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Dermatology Online Journal Mar 2024Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on...
Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on the trunk. Herein, we report a clinically confounding case of disseminated superficial granulomatous pyoderma gangrenosum in a patient with a 5-year history of painful and chronic ulcerations of the bilateral upper extremities and face in a sun exposed distribution. This was a diagnostically challenging case due to the treatment-refractory nature of our patient's skin lesions and the atypical clinical and histologic presentations encountered. We review our clinical decision process and acknowledge other entities that were considered during the clinical course of this case. Additionally, we discuss the lack of responsiveness to various treatment options with eventual successful clearance of this patient's active skin disease with initiation of adalimumab.
Topics: Humans; Pyoderma Gangrenosum; Adalimumab; Female; Male; Granuloma; Middle Aged; Suppuration; Dermatitis
PubMed: 38762855
DOI: 10.5070/D330163283 -
BioRxiv : the Preprint Server For... Apr 2024Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis causing chronic and recalcitrant painful ulcerations. Pathogenic mechanisms are yet poorly understood...
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis causing chronic and recalcitrant painful ulcerations. Pathogenic mechanisms are yet poorly understood limiting therapeutic options, however, IL-12/IL-23 inhibition via ustekinumab has previously been associated with positive outcomes. We aimed to elucidate the dysregulated immune landscape of PG and lesional skin changes associated with IL-12/IL-23 blockade. We applied spatial transcriptomics and comparative computation analysis on lesional biopsies from two patients obtained before and after IL-12/IL-23 blockade with ustekinumab. Our data indicate lesional PG skin exhibits complex patterns of inflammation, including a not previously described major infiltration of B cells and establishment of tertiary lymphoid structures. In both patients, IL-12/IL-23 blockade led to marked clinical improvement but was associated with amelioration of contrasting inflammatory pathways. Notably, plasma cell markers and tertiary structures were recalcitrant to the treatment regime suggesting that B cells might play a role in the refractory nature of PG.
PubMed: 38746177
DOI: 10.1101/2024.04.26.591387 -
JAAD Case Reports Jun 2024
PubMed: 38741660
DOI: 10.1016/j.jdcr.2023.08.045 -
Journal of Gastroenterology and... May 2024Extraintestinal manifestations (EIMs) pose a significant threat in inflammatory bowel disease (IBD) patients. Vedolizumab (VDZ) primarily affects the gastrointestinal...
BACKGROUND AND AIM
Extraintestinal manifestations (EIMs) pose a significant threat in inflammatory bowel disease (IBD) patients. Vedolizumab (VDZ) primarily affects the gastrointestinal tract. However, its impact on EIMs remains uncertain. Therefore, we conducted this meta-analysis to examine the effects of VDZ on EIMs during treatment.
METHODS
Relevant studies were identified by conducting thorough searches across electronic databases, including PubMed, Ovid Embase, Medline, and Cochrane CENTRAL. Primary outcomes focused on the proportion of patients with resolution for pre-existing EIMs in IBD patients receiving VDZ. Secondary outcomes included the proportion of patients with EIM exacerbations and new onset EIMs during VDZ treatment.
RESULTS
Our meta-analysis encompassed 21 studies. The proportion of patients with resolution of pre-existing EIMs in VDZ-treated IBD patients was 39% (150/386; 95% confidence interval [CI] 0.31-0.48). The proportion of patients with EIM exacerbations occurred at a rate of 28% (113/376; 95% CI 0.05-0.50), while new onset EIMs had a rate of 15% (397/2541; 95% CI 0.10-0.20). Subgroup analysis revealed a 40% (136/337) proportion of patients with resolution for articular-related EIMs and a 50% (9/18) rate for erythema nodosum. Exacerbation rates for arthritis/arthralgia, erythema nodosum/pyoderma gangrenosum, and aphthous stomatitis during VDZ use were 28% (102/328), 18% (7/38), and 11% (3/28), respectively. The incidence rate of newly developed EIMs during treatment was 11% (564/4839) for articular-related EIMs, with other EIMs below 2%.
CONCLUSION
VDZ demonstrates efficacy in skin-related EIMs like erythema nodosum and joint-related EIMs including arthritis, arthralgia, spondyloarthritis, and peripheral joint diseases. Some joint and skin-related EIMs may experience exacerbation during VDZ therapy.
PubMed: 38740543
DOI: 10.1111/jgh.16612 -
Cureus Apr 2024Pyoderma gangrenosum is a rare ulcerative skin disease of uncertain etiology, which in some cases can be misdiagnosed as an infectious process. In even more unique...
