moderate-to-severe cutaneous activity [Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A)] >14 despite prior treatment with ≥2 systemic agents were enrolled. Patients were initiated on 80 u RCI twice weekly for 6 months. Primary outcomes included significant decreases in CDASI-A and Physician's Global Assessment (PGA) scores at 6 months. RESULTS: Of nineteen patients enrolled, fifteen patients (11 females, 4 males) with DM (7 classic, 8 amyopathic) completed 6 months of RCI treatment. Patients were treated with a median 3.0 systemic medications prior to enrolment and were taking a median of 2.0 systemic medications at enrolment. Median baseline CDASI-A score was 19.0 and median PGA activity score was 2.5/10. For patient-reported outcomes, baseline median patient global skin score (PtGSS) was 3.0/10 and median dermatology life quality index (DLQI) score was 7.0/10. At 6 months, there were statistically significant improvements in CDASI-A scores (median= 10.0), PGA scores (median= 0.8/10), PtGSS scores (median= 7.0) and DLQI scores (median= 2.0), among others. Adverse effects were mild. CONCLUSIONS: RCI treatment resulted in statistically significant and clinically meaningful improvement in cutaneous DM activity and quality of life. Our results suggest RCI is an effective, safe, and well-tolerated treatment for patients with refractory cutaneous dermatomyositis. CLINICAL TRIAL REGISTRATION: This clinical trial was registered with ClinicalTrials.gov (ClinicalTrials.gov Identifier: NCT01906372).

Update in cutaneous manifestations of COVID-19: Special populations.

Cutaneous abnormalities were among the first clinical findings reported in patients infected with SARS-CoV-2 at the onset of the COVID-19 pandemic, but the significance was initially unclear. Correlations have since been drawn between many of these cutaneous eruptions and their diagnostic or prognostic value. Additionally, COVID-19 vaccines have generated acute and delayed cutaneous reactions with which clinicians should be familiar. In this narrative review, we update the cutaneous abnormalities associated with COVID-19 infection for pediatric and non-White populations, and common cutaneous reactions to COVID-19 vaccines.

Common skin signs of COVID-19 in adults: An update.

Cutaneous findings can be clues to diagnosis and infection severity in viral illnesses, including COVID-19. The authors provide an update on the diagnostic and prognostic value of the 5 most common cutaneous abnormalities associated with COVID-19 in adult patients: morbilliform rash, urticaria, vesicles, pseudo-chilblains, and vaso-occlusive lesions.

Necrotizing neutrophilic dermatosis: A diagnostic challenge with a need for multi-disciplinary recognition, a case report.

INTRODUCTION: Necrotizing neutrophilic dermatoses can clinically resemble necrotizing fasciitis and therefore pose a diagnostic and therapeutic challenge. Given their similar presentations, misdiagnosis and inappropriate or delayed treatments are possible. PRESENTATION OF CASE: We discuss the case of a woman with acute myeloid leukemia who presented with fevers, chills, cough, and a leg wound. She underwent amputation of her lower extremity after she was presumed to have necrotizing fasciitis; however, symptoms persisted. She was ultimately diagnosed with and treated for necrotizing Sweet's syndrome with notable clinical improvement. DISCUSSION: Both, necrotizing neutrophilic dermatoses and necrotizing fasciitis, grossly affect the skin and are associated with rapidly progressing systemic features including fevers, chills, leukocytosis, and elevated inflammatory markers. Recent literature in dermatology addresses these similarities and the appropriate approach to management; however, it is critical that medical and surgical subspecialties have an understanding of necrotizing neutrophilic dermatoses and their clinical presentations, diagnostic approaches, as well as therapeutic interventions. Familiarity with this entity can mitigate the risk of misdiagnosis, morbidity, and mortality. CONCLUSION: With this report, we seek to review the features that are suggestive of and aid in the diagnosis of necrotizing neutrophilic dermatoses to help prevent significant and avoidable morbidity.

Recurrent Staphylococcal Scalded Skin Syndrome in an Extremely Low-Birth-Weight Neonate.

Staphylococcal scalded skin syndrome (SSSS) in premature infants is a rare condition. We present SSSS in an extremely low-birth-weight (ELBW) infant with recurrent and confirmed bacterial sepsis. We present it to emphasize the importance for clinicians to not only recognize the clinical manifestations of SSSS, but also the need to closely monitor infants, especially very low-birth-weight (VLBW) and ELBW infants with SSSS for recurrence and bacterial sepsis. SSSS in preterm infants is a potentially lethal condition and early recognition and appropriate supportive care could be life-saving.