seventy-Second issue
October 30, 2024
Peeling back the layers: What puts patients at risk for epidermal necrolysis mortality?
Epidermal necrolysis (EN) is a rare, mainly drug-induced, widespread epidermal and mucosal detachment, which includes Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Evidence regarding its incidence may be overestimated due to overdiagnosis, while the long-term mortality has rarely been investigated. This retrospective cohort study of the French Health System aimed to determine the incidence of and risk factors for in-hospital and long-term mortality due to EN.
What did they find?
Limitations: This study may have underestimated the incidence and overestimated the mortality due to the following selection criteria: exclusion of SJS patients with associated diagnoses of M pneumoniae pneumopathy, generalized exanthema due to drugs, and lengths of stay of 14 days or longer.
Main Takeaway: Although a rare diagnosis, patients of older age and with comorbidities experience higher rates of mortality due to EN, with post-discharge mortality being further associated with acute in-hospital complications.
Epidermal necrolysis (EN) is a rare, mainly drug-induced, widespread epidermal and mucosal detachment, which includes Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Evidence regarding its incidence may be overestimated due to overdiagnosis, while the long-term mortality has rarely been investigated. This retrospective cohort study of the French Health System aimed to determine the incidence of and risk factors for in-hospital and long-term mortality due to EN.
What did they find?
- The incidence of EN was 2.6 (95% CI, 2.5-2.7) per million person-years.
- The mortality rate at 1 year was 34% (95% CI, 31%-36%). The in-hospital mortality rate was 19% (95% CI, 17%-21%), and the post-discharge mortality rate was 15% (95% CI, 13%-17%).
- Age (adjusted hazard ratio [AHR]=1.03 per year of age; 95% CI: 1.02-1.04), history of cancer (AHR=2.04; 95% CI: 1.53-2.72), dementia (AHR=1.85; 95% CI: 1.12-3.07), liver disease (AHR=1.81; 95% CI: 1.24-2.64), and EN severity (TEN vs SJS: AHR=2.14; 95% CI: 1.49-3.07) were associated with in-hospital mortality.
- Cancer (AHR=3.26; 95% CI: 2.35-4.53), liver disease (AHR=1.86; 95% CI: 1.11-3.13), dementia (AHR=1.95; 95% CI: 1.11-3.43), and acute complications (acute kidney injury: AHR=2.14, 95% CI: 1.26-3.63; sepsis: AHR=2.44, 95% CI: 1.42-4.18) were associated with postdischarge mortality.
Limitations: This study may have underestimated the incidence and overestimated the mortality due to the following selection criteria: exclusion of SJS patients with associated diagnoses of M pneumoniae pneumopathy, generalized exanthema due to drugs, and lengths of stay of 14 days or longer.
Main Takeaway: Although a rare diagnosis, patients of older age and with comorbidities experience higher rates of mortality due to EN, with post-discharge mortality being further associated with acute in-hospital complications.
The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: A Surveillance, Epidemiology, and End Results database analysis
Journal of the American Academy of Dermatology
Journal of the American Academy of Dermatology
People want to SEEr up-to-date research on dermatofibrosarcoma protuberans!
Dermatofibrosarcoma protuberans (DFSP), a rare soft tissue sarcoma of the skin, is a slowly progressive, locally invasive neoplasm with low risk of metastasis, found predominantly on the trunk and upper extremities. Limited data exists on the clinical factors and patient demographics associated with metastasis and survival of DFSP. Using the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database, this retrospective cohort study aimed to characterize the incidence, metastasis, and survival outcomes for DFSP.
What did they find?
Main Takeaway: Clinical features of DFSP, such as increased tumor size and sensitive location, in addition to patient demographics including older age and Black race, pose risks for worse outcomes in patients diagnosed with DFSP.
