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Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

Objective: 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. Method: 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. Results: 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. Conclusion: 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.

A 75-year-old woman was admitted with sepsis and treated with broad-spectrum antibiotics until examination of her lower limbs noted necrotising wounds. Surgical intervention was advised by the plastic surgeons; however, she was deemed unsuitable for intensive care. She underwent incision and drainage of the necrotic area and biopsies were taken. She deteriorated clinically and the decision was made for best supportive care and was therefore transferred to the inpatient palliative care unit for end-of-life care. However, she stabilised, and based on culture sensitivities, antibiotics were restarted. It was also noted that the patient had a 3-month history of loose stools, which had not been addressed previously. The biopsies were suggestive of pyoderma gangrenosum, prompting a dermatology review, and prednisolone and doxycycline were started. The wounds and her loose stools improved, and with ongoing rehabilitation, she made a full recovery. Referral to gastroenterology was made.

There are no published cases about bullous pyoderma gangrenosum induced by leucovorin, fluorouracil and oxaliplatin (FOLFOX) chemotherapy. With the increasing incidence of gastric and colorectal cancers and the increased usage of targeted therapies, some cutaneous adverse effects may become common. An 84-year-old male presented to our clinic with multiple ulcerative plaques covered with hemorrhagic crusts on both extremities after several FOLFOX chemotherapy sessions for gastric cancer and liver metastasis. Two weeks later, multiple bullae also appeared, especially on the acral areas. The histopathology examination was compatible with acute leukocytoclastic vasculitis. The FOLFOX chemotherapy regimen is increasingly administered considering the rising incidence of gastrointestinal cancers. Hence, our understanding of its possible side effects and complications must be heightened.

Refereed/Peer-reviewed Background: Studies have found an increased risk of pyoderma gangrenosum associated with rituximab. The structural properties and pharmacological action of rituximab may affect the risk of pyoderma gangrenosum. Additionally, pyoderma gangrenosum is associated with autoimmune disorders for which rituximab is indicated. Objective: We aimed to determine whether rituximab is disproportionally associated with pyoderma gangrenosum using a systems biology-informed approach. Methods: Adverse event reports were extracted from the US Food and Drug Administration Adverse Event Reporting System (FAERS, 2013–20). The Bayesian Confidence Propagation Neural Network Information Component was used to test for disproportionality. Comparators used to determine potential causal pathways included all other medicines, all medicines with a similar structure (monoclonal antibodies), all medicines with the same pharmacological target (CD20 antagonists) and all medicines used for the same indication(s) as rituximab. Results: Thirty-two pyoderma gangrenosum cases were identified, 62.5% were female, with a median age of 48 years. There was an increased association of pyoderma gangrenosum with rituximab compared with all other medicines (exponentiated Information Component 6.75, 95% confidence interval (CI) 4.66–9.23). No association was observed when the comparator was either monoclonal antibodies or CD20 antagonists. Conditions for which an association of pyoderma gangrenosum with rituximab was observed were multiple sclerosis (6.68, 95% CI 1.63–15.15), rheumatoid arthritis (2.67, 95% CI 1.14–4.80) and non-Hodgkin’s lymphoma (2.94, 95% CI 1.80–3.73). Conclusions: Pyoderma gangrenosum was reported more frequently with rituximab compared with all other medicines. The varying results when restricting medicines for the same condition suggest the potential for confounding by indication. Post-market surveillance of biologic medicines in FAERS should consider a multi-faceted approach, particularly when the outcome of interest is associated with the underlying immune condition being treated by the medicine of interest.

To review the litigation literature related to patients with pyoderma gangrenosum (PG) to characterize the legal issues they face.Data sources include law databases Casetext and Lexis Nexis.All disability and medical liability claims directly involving patients with PG were included.Data extraction came directly from Casetext and Lexis Nexis. All cases extracted came from September 1965 to December 2020. This resulted in 32 cases, 18 of which were excluded because they did not mention the plaintiff having a diagnosis of PG (n = 14).The review found a total of six medical liability cases in which adverse health outcomes were reported, including prolonged suffering (2/6 cases), unnecessary treatments or procedures (2/6), exacerbation of disease (1/6 cases), and permanent scarring (1/6 cases). Despite this, two plaintiffs won their case and only one of them received any monetary award. Similarly, of the eight disability claims in the review, four were ruled in the favor of the plaintiffs and only two resulted in immediate awarding of benefits. Half of medical liability cases occurred in correctional facilities after the denial of appropriate care.The findings demonstrate a need for access to specialty care in incarcerated populations. Cases that occurred in a hospital setting also stress the importance of initiatives such as telemedicine to efficiently increase access to care in a cost-effective manner. Further, PG has been recognized as a severe impairment in disability claims despite patients being denied because they were deemed able to perform other work.

