Pyoderma Gangrenosum In Patient Research Articles

Glucocorticoid sparing effect on Adalimumab for refractory Pyoderma gangrenosum in patients with Rheumatoid Arthritis: A Case series.

Pyoderma gangrenosum (PG) is a rare chronic skin disease characterized by painful skin ulcers. There are no treatment guidelines for PG; however, systemic treatment is often administered. Recently, adalimumab (ADA), a fully human monoclonal antibody against tumor necrosis factor, was approved for the treatment of refractory PG in Japan. However, data are limited, and it is not clear whether ADA has the same effect on the treatment of PG in patients with systemic rheumatic disease (SRD), including rheumatoid arthritis (RA). In addition, the glucocorticoid-sparing effect of ADA in SRD patients with PG has not yet been clarified. Herein, we present two successful cases of RA with glucocorticoid-refractory PG on ADA treatment. Our report suggests that ADA may have a glucocorticoid-sparing effect on refractory PG in patients with RA.

A Comprehensive Systematic Review of Depression and Anxiety in Adults with Pyoderma Gangrenosum

Background: Pyoderma gangrenosum (PG) is a rare, chronic inflammatory skin disease characterized by painful, necrotic ulcers that frequently lead to disfiguring scars and significantly impact patients’ quality of life. This interplay between PG and mental health issues like anxiety and depression underscores the need for holistic approaches to manage both the physical and psychological challenges faced by PG patients. Methods: This systematic review adhered to the PRISMA 2020 guidelines, focusing exclusively on full-text articles published in English between 2015 and 2024. Result: A total of over 600 publications were initially identified through the database search. Following a rigorous three-stage screening process, eight studies were selected for comprehensive analysis. These studies were critically examined to provide an in-depth evaluation of depression and anxiety among adults with pyoderma gangrenosum. Conclusion: Pyoderma gangrenosum, a chronic autoimmune disorder, significantly impacts patients' mental health due to painful ulcers, visible lesions, and social isolation. Untreated depression can exacerbate pain perceptions and reduce treatment satisfaction. Healthcare providers must screen for and address mental health conditions in PG patients, utilizing psychological support and counseling to address both physical and mental health issues.

Pyoderma Gangrenosum: A Retrospective Case Series of 44 Patients.

Pyoderma gangrenosum (PG) poses a significant dermatological challenge due to its rapidly evolving painful necrotic ulcerations. Understanding its multifaceted pathogenesis and diverse clinical presentation is crucial for effective management. We aimed to analyze demographic characteristics, clinical manifestations, lesion distributions, systemic disease associations, therapeutic interventions, and patient outcomes in PG cases. Medical records from 2017 to 2023 of PG patients at IRCCS Sant'Orsola Malpighi Hospital, Bologna, Italy, were retrospectively analyzed. Inclusion criteria encompassed persistent ulcers with clinical and histological evidence of PG, excluding cases with alternative diagnoses or inadequate follow-up. Clinical evaluations, including pain assessment and lesion measurements, were conducted at diagnosis and follow-up visits. A total of 44 patients were evaluated. Pain was a universal symptom, and tissue pathergy was documented in 28.6% of patients. Ulcerative PG was the most common subtype (88.1%). Associations with inflammatory bowel diseases (25%), rheumatoid arthritis (9.1%), and hematological diseases (17.2%) were noted. Lower limbs were frequently affected (63.6%). Treatment approaches included wound management, topical and systemic corticosteroids, and immunosuppressive therapy, with varying response rates. Advanced dressing and steroid therapy were pivotal in mild PG cases, while moderate-to-severe cases often associated with systemic diseases showed incomplete healing despite treatment, especially in patients with inflammatory bowel diseases and hematological disorders. This study contributes to the understanding of PG's complexities, suggesting the use of biological therapy as first line in moderate-to-severe PG.

Monoclonal gammopathy in the setting of Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.

Clinical, epidemiological, and therapeutic hallmarks of pyoderma gangrenosum: a case series of 35 patients.