Dermatofibrosarcoma protuberans (DFSP), a rare soft tissue sarcoma of the skin, is a slowly progressive, locally invasive neoplasm with low risk of metastasis, found predominantly on the trunk and upper extremities. Limited data exists on the clinical factors and patient demographics associated with metastasis and survival of DFSP. Using the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database, this retrospective cohort study aimed to characterize the incidence, metastasis, and survival outcomes for DFSP.
What did they find?
- The incidence of DFSP, measured in cases per million person-years, was significantly higher in Black patients at 8.74 (95% CI, 8.04-9.44) compared to White patients at 4.53 (95% CI, 4.27-4.77).
- Metastatic risk was significantly associated with tumors greater than 3 cm in size (OR 2.24, p < 0.001), tumors of the head and neck (OR 4.88, p < 0.001), and tumors of the genitalia (OR 3.16, p < 0.023).
- Patients 60 or over (HR: 3.47, 95% CI, 1.68-7.20, p < 0.001) and tumors greater than 3 cm (HR: 5.34, 95% CI, 1.85-15.39, p = 0.002) were associated with decreased overall and cancer-specific survival.
Main Takeaway: Clinical features of DFSP, such as increased tumor size and sensitive location, in addition to patient demographics including older age and Black race, pose risks for worse outcomes in patients diagnosed with DFSP.
Changes in patient-reported symptoms after vascular malformation treatment can be measured using Outcome measures for VAscular MAlformations (OVAMA) questionnaire
British Journal of Dermatology
British Journal of Dermatology
OVAMA questionnaire is improving vascular malformation care!
Vascular malformations (VMs) are congenital abnormalities of the vascular and lymphatic systems that can be symptomatic and significantly impact appearance, often reducing patients’ quality of life. Although treatments for VMs exist, no standardized method exists for evaluating treatment outcomes from the patient’s perspective. To address this gap, the Outcome measures for VAscular MAlformations (OVAMA) questionnaire was developed. This prospective study of 63 patients aimed to validate the OVAMA questionnaire by comparing baseline scores to those at 8 weeks, alongside global rating of change (GRC) scales.
What did they find?
Main Takeaway: The OVAMA questionnaire is a reliable tool for evaluating changes after VM treatment, providing a uniform method for patients to report VM appearance and symptoms and ultimately improving the quality of research and clinical management of VMs.
Vascular malformations (VMs) are congenital abnormalities of the vascular and lymphatic systems that can be symptomatic and significantly impact appearance, often reducing patients’ quality of life. Although treatments for VMs exist, no standardized method exists for evaluating treatment outcomes from the patient’s perspective. To address this gap, the Outcome measures for VAscular MAlformations (OVAMA) questionnaire was developed. This prospective study of 63 patients aimed to validate the OVAMA questionnaire by comparing baseline scores to those at 8 weeks, alongside global rating of change (GRC) scales.
What did they find?
- Invasive therapy saw greater improvement in VM symptoms than conservative therapy (P = 0.003).
- Indications of improvement or worsening on the GRC scale saw corresponding changes with the OVAMA questionnaire in almost all constructs, providing validity to the OVAMA questionnaire.
- Of the 75 hypotheses identified to assess the correlation between OVAMA scores and GRC scales, 63 (84%) of the hypotheses were confirmed by OVAMA.
Main Takeaway: The OVAMA questionnaire is a reliable tool for evaluating changes after VM treatment, providing a uniform method for patients to report VM appearance and symptoms and ultimately improving the quality of research and clinical management of VMs.
What is the prevalence and contributing factors of disabilities among leprosy patients in Guinea?
Global Dermatology
Global Dermatology
LeproSEE the impact: Addressing disability and stigma in leprosy care
Leprosy is a chronic bacterial infection that can lead to severe disabilities if not diagnosed and treated early. In 2021, the World Health Organization reported over 140,000 new cases across Asia, the Americas, and Africa. Disabilities related to leprosy, including sensory loss, motor impairments, and deformities, often lead to social discrimination, with factors such as delayed diagnosis, older age, and limited resources further increasing the risk of disability. This cross-sectional study aims to assess the frequency of disability and contributing factors among 115 leprosy patients at Guinea’s Kindia Rehabilitation Centre between 2017 and 2021.