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) can be associated with various cutaneous manifestations. Several case reports have described skin ulceration resembling pyoderma gangrenosum (PG), particularly in granulomatosis with polyangiitis (GPA); however, the true incidence of this PG-like ulceration in various diseases is unknown. In addition, PG is frequently misdiagnosed, and diagnosis may rely on exclusion of other causes of ulcers. We aimed to describe clinical and histopathological features of PG-like ulcerations occurring in association with AAV and identify clues to differentiate these ulcers from PG. Retrospective search was conducted to include patients with AAV presenting with PG-like ulcers treated at our institution. This large case series highlights presentation of PG-like ulcers occurring in patients with AAV. Care should be taken to avoid delayed or missed diagnosis of AAV. Distinction between AAV and PG is challenging yet mandatory for proper treatment. Diagnosis relies on a constellation of detailed cutaneous clinical examination, systemic symptoms or illness, histopathological features and laboratory tests.

Diagnosing and treating neutrophilic dermatoses (NDs) in clinical practice can be challenging because of various presentations and stubborn treatment responses. Establishing a diagnosis is necessary, though, because many NDs are associated with underlying conditions, including malignancy. In this article, the authors provide information about Sweet syndrome, pyoderma gangrenosum, and other NDs and describe their clinical presentation, pathophysiology, diagnostic criteria, and associated conditions. The authors also present a case report describing the coexistence of two NDs and hidradenitis suppurativa in one patient and review the treatment modalities for those conditions.

We found that the systematic use of Delphi consensus diagnostic criteria resulted in substantial changes in management patterns in cases of suspected pyoderma gangrenosum (PG), including a reduction in systemic corticosteroid use, and significantly improved clinical outcomes. This improvement may have resulted from a combination of reduced misdiagnosis, optimized management and reduced iatrogenic harm. Increased utilization of validated diagnostic criteria for PG could optimize provider heuristics, increase diagnostic accuracy, and optimize management and clinical outcomes.

Juvenile gangrenous vasculitis of the scrotum (JGVS) is a rare entity with scant reports in the literature. The disease course, treatment, and prevalence have not been well described in the literature. It's hypothesized that JGVS is a variant of pyoderma gangrenosum or a male counterpart of Lipschütz ulcer. This review will analyze the current literature on JGVS and provide a current guide based on the best available data. The initial search of databases yielded 107 studies of which 14 pertained to the topic. The majority of the included studies were case reports (n = 9) reported in Spain. A total of 17 patients were included in the study. The mean age of patients was 22.45 years (range, 13-35 years). The majority of patients presented with multiple, acute, painful, well-circumscribed, round scrotal ulcerations. The majority of patients presented with flu-like symptoms. An increased level of awareness of JGVS diagnosis is now warranted among physicians. Despite the analogies with Lipschütz ulcer, we believe that JGVS is a distinct entity.

To summarize clinical outcomes of paradoxical pyoderma gangrenosum (PG) onset in patients on biologic therapy.The authors conducted MEDLINE and EMBASE searches using PRISMA guidelines to include 57 patients (23 reports).Of the included patients, 71.9% (n = 41/57) noted PG onset after initiating rituximab, 21.1% (n = 12/57) noted tumor necrosis factor α (TNF-α) inhibitors, 5.3% (n = 3/57) reported interleukin 17A inhibitors, and 1.8% (n = 1/57) reported cytotoxic T-lymphocyte-associated protein 4 antibodies. The majority of patients (94.3%) discontinued biologic use. The most common medications used to resolve rituximab-associated PG were intravenous immunoglobulins, oral corticosteroids, and antibiotics, with an average resolution time of 3.3 months. Complete resolution of PG in TNF-α-associated cases occurred within an average of 2.2 months after switching to another TNF-α inhibitor (n = 1), an interleukin 12/23 inhibitor (n = 2), or treatment with systemic corticosteroids and cyclosporine (n = 3), systemic corticosteroids alone (n = 1), or cyclosporine alone (n = 1).Further investigations are warranted to determine whether PG onset is associated with underlying comorbidities, the use of biologic agents, or a synergistic effect. Nevertheless, PG may develop in patients on rituximab or TNF-α inhibitors, suggesting the need to monitor and treat such adverse effects.

