ABSTRACT
Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that is occasionally associated with primary immunodeficiency. Though contributions from dysregulation of the innate immune system, neutrophil dysfunction and genetic predisposition have been postulated, the precise pathogenesis of PG has not yet been elucidated. This article reviews reported cases of coexisting PG and primary immunodeficiency in order to gain insight into the complex pathophysiology of PG. Our findings suggest that variations in genes such as RAG1, ITGB2, IRF2BP2 and NFκB1 might play a role in genetically predisposing patients to develop PG. These studies support the feasibility of the role of somatic gene variation in the pathogenesis of PG which warrants further exploration to guide targeted therapeutics.
Subject(s)
Dermatitis , Pyoderma Gangrenosum , Humans , Pyoderma Gangrenosum/genetics , Genetic Predisposition to DiseaseABSTRACT
ABSTRACT: Pyoderma gangrenosum is an inflammatory skin disease that presents with rapidly progressive ulcers with violaceous, undermined borders. Despite most commonly affecting the lower extremities, pyoderma gangrenosum can rarely present in the genital, anal, and perineal regions. We describe 2 cases and report a review of published cases.
Subject(s)
Perineum , Pyoderma Gangrenosum , Humans , Pyoderma Gangrenosum/drug therapy , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/pathology , Perineum/pathology , Male , Female , Adult , Anus Diseases/pathology , Middle Aged , Anal Canal/pathology , Treatment OutcomeABSTRACT
In this CME, we review two specific categories of ulcers: inflammatory (where inflammation is the primary pathologic process leading to ulceration) and vaso-occlusive (where occlusion is the primary process). Inflammatory ulcers include pyoderma gangrenosum and vasculitides, whereas livedoid vasculopathy, calciphylaxis and Martorell ulcers are vaso-occlusive ulcers. Determining the causes of ulcers in these conditions may require laboratory evaluation, biopsy and imaging.
ABSTRACT
In the second part of this CME, we present an approach for the management of inflammatory and vaso-occlusive ulcers and highlight the need for further research in this field. The three overarching principles for management are etiology-specific treatment, ulcer care, and consideration of patient comorbidities and risk factors for poor healing. Both etiology-specific treatment and management of patient comorbidities and risk factors often require collaboration with providers from other specialties. Ulcer care is governed by TIME, or tissue debridement, infection control, management of moisture imbalance and epithelial edge advancement. As wound healing is a dynamic process, management should be adapted to changes in the status of the ulcer.
ABSTRACT
INTRODUCTION: Neutrophilic dermatoses (NDs) often occur secondary to inflammatory conditions, medication exposure, and hematologic malignancy. While malignancy-associated NDs (MA-NDs) have been well reported among those with hematologic cancers, little is known about drug-induced NDs (DI-NDs) within this population. The objective of this study was to compare the presentations and outcomes of patients with hematologic malignancies who developed MA-NDs and DI-NDs. METHODS: Cases of ND occurring between 2013 and 2023 among those with hematologic malignancies were identified from the electronic medical records of our institution. Patient characteristics, recent medication exposures, cancer mutations, and disease outcomes were reviewed. Patients were categorized with DI-ND if they were recently exposed to one of four medications known to be commonly associated with ND or were otherwise categorized with MA-ND. We report a descriptive analysis of cases of DI-ND and MA-ND. RESULTS: We identified 52 patients with ND and co-occurring hematologic malignancy including 16 cases of DI-ND (30.8%) and 36 cases of MA-ND (69.2%). The most common ND in both groups was Sweet's syndrome. Chronic underlying conditions including solid tumors, inflammatory disorders, chronic viral infection, and tobacco use were more common among those with MA-ND. Among those with DI-ND, tyrosine kinase inhibitors were the most commonly associated drugs (43.8%). The most common cancer mutation among those with DI-ND was FLT3 (43.8%), while the most common mutation among those with MA-ND was TP-53 (19.4%). Among those who had died at the time of data collection, 90.0% of those with DI-ND and 66.7% of those with MA-ND died within 1 year of ND diagnosis. CONCLUSION: Most cases of ND occurring with hematologic malignancies develop secondary to cancer rather than drug exposure. Different cancer mutations may predispose to DI-ND and MA-ND. Further research is needed to establish diagnostic criteria for DI-ND and to determine the pathogenic role of specific cancer mutations, particularly FLT3, in the development of ND.
Subject(s)
Hematologic Neoplasms , Sweet Syndrome , Humans , Hematologic Neoplasms/complications , Female , Male , Middle Aged , Aged , Sweet Syndrome/epidemiology , Retrospective Studies , Adult , Aged, 80 and over , Drug Eruptions/etiology , Drug Eruptions/epidemiology , Neutrophils , Antineoplastic Agents/adverse effectsABSTRACT
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.
Subject(s)
Inflammatory Bowel Diseases , Pyoderma Gangrenosum , Skin Ulcer , Humans , Middle Aged , Autopsy , Pyoderma Gangrenosum/complications , Pyoderma Gangrenosum/diagnosis , Retrospective StudiesABSTRACT
The heterogeneity of atypical wounds can present diagnostic and therapeutic challenges; however, as the prevalence of atypical wounds grows worldwide, prompt and accurate management is increasingly an essential skill for dermatologists. Addressing the underlying cause of an atypical wound is critical for successful outcomes. An integrated approach with a focus on pain management and patient engagement is recommended to facilitate enduring wound closure. Advances in treatment, in addition to further research and clinical training, are necessary to address the expanding burden of atypical wounds.
Subject(s)
Wound Healing , Humans , Pain Management/methods , Wounds and Injuries/therapy , Wounds and Injuries/diagnosisABSTRACT
BACKGROUND: Trends in emergency department (ED) visits with a primary dermatologic diagnosis are not well characterized. OBJECTIVE: The goal is to determine how the number of ED visits attributable to dermatologic disease is changing and to characterize the type of dermatologic conditions seen. METHODS: This is a cross-sectional study using the National Hospital Ambulatory Medical Care Survey: Emergency Department Summary Tables 2008-2017. Data were compiled into tables on the amount and type of dermatologic ED visits. RESULTS: The percentage of dermatologic visits ranged from an estimated 3.5% to 4.3%, peaking in 2014. Cellulitis was the most common diagnosis and accounted for an estimated 1.3% of all visits in 2016, and an estimated 1.2% of visits in 2017. The second most common diagnosis was cutaneous abscess, which accounted for an estimated 0.8% in 2016 and 0.9% in 2017. CONCLUSION: The number of ED visits attributed to disease of the skin and subcutaneous tissue has not followed a general trend of increase or decrease. The most common diagnoses are infections. We can best serve these patients by emphasizing the importance of ED provider education on the management of cutaneous infections and by working to increase the accessibility of dermatologic care.
Subject(s)
Emergency Medicine , Skin Diseases , Cellulitis/epidemiology , Cellulitis/therapy , Cross-Sectional Studies , Emergency Service, Hospital , Health Care Surveys , Humans , Skin Diseases/diagnosis , Skin Diseases/epidemiology , Skin Diseases/therapy , United StatesABSTRACT
This survey study investigates the minimal important difference in pain indicating that a patient with pyoderma gangrenosum may be responding to treatment.