What did they find?
Main Takeaway: Leprosy-related disabilities remain a significant issue in Guinea, especially among males and multibacillary cases. These findings emphasize the need for improved healthcare training, community awareness, and early intervention strategies to reduce disability and stigma.
Leprosy is a chronic bacterial infection that can lead to severe disabilities if not diagnosed and treated early. In 2021, the World Health Organization reported over 140,000 new cases across Asia, the Americas, and Africa. Disabilities related to leprosy, including sensory loss, motor impairments, and deformities, often lead to social discrimination, with factors such as delayed diagnosis, older age, and limited resources further increasing the risk of disability. This cross-sectional study aims to assess the frequency of disability and contributing factors among 115 leprosy patients at Guinea’s Kindia Rehabilitation Centre between 2017 and 2021.
What did they find?
- 52.2% (60) of included patients were male, and 55.7% (64) were from rural communities. The majority of patients were aged 15-30 (43.5%, 50), with an average age of 37.7.
- 82.1% (94) of patients had multibacillary leprosy.
- 55.6% (64) of patients experienced symptoms for 7-12 months.
- Disabilities were present in 75.7% (87) of patients, with 48.7% (56) showing grade II disability.
- A significantly higher rate of disability was reported in males (P = 0.004) and multibacillary cases (P = 0.001).
- Nearly 78% (90) of patients received corticosteroid therapy, and 92.2% (106) were reported cured upon discharge.
Main Takeaway: Leprosy-related disabilities remain a significant issue in Guinea, especially among males and multibacillary cases. These findings emphasize the need for improved healthcare training, community awareness, and early intervention strategies to reduce disability and stigma.
(Port) WHINE no more with OCT
Optical coherence tomography (OCT) is a noninvasive imaging technique that uses light waves to capture images of the skin and cutaneous blood vessels. Port-wine birthmarks (PWBs) derive their name from their wine-splashed appearance, caused by a congenital malformation of capillaries that often requires treatment to prevent progression. Current treatments, such as pulsed-dye lasers, face challenges in fully clearing lesions due to the diversity of vessels within the PWBs. This study hypothesized that OCT could effectively visualize PWB vessels and assess their structural variability, aiding in future treatment planning.
What did they find?
Main Takeaway: Optical coherence tomography shows promise in its ability to better map and characterize port-wine birthmarks, but further validation is needed to validate its efficacy in guiding treatment.
Optical coherence tomography (OCT) is a noninvasive imaging technique that uses light waves to capture images of the skin and cutaneous blood vessels. Port-wine birthmarks (PWBs) derive their name from their wine-splashed appearance, caused by a congenital malformation of capillaries that often requires treatment to prevent progression. Current treatments, such as pulsed-dye lasers, face challenges in fully clearing lesions due to the diversity of vessels within the PWBs. This study hypothesized that OCT could effectively visualize PWB vessels and assess their structural variability, aiding in future treatment planning.
What did they find?
- 10 participants, with Fitzpatrick skin types I-IV, with a total of 15 PWB lesions, were included in the study.
- Average blood vessel density across lesions measured with OCT was 11.6% (range 0.5%-41.3%) for affected and 8.7% (0.4%-33.5%) for control skin.
- Average blood vessel diameter across lesions measured 94.6μm (range 12.0μm-425μm) in affected and 69.0μm (11.3μm-191.6μm) in control skin.
- The diameter of vessels increased by an average of 25.7μm (P<0.05, 95% CI, 18.3μm-33μm) when comparing PWBs and control areas at the same cutaneous depth.
- The density of the vessels increased by an average of 2.9% (P<0.05, 95% CI, 1.9%-4.0%) when comparing PWBs and control areas at the same cutaneous depth.
Main Takeaway: Optical coherence tomography shows promise in its ability to better map and characterize port-wine birthmarks, but further validation is needed to validate its efficacy in guiding treatment.