Over the past few decades, advances in medical research and diagnostic tools have shed light on some aspects of pyoderma gangrenosum (PG). Nevertheless, the multifactorial etiology, pathogenesis, and optimal management strategies for PG need to be further investigated. To address these knowledge gaps and contribute to a better understanding of this complex dermatological disorder, we collected epidemiological, clinical, and therapeutic aspects of a case series of PG patients occurring in our department over the past 10 years. We performed a single-centered, retrospective, observational study analyzing all cases with a diagnosis of PG observed at the Dermatology clinic of the Fondazione Policlinico A. Gemelli IRCCS Catholic Universityfrom January 1, 2013, to January 1, 2023. For each case, we retrieved demographic data, the presence of other skin and systemic conditions, and the histopathological and clinical characteristics of PG, such as clinical variant, number of lesions, disease localization, previous therapy, response to treatment, and occurrence of relapse. We included 35 patients, 22 females and 13 males with a mean age of 40.0 years. Twenty patients (57.1%) had multiple localizations of disease, and the most commonly involved site was the lower limbs (85.7%). The lesions were mainly associated with inflammatory bowel diseases (51.4%) and hidradenitis suppurativa (37.1%). Clinical resolution with complete re-epithelialization was achieved in 25 patients (71.4%) with an average time of 20.8 months. On average, patients who underwent therapy with biological drugs had better outcomes. PG is a severe, rare, and pleomorphic disease associated with a broad spectrum of conditions. Corticosteroids remain the primary first-line approach for severe forms, but using biological immunosuppressants is promising.

Bioabsorbable Temporizing Matrix (BTM): Not Just for Burns.

A skin substitute developed in Australia 2 decades ago for use in acute burns was recently introduced into the United States for the treatment of open wounds. This product has been shown to be very efficacious for coverage of debrided burn wounds. It consists of an inorganic synthetic woven layer that induces cellular ingrowth and deposition of interstitial tissue. It is covered by an attached silicone layer that seals the wound. The product is placed with the woven side directly on the wound. It is fixed in place and optimally covered by a negative pressure dressing for the first 5-10 days. Due to its synthetic composition, it is highly resistant to infection and can stay on the wound for as long as needed. The authors have used this matrix in a wide variety of complex reconstructions in 27 patients, consisting of 10 females and 17 males. Eleven had traumatic wounds of various etiologies (deep burns, crush injuries, Morell-Lavallee lesion, hand injuries, multiple trauma, open fractures, compartment syndromes and soft tissue avulsions). The following wounds were also treated with BTM: pressure ulcers, axillary hidradenitis, scalp wounds, severe facial acne keloidalis, Fournier's gangrene, a diabetic foot ulcer, a chronic venous ulcer, a cutaneous pyoderma gangrenosum, a radiation ulcer with exposed Achilles tendon, a disfiguring scar from an old Dupytrens excision and a non-healing chemotherapy extravasation ulcer. Hand and leg wounds with exposed tendon achieved coverage without tethering. The scalp wounds developed a neodermis and were skin-grafted. A pyoderma gangrenosum patient with excessive slough and uncontrolled bleeding was temporarily immunosuppressed, aggressively debrided and covered with the matrix. Her bleeding resolved. She developed a neodermis, and had reduced symptoms. The Fournier's patient had immediate skin-grafting of his penis and testicles, but the remaining extensive perineal and upper thigh wounds were treated with the matrix and healed without additional skin grafting. A hand patient with a forearm fasciotomy wound was covered with the matrix. As the swelling resolved the size of the defect decreased to the point that the wound was delaminated. The adjacent skin was elevated and used to cover the open area, achieving full closure. There were no complications attributed to the use of the matrix. Several patients healed secondarily with the silicone sheeting gradually peeling off. Seven patients have received or are awaiting skin grafts to be placed over the neodermis. Although this is an early review of the use of this synthetic matrix in the US, it comes with a positive legacy from Australia. The findings thus far indicate that there is a wide range of applications for this product well beyond burn care. Its safety record, resistance to infection and ease of use facilitate surgery.