A 50-year-old man developed a sterile cavernosal abscess followed by prominent features of necrotizing neutrophilic dermatosis. We conducted a literature review, which revealed that aseptic abscesses in the corpus cavernosum occur in association with neutrophilic dermatosis. Patients with this condition frequently receive unnecessary antibiotic treatment and surgical interventions. Although this condition responds to systemic corticosteroids, the functional prognosis of the penis is poor. Abscess formation may be the initial presentation of neutrophilic dermatoses, and underlying conditions may even be absent. Clinicians need to be aware of this condition to distinguish it from bacterial infection and initiate early disease-specific treatments.

Pyoderma gangrenosum (PG) is a rare chronic skin disease characterised by painful skin ulcers. There are no treatment guidelines for PG, but systemic treatments including biologics are often used. Recently, adalimumab (ADA), a fully human monoclonal antibody against tumour necrosis factor, was approved for refractory PG treatment in Japan. Herein, we report a case of rheumatoid arthritis with refractory PG 2 months after orthopaedic surgery of the foot during treatment with low-dose etanercept and methotrexate. Although adding a moderate dose of glucocorticoid did not improve her PG, the patient showed a remarkable response after switching from etanercept to ADA in a higher dose than that used to treat rheumatoid arthritis. This higher dose of ADA may be effective for the treatment of refractory PG after the failure of other tumour necrosis factor inhibitors.

BACKGROUND AND AIMS: Extra-intestinal manifestations (EIMs) are a common complication of inflammatory bowel diseases (IBD), affecting up to half of the patients. Despite their high prevalence, information on standardised definitions, diagnostic strategies, and treatment targets is limited. METHODS: As a starting point for a national EIM study network, an interdisciplinary expert panel of 12 gastroenterologists, 4 rheumatologists, 3 ophthalmologists, 6 dermatologists, and 4 patient representatives was assembled. Modified Delphi consensus methodology was used. Fifty-four candidate items were derived from the literature review and expert opinion focusing on five major EIMs (erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis) were rated in three voting rounds. RESULTS: For use in a clinical practice setting and as part of the creation of a prospective registry of patients with EIMs, the panel developed definitions for erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis; identified the appropriate and optimal subspecialists to diagnose and manage each; provided methods to monitor disease course; offered guidance regarding monitoring intervals; and defined resolution and recurrence. CONCLUSIONS: Consensus criteria for appropriate and optimal means of diagnosing and monitoring five EIMs have been developed as a starting point to inform clinical practice and future trial design. Key findings include straightforward diagnostic criteria, guidance regarding who can appropriately and optimally diagnose each, and monitoring options that include patient and physician-reported outcomes. These findings will be used in a national multicenter study network to optimise the management of EIMs.

In this 52-week, phase 3 open-label study, efficacy and safety of adalimumab were evaluated in Japanese patients with active ulcers due to pyoderma gangrenosum (PG) during a 26-week treatment period and another 26-week extension period. Patients received adalimumab 160 mg at week 0, 80 mg at week 2, and 40 mg every week from week 4. At week 26, 12 of 22 patients (54.5%, p 0.001) achieved the primary efficacy endpoint of PG area reduction 100 (PGAR 100, complete skin re-epithelialization) for the target ulcer. Nine patients with Physician's Global Assessment (PGA) score of 1, 2, or 3, including four patients achieving PGAR 100, continued into the extension period. During the extension period, six of nine patients (66.7%) achieved PGAR 100 for the target PG ulcer at 52 weeks; one patient who achieved PGAR 100 before week 26 experienced a relapse 162 days after achieving this endpoint. Six patients achieved PGA 0 by week 52, and one patient reported new ulcers at day 57 of the extension period. Continued improvements from study baseline to week 52 were observed in pain (mean [95% CI] -4.0 [-6.5 to -1.5] numeric rating scale) and Dermatology Life Quality Index (-7.3 [-15.1 to 0.4]). In addition to the adverse events (AE) reported in 18 patients (including four serious AE) through week 26 (most commonly infections [n = 11]), there was one 1 additional AE (infection) during the extension period. These results suggest that adalimumab is effective and generally well tolerated in Japanese patients with active PG ulcers.