Pyoderma gangrenosum is a rare ulcerative skin disease of uncertain etiology, which in some cases can be misdiagnosed as an infectious process. In even more unique cases, this can occur in the postoperative period. Termed postsurgical pyoderma gangrenosum, this type of inflammatory skin condition requires a high index of suspicion to be able to appropriately treat and reduce complications. We present a 55-year-old female who presented with multiple wounds following mastopexy and abdominoplasty. With a prompt diagnosis and a multidisciplinary approach, we could accurately care for the patient and minimize poor aesthetic sequela.
PubMed: 38738036
DOI: 10.7759/cureus.58060 -
Anais Brasileiros de Dermatologia May 2024Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers.
BACKGROUND
Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers.
OBJECTIVE
We aimed to evaluate the demographic, clinical characteristics, treatments and factors affecting the treatment responses of patients with PG.
METHODS
We performed a multicenter study of 12 tertiary care centers. We analyzed the data of the patients who were followed up with a diagnosis of PG between the years 2012‒2022 retrospectively.
RESULTS
We included a total of 239 patients of whom 143 were female and 96 were male, with an average age of 54.2 ± 17.4 years. The most common treatment was systemic steroids (n = 181, 75.7%). Among these patients, 50.8% (n = 92) used systemic steroids as the sole systemic agent, while 49.2% (n = 89) used at least one adjuvant immunosuppressive agent. The independent factors determined in regression analysis to influence response to systemic steroids positively were disease onset age ≥ 30-years, negative pathergy, absence of leukocytosis, negative wound culture, presence of a single lesion, and absence of upper extremity involvement. Biological agents were used in 18.4% (n = 44) of the patients in the present study. We also analyzed pathergy positive PG and early onset (onset age < 30) PG separately due to their distinct clinical features which were revealed during statistical analysis.
STUDY LIMITATIONS
Retrospective nature of the present study.
CONCLUSIONS
Analyses of the factors influencing treatment responses are addressed in this study. Also, we concluded that investigation for accompanying autoinflammatory diseases of pathergy positive PG and early onset PG is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments.
PubMed: 38735817
DOI: 10.1016/j.abd.2024.02.002 -
International Journal of Dermatology May 2024
PubMed: 38727093
DOI: 10.1111/ijd.17200 -
Cureus Apr 2024Pyoderma Gangrenosum (PG) is a distinctive dermatologic condition characterized by recurrent inflammatory ulcers, often manifesting with violaceous borders and...
Pyoderma Gangrenosum (PG) is a distinctive dermatologic condition characterized by recurrent inflammatory ulcers, often manifesting with violaceous borders and undermined edges. We describe a 40-year-old male who presented with acute on chronic necrotic ulcer of the left index finger following foreign body penetration. Despite multiple emergency department visits and treatments for presumed recurrent cellulitis, including various debridements, his condition persisted without symptomatic relief. A high index of clinical suspicion, due to recurrent presentations and potential pathergy, prompted an excision biopsy which confirmed Pyoderma Gangrenosum (PG). Regrettably, due to delays in appropriate management, the patient chose amputation because of intolerable pain, highlighting the critical importance of timely diagnosis for optimal patient outcomes.
PubMed: 38716010
DOI: 10.7759/cureus.57762 -
Journal of the American Academy of... Apr 2024
Low incidence of invasive fungal infection and risk factors in a large observational cohort of patients initiating tumor necrosis factor-alpha inhibitors for dermatologic conditions.
PubMed: 38685411
DOI: 10.1016/j.jaad.2024.03.046 -
Cureus Mar 2024Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic dermatosis. The ulcerative subtype presents with a tender nodule or pustule that progresses into a...
Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic dermatosis. The ulcerative subtype presents with a tender nodule or pustule that progresses into a painful, necrotic ulcer.New lesions arise after minor trauma in one-third of patients, a phenomenon termed "pathergy." We present a 62-year-old Caucasian female with primary sclerosing cholangitis, hepatic cirrhosis, chronic hepatitis B, and severe PG. At the initial presentation, she had lesions on her face and four extremities. She had severe full-thickness ulcerations on the bilateral cheeks and underwent incision and drainage with washout of bilateral maxillary abscesses, left sinus curettage, and wound debridement. She has required multiple hospitalizations for severe flares. Treatment with steroids was complicated by spinal compression fractures. Steroid-sparring agents were ineffective. Her lesions involved bilateral cheeks, temples, temporal scalp, and eyelids with oroantral fistulae. Her facial ulcerations included a large septal perforation causing saddle nose deformity and eradication of a branch of the left facial nerve causing incomplete eye closure. She underwent bilateral facial wound irrigation with antibiotic irrigation and wound debridement. Due to social factors, she has been lost to follow-up and a definitive etiology of her PG has not yet been elucidated. Although rare, PG should remain a consideration in patients with ulcerative lesions on the head and neck. Wound debridement is typically discouraged given the risk of pathergy, but there may be a role for surgical intervention in adequately immunosuppressed patients.
PubMed: 38681354
DOI: 10.7759/cureus.57136