Pyoderma gangrenosum: A single-center case series study of 32 cases

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. It often presents as ulcerated lesions with a violet/erythematous border and an irregular undercut margin. In this largest single-centre case series study in Antalya-Turkey, we reviewed 32 PG patients diagnosed consecutively within the last 5-year period. Consistent with the literature, PG morphologically often presented with the ulcerative clinic (90.6%), and inflammatory bowel disease (15.6%) was the most common etiologic factor. In our study, female gender predominance (78.2%), lower extremity localization (93.7%), and the rate of multiple ulcers at diagnosis (90.6%) were more prominent than in the literature. Unlike the literature, oral mucosa involvement and syndromic form (both, 6.2%) of the disease were detected more frequently. Our results indicate that PG patients may show differences according to geographical and ethnic differences and/or characteristics of the healthcare institution.

The presence of neutrophil extracellular traps in different forms of pyoderma gangrenosum

AbstractWe demonstrated that there were abundant neutrophil extracellular traps (NETs) in the skin biopsies from various types of pyoderma gangrenosum (PG), based on the observation of extended and compact areas of immunolabeling of MPO and Cit H3 proteins. We suggest that neutrophils could undergo an aberrant NET formation in the lesions of PG patients, in the vast majority of idiopathic PG. We did not detect NETs in the skin ulcers of an antiphospholipid syndrome patient with a similar appearance to classical ulcerative PG, while rich NETs were found in the various types of PG. These findings suggest that the presence of NETs in skin tissues could serve as a marker for making differential diagnoses of various types of PG from other similar conditions.

Minimum data set for treatment effectiveness in pyoderma gangrenosum (MIDSTEP): an international protocol of an e-Delphi study to develop a clinical physician-driven treatment effectiveness registry on behalf of the UPGRADE initiative.

Pyoderma gangrenosum (PG) is a rare inflammatory condition with an immense disease burden that remains understudied. With limited approved treatments and low-quality clinical evidence, PG continues to have poor patient outcomes. Unfortunately, improvement in PG treatments and patient care is based on additional research endeavors that can only be developed from existing high-quality data. The following protocol outlines the development of the Minimum Data Set for Treatment Effectiveness in Pyoderma gangrenosum (MIDSTEP), a core set of domains and domain items for the Pyoderma Gangrenosum Treatment Effectiveness (PyGaTE) international registry. The outcomes and benefits are focused on providing real-world data for physicians to improve their clinical decisions on PG treatment and inform clinical trial design, promoting clinical research among the international scientific community. MIDSTEP is a multi-phase project. The first phase will produce a domain item list from a literature review to take into the second phase which would finalize the core data set by an e-Delphi exercise. There will be a single stakeholder group participating together in the e-Delphi consisting of PG experts (healthcare providers, researchers, methodologists, industry representatives, and regulators), ulcerative PG patients, and PG patient advocates. The methodology outlined in the protocol is a systematic method based on several guidelines through COMET and established dermatologic registries and outcome sets with systematic methodologies of their own. The third phase will identify the instruments for the items, the 'when to measure' the items, and the platform for the registry. The last phase is the implementation and continued maintenance of the international registry PyGaTE. By solidifying a consensus on standardized outcomes and collecting information on PG treatment effectiveness in a centralized database, existing treatments can be compared more systematically and analyzed with increased evidence. MIDSTEP and the PyGaTE international registry will have the ambitious goal to generate and disseminate real-world data that can be used by all stakeholders to improve health outcomes for PG patients. Future potential for the outcome of this project includes the development of a gold-standard PG treatment.

Recalcitrant Pyoderma Gangrenosum: Clinical Burden and Unmet Needs.