Leg ulcers in adults are a major public health concern. Their incidence increases with age and many causes have been identified, predominantly associated with vascular diseases. Leg ulcers in children and teenagers are less frequent. The aim of our study was to identify the causes of leg ulcers in children and teenagers, and to evaluate their management.This retrospective multicenter study was conducted by members of the Angio-dermatology Group of the French Society of Dermatology and of the French Society of Pediatric Dermatology. Data from children and teenagers (18 years), seen between 2008 and 2020 in 12 French hospitals for chronic leg ulcer (disease course4 weeks), were included.We included 27 patients, aged from 2.3 to 17.0 years. The most frequent causes of leg ulcer were: general diseases (n=9: pyoderma gangrenosum, dermatomyositis, interferonopathy, sickle cell disease, prolidase deficiency, scleroderma, Ehlers-Danlos syndrome), vasculopathies (n=8: hemangioma, capillary malformation, arteriovenous malformation), trauma (n=4: bedsores, pressure ulcers under plaster cast), infectious diseases (n=4: pyoderma, tuberculosis, Buruli ulcer) and neuropathies (n=2). Comorbidities (59.3%) and chronic treatments (18.5%) identified as risk factors for delayed healing were frequent. The average time to healing was 9.1 months.Leg ulcers are less frequent in children and teenagers than in adults and their causes differ from those in adults. Comorbidities associated with delayed healing must be identified and managed. Children and teenagers tend to heal faster than adults, but a multidisciplinary management approach is necessary.

Pyoderma gangrenosum is a painful recurrent ulcerative neutrophilic dermatosis in which the pathogenesis is incompletely defined. Current evidence suggests that PG is associated with dysregulation of components of both the innate and adaptive immune system with dysregulation of neutrophil function and contribution of the Th17 immune axis. PG can be present in numerous heterogeneous clinical presentations and be associated with multiple inflammatory conditions including rheumatoid arthritis, inflammatory bowel disease and hidradenitis suppurativa. However, no critical evaluation of the observed molecular characteristics in PG studies in association with their clinical findings has been assessed. Additionally, emerging evidence suggests a potential role for other cell types and immune pathways including B cells, macrophages, autoantibodies and the complement system in PG, although these have not yet been integrated into the pathogenesis of disease. This systematic review aims to critically evaluate the current molecular observations regarding the pathogenesis of PG and discuss associations with clinical characteristics as well as the evidence supporting novel cell types and immune pathways in PG.

We report a small case series of childhood-onset Takayasu arteritis (c-TA) presenting as pyoderma gangrenosum (PG)-like vasculitic ulceration. The cutaneous vasculitic ulcers in systemic vasculitis are rare and severe, sometimes leading to delayed diagnosis and treatment. We summarised the clinical features and highlighted the warning signs of c-TA associated with PG-like vasculitic ulceration.

Pyoderma gangrenosum (PG) is the prototypical neutrophilic dermatosis, commonly associated with inflammatory bowel disease, with pulmonary involvement being the commonest extracutaneous manifestation. PG with tracheobronchial involvement may present as upper airway obstruction and can be life-threatening.To evaluate the clinical characteristics and predictors of PG with pulmonary involvement, we reported a case of PG with tracheobronchial involvement in China, and performed a literature retrieval on PG with pulmonary involvement. Demographic data, clinical presentations, underlying diseases, radiological and histopathological findings, treatments, and clinical outcomes were collected and subjected to statistical analysis. Forty-seven cases (including ours) were identified. Diseases associated with PG with pulmonary involvement were similar. Clinical presentation of PG with pulmonary involvement was nonspecific, with cough and dyspnea being the most common clinical symptoms, and pulmonary infiltrates and cavitation being the most common radiological signs. Further univariate analysis suggested stridor and young age (PG with tracheobronchial involvement can be life-threatening, with young age and stridor being possible predictors. Therefore, prompt airway assessment and management are required in younger patients with PG with pulmonary involvement presenting with stridor.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

Atypical wounds account for approximately 5% to 20% of chronic ulcerations. Typically, clinical suspicion of an uncommon etiology is warranted for wounds that do not show signs of healing with conventional care, that are associated with pain out of proportion to the clinical presentation, or that are atypical in appearance. This review provides a general overview of various atypical wound etiologies, clinical presentations and appearance, and current treatment protocols. The clinical presentation, pathophysiologic etiology, and current literature on each etiology are presented. The etiologies discussed are pyoderma gangrenosum, calciphylaxis, lichen planus, necrobiosis lipoidica, infectious ulcers, hidradenitis suppurativa, artefactual ulcers, hydroxyurea-induced ulcers, vasculopathies, and neoplastic ulcers. Patients with atypical wounds experience a poorer prognosis and slower healing rate compared with patients with typical wound etiologies (eg, vascular and diabetic wounds). Biopsy is often vital in wound care to identify and differentiate wound etiologies. It is important to note that multiple characteristics or histologic features can overlap in a biopsy with atypical wounds. Therefore, a biopsy will still require an understanding of the presentation of these different wounds and should only be used when appropriate. The proper diagnosis for an atypical wound can greatly hasten wound closure, decrease the cost for the patient and the health care system, and improve the patient’s quality of life. Because of the limited availability of patient populations with atypical wound etiologies, literature concerning specific pathologies is limited. More research on each pathology is needed, as is a universally accepted treatment protocol for atypical wounds.