Pyoderma gangrenosum (PG) is a rare, autoinflammatory disease leading to aseptic ulcers which carries a significant disease burden and is often difficult to treat, with many patients failing first-line treatment and requiring additional therapies. Such cases are typically referred to in the literature as "recalcitrant", "refractory", or "resistant", though little is known about the clinical characteristics of such cases. We performed a narrative literature review to characterize patient demographics and clinical course associated with difficult to treat pyoderma gangrenosum cases in order to identify trends to guide future clinical management and therapeutic innovation. We identified 148 cases with clinical manifestations and associated patient demographics stratified by ulcer and patient features. Consistent with previous work, a greater prevalence of PG was observed among female patients and those with a history of inflammatory bowel disease, however interestingly despite an aggressive course to their PG, few patients had comorbidities complicating their disease course. Additionally, despite the requirement of three or more treatments for most patients' disease to resolve, the majority healed within the typical window observed in previous clinical studies with low rates of recurrence. Biologics were the most common medication patients were on at time of remission. Collectively, our results suggest a potential benefit for a reduced threshold for biologic initiation in PG patients and a need for standardization of language in the field to facilitate treatment outcomes comparisons and interventions.

Predilection sites of pyoderma gangrenosum: Retrospective study of 170 clearly diagnosed patients.

Pyoderma gangrenosum (PG) is a non-infectious, neutrophilic dermatosis that was difficult to diagnose in clinical practice. Today, the PARACELSUS score is a validated tool for diagnostics. Based on this score, patients with clearly diagnosed PG were examined with regard to predilection sites. In this retrospective study, the data of patients from the University Hospitals of Essen and Erlangen were analysed in whom the diagnosis of PG could be clearly confirmed using the PARACELSUS score. A total of 170 patients, 49 men (29%) and 121 women (71%) with an average age at first manifestation of 55.5 years, could be included in the analysis. The predilection sites were identified as the lower legs in 80.6% of the patients and the extensor sides in 75.2%. Other localisations of PG were the thighs in 14.1%, mammae and abdomen in 10.0% each, back and gluteal in 7.1% each, feet in 5.9%, arms in 4.7%, genital in 3.5% and head in 2.9%. This retrospective study is the first to identify a collective of PG patients with the highest data quality using the PARACELSUS score. It could be shown that PG can basically occur on the entire integument. However, the predilection sites of PG, which have now been reliably identified for the first time, are the lower legs and in particular the extensor sides.

A novel murine model of pyoderma gangrenosum reveals that inflammatory skin-gut crosstalk is mediated by IL-1β-primed neutrophils.

Pyoderma gangrenosum (PG) is a debilitating skin condition often accompanied by inflammatory bowel disease (IBD). Strikingly, ~40% of patients that present with PG have underlying IBD, suggesting shared but unknown mechanisms of pathogenesis. Impeding the development of effective treatments for PG is the absence of an animal model that exhibits features of both skin and gut manifestations. This study describes the development of the first experimental drug-induced mouse model of PG with concomitant intestinal inflammation. Topical application of pyrimidine synthesis inhibitors on wounded mouse skin generates skin ulcers enriched in neutrophil extracellular traps (NETs) as well as pro-inflammatory cellular and soluble mediators mimicking human PG. The mice also develop spontaneous intestinal inflammation demonstrated by histologic damage. Further investigations revealed increased circulating low density IL-1β primed neutrophils that undergo enhanced NETosis at inflamed tissue sites supported by an increase in circulatory citrullinated histone 3, a marker of aberrant NET formation. Granulocyte depletion dampens the intestinal inflammation in this model, further supporting the notion that granulocytes contribute to the skin-gut crosstalk in PG mice. We anticipate that this novel murine PG model will enable researchers to probe common disease mechanisms and identify more effective targets for treatment for PG patients with IBD.

Pyoderma Gangrenosum Is Associated With Increased Risk of Inflammatory Pouch-Related Complications: A Retrospective Cohort Study.

Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis that is a well-established extraintestinal manifestation (EIM) of inflammatory bowel disease. The clinical implications of developing PG in patients with ulcerative colitis (UC) who undergo total proctocolectomy colectomy and ileal pouch anal anastomosis (TPC-IPAA) surgery remain unknown. Study participants were selected from patients enrolled in the Carlino Family Inflammatory Bowel and Colorectal Disease Biobank between 1998 and 2021 with a pre-colectomy diagnosis of UC and who underwent TPC-IPAA surgery. A retrospective study comparing patients with PG and those without PG was performed. The outcomes measured included the development of pouchitis, pouchitis classification, presence of pouch fistula, anal fistula, anal stenosis, and pouch failure. In this study, 357 IPAA patients were included, 10 of whom suffered PG. Patients with PG and without PG had similar demographics and clinical characteristics. Both groups had similar rates of pouchitis (80% in PG patients and 64% in patients without PG, P = .504). However, IPAA patients with PG had a higher risk of developing pouch fistula (50% vs 10%, P = .002), anal fistula (40% vs 12%, P = .031), and Crohn's-like disease of the pouch (70% vs 15%, P = .003) compared to patients without PG. Patients who developed PG prior to their first episode of pouchitis were more likely to eventually experience pouch failure (odds ratio: 20.7, 95% confidence interval: 3.9, 110.7, q = 0.003 after false discovery rate adjustment). Among UC patients who undergo TPC-IPAA surgery, the development of PG portends poor pouch outcomes and is predictive of pouch failure.

Human OTULIN mutations can cause distinct inflammatory disease phenotypes depending on the protein domain that is mutated

Abstract Linear ubiquitination is a post-translational modification that controls many immune signaling pathways. Linear ubiquitin chains are added to targets by LUBAC and removed by OTULIN, thus the opposing actions of LUBAC and OTULIN are critical for immune homeostasis. To understand mechanisms of how ubiquitination controls inflammation, we leveraged the use of biospecimens from patients with a novel bi-allelic OTULIN mutation. These patients have pyoderma gangrenosum (PG), an extremely rare inflammatory skin disease. OTULIN mutations have been previously described to cause a systemic autoinflammatory disease called ORAS, yet these patients define a new monogenic disease. We sought to understand this discrepancy through functional analyses of OTULIN mutations that culminate in PG vs ORAS. ORAS-causing OTULIN mutations impair the catalytic domain. However, the mutation in PG patients affects the domain responsible for binding LUBAC. Further, OTULIN’s ability to downregulate linear ubiquitin or NF-kB activity was unaffected. Patients’ transcriptional signatures showed elevated expression of genes involved in neutrophil activation and antigen presentation, and reduced expression of genes involved in Th17 differentiation, TLR signaling, and NOD-like receptor signaling. Deep immunophenotyping by CyTOF revealed that PG patients have higher frequencies of T cells and subtle defects of B cells and myeloid cells. These patients’ unique phenotype suggests an unrecognized function for OTULIN in skin inflammation. This discovery adds to the emerging spectrum of human immune-mediated diseases caused by defects in the ubiquitin pathway.

Successful management of recurrence of lung surgery-induced pyoderma gangrenosum after pacemaker implantation: a case report