Introduction: Crohn’s disease (CD) is a chronic inflammatory disease of the gastrointestinal tract. The diversion through a colostomy or an ileostomy is sometimes required for disease control. In these patients, common stoma-related complications sum up with CD-related complications and often require revisional surgery. Methods: The aim of the study was to assess stoma morbidity after surgery for CD and to identify the burden of CD-related or CD-associated complications. Thus, details of past medical history, surgery, and follow-up of 54 consecutive patients operated on for CD with any sort of stoma were retrieved from the stoma therapist prospectively maintained database. Results: In our series, 23 patients had a colostomy, and 31 patients had an ileostomy. Complications occurred after stoma creation in 38 patients (70%) at a median of 1.3 months (interquartile range 0.6–7.2). CD-related complications arose in 8 patients (including pyoderma gangrenosum in 3 patients, peristomal fistulae in 2, granulomas in 2, and peristomal abscess in 1). Patients with CD-related complications tended to have a shorter disease duration (p = 0.07) and higher occurrence of CD-related complications was associated with end-stoma (p = 0.006). In this cohort, 11 cases had to be surgically treated for peristomal fistulae or abscess, parastomal hernia, prolapse, pyoderma gangrenosum, and recurrent CD. Discussion/Conclusions: In patients with CD, stoma creation is burdened by a high rate of postoperative complication and a relevant rate is specifically related to CD. Often these patients are required to be reoperated on to redo the stoma. Moreover, end-stoma configuration and aggressive CD phenotype are associated to a higher rate of complications.

The aim: To study the clinical case data for the feasibility of the obligatory inclusion of endoscopic methods of the gastrointestinal tract examination in patients with pyoderma gangrenosum of an unknown etiology. Clinical case: A patient under our supervision was with a not previously treated pyoderma gangrenosum of the shin skin. In the process of differential diagnostics by colonoscopic examination, nonspecific ulcerative colitis was diagnosed without clinical intestinal manifestation. A prescribed pathogenetic treatment of nonspecific ulcerative colitis led to the healing of the ulcer on the leg and induction of colitis remission. Thus, the first manifestation of asymptomatic colitis was pyoderma gangrenosum. Conclusions: Patients with pyoderma gangrenosum should be aware of the possibility of NUC, even in the absence of gastrointestinal symptoms, to get an early diagnosis and adequate treatment, to avoid disease manifestation and further complications. The inclusion of obligatory endoscopic examination of the gastrointestinal tract will increase the diagnosis of the etiology of severe skin lesions and increase the detection of asymptomatic nonspecific ulcerative colitis.

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The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors’ intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Pyoderma gangrenosum (PG) is a sterile inflammatory neutrophilic dermatosis that can present as a peristomal, pustular, bullous, vegetative, or ulcerative variant. It commonly affects the lower extremities, mainly in the pretibial area. We describe a case of extensive PG in an 18-year-old Indonesian man, involving the upper third of the body. Dermatological examination showed multiple painful ulcers with violaceous borders on the face, occipital region of scalp, neck, shoulder, upper chest, and back. The diagnosis of PG was established based on the histopathological examination that revealed massive dermal neutrophilic infiltration mixed with lymphocytic inflammatory infiltrates accompanied by leukocytoclastic vasculitis. The patient was treated with methylprednisolone equivalent to 1 mg/kg/day of prednisone. An excellent response to therapy also confirmed the diagnosis of PG. Since lesions of PG can appear on any part of the body, including the upper third of the body, a complete and appropriate examination is useful in establishing diagnosis.

Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and "real-world" practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.

Background: Pyoderma gangrenosum (PG) is a rare ulcerating skin disease associated with multiple comorbidities and increased mortality. In recent decades, newer biologics such as interleukin inhibitors have been used to treat PG; however, the literature is scarce, consisting predominantly of case reports and caseseries. The aim of our review was to evaluate the effectiveness and safety of interleukin inhibitors for the treatment of PG in adults. Summary: A literature search was conducted using search terms related to PG and interleukin inhibitors in databases such as PubMed, Embase, Scopus, Web of Science, and Cochrane Library. The study eligibility criteria included patients diagnosed with PG, over the age of 18, and treated with an interleukin inhibitor. Our study included 60 papers describing 81 patients fulfilling the eligibility criteria. The treatment with interleukin inhibitors resulted in 70% (95% CI 59–80%) response and 57% (95% CI 45–68%) complete response rates, and few (4%) mild adverse events, hence supporting the off-label use for the treatment of recalcitrant PG in adults. The response and complete response rates were 59% (17/29) and 38% (11/29) for anakinra, 64% (7/11) and 55% (6/11) for canakinumab, and 79% (27/34) and 71% (24/34) for ustekinumab, respectively. Limitations include publication bias that might have overestimated the efficacy as successful cases responding to treatment are more likely to be reported than nonresponding cases. Additionally, the heterogeneity of the treatment groups does not allow conclusions of superiority or inferiority of the different interleukin inhibitors to be drawn. Further studies are needed to investigate the efficacy of the different interleukin inhibitors and to investigate the importance of underlying disease for treatment response.