BackgroundPyoderma gangrenosum begins as a painful erythema with induration, vesicles, or hemorrhagic pustules, which develops into serous ulcers. In addition to ulcerative colitis and myelodysplastic syndrome, surgical intervention may also induce this disease.Case presentationA 90-year-old man had previously undergone video-assisted thoracic surgery left upper lobectomy for left lung cancer. Blood tests on the 6th postoperative day showed elevated levels of white blood cells and C-reactive protein. The wound appeared red and drained pus; however, the wound culture was negative for bacteria. A skin biopsy was performed on the 13th postoperative day, and the patient was diagnosed with pyoderma gangrenosum. Tacrolimus hydrate ointment was administered, symptoms gradually improved around the 18th postoperative day, and the erythematous area shrank. The patient was discharged on the 50th postoperative day.Six months after lung surgery, a pacemaker was implanted in the left subclavian region. On the 6th postoperative day, the wound appeared reddish-brown and exudate was observed. On the 10th postoperative day, wound dehiscence was observed, and the pacemaker was removed. The patient was diagnosed with recurrent pyoderma gangrenosum and was re-treated with ointment. On the 29th postoperative day, a leadless pacemaker, which can be implanted with a small incision, was selected for treating arrhythmia. The patient was discharged 7 days after the second implantation.ConclusionWe report a recurrent pyoderma gangrenosum case following lung cancer surgery after pacemaker implantation as the second surgery, in which disease recurrence could be prevented by changing to a leadless pacemaker. Surgery and other invasive procedures should be avoided in pyoderma gangrenosum patients.

Different molecular pathways are disrupted in Pyoderma gangrenosum patients and are associated with the severity of the disease

Pyoderma gangrenosum (PG) is a rare inflammatory skin disease classified within the spectrum of neutrophilic dermatoses. The pathophysiology of PG is yet incompletely understood but a prominent role of genetics facilitating immune dysregulation has been proposed. This study investigated the potential contribution of disrupted molecular pathways in determining the susceptibility and clinical severity of PG. Variant Enrichment Analysis, a bioinformatic pipeline applicable for Whole Exome Sequencing data was performed in unrelated PG patients. Eleven patients were enrolled, including 5 with unilesional and 6 with multilesional PG. Fourteen pathways were exclusively enriched in the “multilesional” group, mainly related to immune system (i.e., type I interferon signaling pathway), cell metabolism and structural functions. In the “unilesional” group, nine pathways were found to be exclusively enriched, mostly related to cell signaling and cell metabolism. Genetically altered pathways involved in immune system biology and wound repair appear to be nodal pathogenic drivers in PG pathogenesis.

Intravenous immunoglobulin treatment for refractory pyoderma gangrenosum.

Intravenous immunoglobulins (IVIG) have been increasingly used for various inflammatory dermatoses with success. Small case series and case reports suggest a role for IVIG in the management of refractory pyoderma gangrenosum (PG). The objective was to study the characteristics of PG patients treated with IVIG and the efficacy and safety of IVIG for patients with refractory PG. An analysis was performed of all patients with PG treated with IVIG from 2012 to 2022 at an Australian tertiary hospital seeing a high volume of PG patients. We identified 12 patients, 9 females and 3 males, with median age of 61 years (29-77) at IVIG commencement. All patients were taking systemic corticosteroid therapy prior to IVIG treatment, and all had been treated with a steroid-sparing agent-including ten patients who had been treated with a biologic agent. IVIG was used with corticosteroids in one patient, concurrently with a steroid-sparing agent in nine patients and with a biologic agent in eight patients. Eleven patients demonstrated treatment response to IVIG-six with excellent response and five with good response. Three patients had complete healing of their most active ulcer. One patient did not respond to IVIG. Nine patients were able to wean their prednisolone dose and one patient was able to cease prednisolone. Four adverse events were recorded, and only one patient had to cease treatment due to aseptic meningitis and headaches. Our experience suggests that IVIG may be an efficacious treatment for patients with refractory PG due to its pleiotropic and immunomodulatory effects, particularly for patients with malignancy or other systemic conditions where high-dose immunosuppressive agents are contraindicated.