Pyoderma gangrenosum (PG), which most frequently affects the lower extremity, is a complicated disease state that results from a combination of inflammation, neutrophilic invasion, and genetic predisposition. There may also be certain comorbidities involved or it may be idiopathic. The many variations of PG mean that it often presents and responds differently to various treatments based on the specific case. Overall, there have been improvements in understanding the disease; however, further research should focus on finding better ways to predict and prevent this rapidly progressive, painful disease.

Pyoderma gangrenosum is a neutrophilic dermatosis, which mimics both infection and necrotizing fasciitis, that can present after surgical interventions. We present the case of a 62-year-old male who underwent one-stage bilateral total knee arthroplasty. Nine days after the surgery, he presented with wound breakdown, high fever, and elevated white blood cell count. Repeated debridement was performed, and empiric antibiotics were given. All tissue cultures and aspirates remained negative throughout treatment course, and the patient remained unresponsive to therapy. The patient was eventually diagnosed with pyoderma gangrenosum after infectious etiologies were ruled out and after a skin biopsy and dermatologic consultation. His condition rapidly improved after treatment with corticosteroids, and soft-tissue defects were repaired with skin substitute and full-thickness skin grafting. In patients with aseptic wound breakdown after total knee arthroplasty, pyoderma gangrenosum is a rare but devastating complication and should be considered.

Inflammatory bowel diseases (IBDs) are systemic diseases that manifest not only in the gut and gastrointestinal tract, but also in the extraintestinal organs in many patients. The quality of life for patients with IBD can be substantially affected by these extraintestinal manifestations (EIMs). It is important to have knowledge of the prevalence, pathophysiology, and clinical presentation of EIMs in order to adapt therapeutic options to cover all aspects of IBD. EIMs can occur in up to 24% of patients with IBD before the onset of intestinal symptoms, and need to be recognized to initiate appropriate diagnostic procedures. EIMs most frequently affect joints, skin, or eyes, but can also affect other organs, such as the liver, lung, and pancreas. It is a frequent misconception that a successful therapy of the intestinal inflammation will be sufficient to treat EIMs satisfactorily in most patients with IBD. In general, peripheral arthritis, oral aphthous ulcers, episcleritis, or erythema nodosum can be associated with active intestinal inflammation and can improve on standard treatment of the intestinal inflammation. However, anterior uveitis, ankylosing spondylitis, and primary sclerosing cholangitis usually occur independent of disease flares. This review provides a comprehensive overview of epidemiology, pathophysiology, clinical presentation, and treatment of EIMs in IBD.

Objective — to highlight the issue of difficulties in the diagnosis and treatment of patients with pyoderma gangrenosum (PG). Materials and methods. A literature review and analysis of the results of the clinical examination of patients with PG was performed. Pyoderma gangrenosum is most commonly observed in young and middle-aged people, more frequently in women. The pathogenesis of PG has not been fully studied. It is believed to be related to genetic mutations, neutrophil dysfunction and impaired immunity/inflammation regulation. Maverakis and others developed diagnostic criteria for PG. First-line treatment is aimed at optimizing the local wound care. For more severe diseases, systemic therapy is required. Oral corticosteroids (0.5—1 mg/kg/day) are the basis of treatment and used for control of PG. Cyclosporine can be used either alone or in combination with corticosteroids. At present, there is an increasing evidence of effectiveness of biological therapy as treatment method for a number of cytokines. Results and discussion. This article presents a clinical case of a patient diagnosed with PG. Based on recent guidelines, systemic corticosteroid therapy (Medrol starting at 48 mg/d), systemic anti-inflammatory and vascular drugs, topical therapy and treatment results were presented. Conclusions. Without treatment, the disease lasts for months and years. Ulcers sometimes grow very fast, reaching huge sizes in a matter of days. After the healing of some ulcers new ones often emerge. Pain syndrome, the addition of a secondary infection and the continuous progression of the pathological process lead to a decrease in the patient’s quality of life, as well as to a deterioration in his social adaptation. Therefore, timely adequate diagnosis, treatment and full compliance of the patient are the key to success.