Successful treatment of pyoderma gangrenosum in a patient winh ulcerative colitis and COVID-19 infection: case report

Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis, which causes ulceration of the skin. The condition is commonly associated with underlying systemic disorders such as inflammatory bowel disease, arthritis, and hematological disorders. Herein, we describe the case of a familiar association between pyoderma gangrenosum and ulcerative colitis complicated by the newly COVID-19 infection. It is of particular clinical interest to address pyoderma gangrenosum lesions with concurrent treatment of associated disorders that may have influence on the course of the ulcer. Moreover, the therapy should cover as many diseases as possible while not being excessive to minimize possible side effects. Thus, the selection of treatment that spans mutual pathophysiological features of all comorbidities represents the best option to make when handling pyoderma gangrenosum patients. We present a case of the development of gangrenous pyoderma against the background of ulcerative colitis complicated by the new coronavirus infection COVID-19, which will raise awareness of gangrenous pyoderma as a relatively rare cause of ulcerative necrotic skin lesions, which can easily be confused with an infectious process. Of particular clinical importance in the conditions of the COVID-19 pandemic is the correct treatment of gangrenous pyoderma. Only an integrated approach can give a positive result from the therapy.

Pyoderma gangrenosum and dehydrated human amnion/chorion membrane: a potential tool for an orphan disease.

Pyoderma gangrenosum (PG) is an often-misdiagnosed, painful, inflammatory and ulcerative skin disorder. It is an orphan disease, where standard wound treatments such as sharp surgical debridement are contraindicated. This retrospective case series sought to evaluate the application of dehydrated human amnion/chorion membrane (DHACM) as a skin substitute in cases that were refractory to a range of standard-of-care techniques. This retrospective case series involved wounds which failed to close with standard escalating treatments, including anti-inflammatory and immunosuppressive therapies. Subjects were transitioned to DHACM and wound sizes were monitored until closure. Wounds (n=5) for all three subjects had stalled with standard therapies for at least 2.5 months but responded quickly to routinely applied DHACM treatments, and closure was achieved in each case. This retrospective pilot case series examined the use of DHACM as an alternative wound treatment for PG patients failing standard therapies. DHACM treatments re-initiated the trajectory towards wound closure for each stalled PG ulcer. The results suggest a treatment algorithm starting with early recognition, wound closure via treatment escalation, and lastly a gradual reduction in treatment for durable closure. DHACM treatment should be formally evaluated as an adjunct to PG ulcers that have remained refractory to more commonly used immunomodulating therapies.

Clinical characteristics and comorbidities of the most common atypical wounds in Northern Finland in 1996-2019: A retrospective registry study.

Background and AimsAtypical wounds comprise about 20% of all chronic wounds. Their diagnosis and treatment are challenging and require multidisciplinary cooperation. More knowledge is needed about the clinical characteristics and comorbidities of atypical wounds to enhance the treatment of these wounds.MethodsWe studied clinical characteristics and comorbidities of the atypical wounds by using the patient data retrieved from the Oulu University Hospital patient database from the year 1996 to the end of 2019 with the following International Classification of Diseases codes: L88, L95.0, L95.8, L95.9, and L98.1.ResultsIn our data, there were 135 patients with atypical wound, more commonly seen in females (N = 84, 62.2%) than in males (N = 51, 37.8%) (p < 0.05). The mean age of patients at the time of diagnosis was 57.3 years; those with pyoderma gangrenosum (PG) and factitious wounds were the youngest (53.1 and 53.0 years, respectively). The majority of subjects (N = 126, 93.3%) were diagnosed with comorbidity at the time of the diagnosis. The most common type of wound was PG (N = 49/135, 36.3%), followed by vasculitis and factitious wounds. The prevalence of inflammatory bowel diseases and rheumatoid arthritis in PG patients was high (18.4% for both). All patients with Martorell hypertensive ischemic leg ulceration/calciphylaxis had a diagnosis of hypertension and diabetes mellitus. Psychiatric diseases were more common in patients with factitious wounds than in other types of wounds, whereas patients with vasculitis wounds had more commonly hypertension, obesity, and diabetes mellitus; however, these did not reach statistical significance.ConclusionSpecific comorbidities associated with atypical wounds, such as high psychiatric comorbidity in factitious wounds. There is a female predominance in atypical wounds and patients are typically younger than patients with other types of wounds. Recognition of the typical clinical picture and comorbidities of atypical wounds may help in identifying these patients and thus also improving their treatment.