Pyoderma gangrenosum is a rare skin condition which is difficult to diagnose as it often presents similarly to common complications such as wound infection, tissue ischemia and necrotizing fasciitis. We present a patient who underwent immediate DIEP free flap breast reconstruction and developed post-surgical pyoderma gangrenosum (PSPG), initially with non-characteristic signs and symptoms. Our patient first developed severe headache, fever and erythema surrounding her wound. The classical signs of painful ulcers with undermined bluish borders and surrounding violaceous rash were not seen for nearly 4 weeks. This, in combination with positive wound culture, made for a challenging diagnosis in an elective breast reconstruction patient. Diagnosis of PSPG relies on clinical signs first and is supported by wound swab culture and sensitivity and tissue biopsy for histopathology. The treatment of choice for PSPG is high-dose systemic steroids followed by an oral prednisolone taper over a period of 4–6 weeks. Early diagnosis of PSPG can optimize outcomes and reduce morbidity. We should consider PSPG as a differential diagnosis when post-operative wound infection is suspected, particularly if a non-classical pattern is seen. We could also consider discussion of PSPG as a rare, yet serious complication during the consent process. Level of evidence: Level V, diagnostic study.

Introduction: Spider bites are common worldwide. Frequently symptoms resolve without any adverse outcome, but in rare cases the bite can cause severe morbidity. The most typical presentation of Mediterranean recluse spider (Loxosceles Rufescens) bite is a dermatonecrotic lesion of the skin (skin loxoscelism). When the only manifestation of a spider bite is an ulcerated skin lesion, clinical suspicion and differential diagnosis strongly depend on its site. We present the case of an ulcerated wound of the breast, diagnosed as a Mediterranean recluse spider bite. Case presentation: A 79-year-old woman presented a 10cm-wide soft tissue ulceration of her left breast. At first, the diagnostic hypothesis of an ulcerated cancer was ruled out. Two family members revealed a recent history of Mediterranean recluse spider bite and the same clinical diagnosis was made for our patient. A wide excision was performed, with complete resolution of symptoms. Discussion: No specific diagnostic criteria for spider bites are available. Diagnosis is usually clinical. Skin loxoscelism could be easily mistaken for cellulitis, various types of skin infections, cutaneous anthrax, vasculitis, scorpion sting, pyoderma gangrenosum, erythema migrans of Lyme disease or prurigo nodularis. A thorough anamnestic interview is fundamental to raise the diagnostic hypothesis. When possible, a biopsy is recommended and it is extremely important when the ulcer can mimic a cancer, as is the case in breast tissue. Conclusion: We recommend a wide excision of the wound after failure of conservative treatment, in order to obtain local control and to perform histological examination on a more representative specimen.

Negative-pressure wound therapy has been shown to be effective in re-epithelialisation in recalcitrant pyoderma gangrenosum. This case documents a patient whose co-morbidities limited the standard treatment options and required an alternative management plan. The STOP GAP randomised control trial found that both oral prednisolone and ciclosporin were equally effective in the treatment of pyoderma gangrenosum. However, in this case, the patient had type 2 diabetes and prednisolone resulted in persistently elevated blood glucose levels. Lower doses were ineffective and it was subsequently stopped once other treatments showed a clinical improvement. A recent diagnosis of breast cancer prevented the use of ciclosporin and other immunosuppressive treatments. After multidisciplinary discussion, involving breast surgeons, oncology, dermatology and tissue viability, doxycycline was commenced alongside a portable negative-pressure device with twice weekly tissue viability input. The introduction of this device resulted in a rapid reduction in the wound size and facilitated healing with an excellent outcome.

A woman in her 30s was diagnosed with ulcerative colitis (UC) 4 years ago and treated with tacrolimus, azathioprine, and prednisolone 5mg (PSL). Skin ulcers appeared on the right lower leg during the course of treatment, diagnosed as pyoderma gangrenosum (PG). The patient initially improved with an increased PSL and infliximab dose, but then developed multiple skin ulcers and folliculitis throughout her body. She was transferred to our hospital for PG exacerbation treatment. She developed fever after transfer and contrast-enhanced computed tomography showed multiple abscesses in the lungs and kidneys. PSL was decreased and infliximab was discontinued. Antibiotic therapy and granulocyte/monocyte apheresis (GMA) were started. Fever persisted even after antibiotic treatment, and her general condition did not improve. A right renal abscess puncture was performed. Pus was sterile. A sterile abscess associated with PG was suspected. The PSL dose was increased to 1mg/kg and infliximab restarted. Thereafter, the patient's general condition improved, and both lung and renal abscesses contracted. Skin ulcer epithelialization was also observed. Abdominal symptoms were mild during the course of the disease, and colonoscopy showed only a localized ulcerative lesion in the rectum. The patient was later transferred to the department of dermatology at our hospital for PG treatment. Aseptic abscesses are caused by neutrophil infiltration without infection and have been reported to be associated with neutrophilic dermatosis and inflammatory bowel disease. UC-associated aseptic abscess is rare. This is only the sixth case in Japan. Aseptic abscesses can occur in various sites, including subcutaneous and deep organs, but this is the first kidney abscess case. In previous reports, PSL, infliximab, colchicine, and infliximab+GMA were used for aseptic abscesses associated with UC. They all showed abscess reduction. Aseptic abscesses associated with PG should be considered if abscess lesions occur during the course of UC, and a treatment strategy including enhanced immunosuppression should be considered.

Pyoderma gangrenosum is a rare neutrophilic inflammatory skin disorder commonly seen over lower limbs. Involvement of penile area is rare. We report this rare case of occurrence of ulcerative type of pyoderma gangrenosum over penis with pustular type elsewhere over the body, healing with keloids in an immunocompetent young man with no systemic associations.

Interleukin (IL)-18 is a pleiotropic, pro-inflammatory cytokine involved in the regulation of innate and adaptive immune responses. IL-18 has attracted increasing attention as a key mediator in autoinflammatory diseases associated with the development of macrophage activation syndrome (MAS) including systemic juvenile idiopathic arthritis and adult-onset Still’s disease. In these diseases, dysregulation of inflammasome activity and overproduction of IL-18 might be associated with the development of MAS by inducing natural killer cell dysfunction. Serum IL-18 levels are high in patients with these diseases and therefore are useful for the diagnosis and monitoring of disease activity. In contrast, a recent study revealed the overproduction of IL-18 was present in cases of autoinflammation without susceptibility to MAS such as pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. The pathogenic and causative roles of IL-18 remain unclear in these autoinflammatory diseases. Further investigations are necessary to clarify the role of IL-18 and its importance as a therapeutic target in the pathogenesis of autoinflammatory diseases.

Pyoderma gangrenosum (PG) is a rare ulcerative skin disease; its etiology is unknown, though it is often associated with autoimmune diseases. Pyoderma gangrenosum results in significant morbidity and exquisite pain that affects health-related quality of life. Wound healing is delayed, and patients often experience relapse. Pyoderma gangrenosum is susceptible to pathergy and deterioration with surgical intervention or other trauma; therefore, treatment includes atraumatic wound care, infection management, and local or systemic immunosuppression.We describe the use of modified negative pressure wound therapy (NPWT) with intralesional and topical steroids for the treatment of PG in a 15-year-old female patient with ulcerative colitis and a staged J-pouch ileoanal reconstruction. The patient and her family refused all systemic therapy due to prior steroid-associated weight gain. She was unable to tolerate conscious dressing changes, further complicating the treatment plan. Procedural interventions such as NPWT have been used previously for PG; however, they can cause wound pathergy and subsequent wound deterioration. Modified NPWT in conjunction with topical and intralesional steroids induced wound healing without producing pathergy.Timely recognition of PG is crucial to appropriate delivery of care. Modified NPWT and localized corticosteroid treatment were key to promoting wound healing in this case of pediatric PG.

Background and Aims: Atypical 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. Methods: We 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. Results: In 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

A dog was referred because of the presence of painful ulcers with violaceous borders and multiple dermal and subcutaneous haemorrhagic nodules on the bridge of the nose, on the dorsal aspect of the front paws, and on all four legs. Lesions had not responded to antibacterial and immunomodulatory therapy. Nine months earlier, the dog had been diagnosed with idiopathic epilepsy and treated with potassium bromide ever since. Histopathological examination of lesions revealed an interstitial neutrophilic dermatitis multifocally extending to the subcutaneous tissue. All special stains were negative for infectious agents, and due to the lack of tropism for follicular structures as well as negative bacterial and fungal cultures, a diagnosis of a sterile neutrophilic process similar to pyoderma gangrenosum was made. A cutaneous drug reaction was suspected, potassium bromide was suspended, and after 6 weeks the ulcerative lesions were completely healed. The present report describes a case of an ulcerative neutrophilic dermatitis presumed to be associated with administration of potassium bromide that resembled human bromoderma.