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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.

The role of wound debridement in pyoderma gangrenosum (PG) is controversial, largely due to concerns regarding pathergy. This study sought to evaluate the clinical outcomes and utility of wound debridement in PG management. We conducted a retrospective cohort study of 104 patients diagnosed with PG at a single tertiary referral centre, stratified into two treatment groups: those receiving debridement in conjunction with immunosuppressive therapy (n = 38) and those treated with immunosuppression alone (control group, n = 66). The primary outcomes measured were remission (absence of active PG lesions without necessitating additional treatment), time to remission and disease progression (new lesions or expansion of existing ones). Remission was achieved by 60.53% (n = 23) in the debridement group versus 87.88% (n = 58) in the control group (p = 0.003). The mean time to remission was 12.3 months for the debridement group versus 8.67 months for the control group (p = 0.2). Multivariate Cox regression analysis indicated that debridement significantly decreased the likelihood of disease remission (adjusted hazards ratio [HR]: 0.45, 95% confidence interval [CI]: 0.26–0.78, p = 0.005). Disease progression was significantly higher in the debridement group (68.42%, n = 26) compared to the control group (15.15%, n = 10) (p < 0.001). Additionally, 28.95% (n = 11) of patients in the debridement group required repeated procedures, and 10.53% (n = 4) underwent amputations due to deteriorating conditions. The timing and duration of immunosuppressive therapy relative to the procedure did not mitigate the risk of post‐surgical exacerbations. These findings suggest that debridement is associated with poorer healing outcomes in PG, advocating for its contraindication in the management of this condition. [ABSTRACT FROM AUTHOR]

Background and Objective: Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that can be associated with primary immunodeficiency. The pathogenesis of PG has not yet been elucidated, although contributions from dysregulation of the immune system in patients with apparent genetic predispositions have been postulated. We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-guided systematic review with the objective of identifying inborn errors of immunity in the presence of PG as well as their clinical characteristics of severity including number of PG lesions and anatomic areas affected, and treatment outcomes. Methods: A literature search was performed using PubMed/MEDLINE, Embase, Cochrane Library, and Web of Science through August 24, 2023, for studies published in English using the search terms: "pyoderma gangrenosum," "inborn error of immunity," "immune defect*," and a list of genetic mutations potentially associated with PG. Results: Seventy-four cases of PG associated with inborn errors of immunity were identified. The results demonstrate an association of PG with a variety of inborn errors of immunity, including genetic mutations not classically associated with the condition. Genetic mutations such as BTK, IL1RN, ITGB2, LPIN2, MEFV, NFkB1, NLRP3, NLRP12, NOD2, PSMB8, PLCG2, PSTPIP1, RAG1, TTC37, and WDR1, as well as complement component 2/complement component 4 (C2/C4) and complement component 7 (C7) deficiencies were identified in the presence of either idiopathic or syndromic PG. Of note, mutations in genes such as PSMB8, NLRP3, and IL1RN were found to be associated with a more severe and atypical course of PG, whereas mutations in RAG1 as well as those causing a C2/C4 deficiency were associated with the mildest clinical presentations of PG. Mutations in NFkB1, ITGB2, and PSTPIP1 were associated with the most heterogeneous clinical presentations. Conclusions: Human inborn errors of immunity may be implicated in the genetic predisposition to PG and may influence the clinical presentation. Due to the rarity of these diseases, further work must be done to describe the association between inborn errors of immunity and PG. Identifying inborn errors of immunity that may contribute to the development of PG may assist in further elucidating the mechanism of PG, guiding targeted treatment, and improving clinical outcomes for these patients. [ABSTRACT FROM AUTHOR]

Background: Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The management of PASH syndrome is difficult, with no consensus on treatment guidelines. Since PASH syndrome can increase morbidity and adversely impact quality of life, better characterization of effective therapies is needed. Methods: A retrospective cohort study was conducted to identify all patients with pyoderma gangrenosum (PG) treated at The Ohio State University Wexner Medical Center between 2015 and 2021. PG diagnosis was confirmed via PARACELSUS score. Subsequent chart review identified eight patients with concomitant hidradenitis suppurativa (HS) and acne who were clinically diagnosed with PASH syndrome. Results: Eight patients were clinically diagnosed with PASH syndrome based on their clinical presentation at our institution. Seven patients had failed some type of medical therapy prior to presentation, including topical corticosteroids, oral corticosteroids, oral antibiotics, and biologics. One patient had also tried surgical drainage at an outside institution. Six patients were effectively treated with biologics, usually in combination with other therapies. One patient experienced improvement of her skin lesions after diagnosis and treatment of her underlying hematologic malignancy. Conclusions: Medical management with biologics in combination with corticosteroids and/or antibiotics was effective in the management of most patients. Diagnosis and treatment of an underlying condition should be prioritized in refractory cases. If workup is negative, surgical management may be considered. Further investigation with a greater number of patients is required to develop management guidelines for PASH syndrome. [ABSTRACT FROM AUTHOR]

Background: 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. Methods: 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 University from 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

This systematic review explores the potential connection between Familial Mediterranean fever (FMF) and hidradenitis suppurativa (HS), two autoinflammatory disorders. The study reveals that mutations in the MEFV gene, associated with FMF, were also found in HS patients. The prevalence of FMF in HS patients ranged from 0.7% to 4.2%, with a higher risk compared to the general population. HS patients with FMF or MEFV mutations exhibited specific clinical characteristics and some experienced positive effects from IL-1β inhibitors. However, the study's small sample size and observational nature limit its findings, emphasizing the need for further research in this area. [Extracted from the article]

This article discusses the relationship between pyoderma gangrenosum (PG) and immune-mediated rheumatic diseases (IMRD). PG is a rare skin disorder associated with immune system dysfunction, often occurring alongside conditions like inflammatory bowel disease and arthritis. The study found that patients with IMRD were more likely to develop PG, especially after being diagnosed with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. The findings suggest a potential connection between these conditions and PG. The study emphasizes the importance of recognizing PG in patients with IMRD, particularly rheumatoid arthritis, as early detection and treatment can lead to better outcomes. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) is a rare inflammatory dermatologic condition with neutrophilic infiltration of the skin that causes pustules and ulcerations. Janus kinase (JAK) inhibitors are immunomodulating agents that have been recently described in the literature as an effective treatment for PG. We describe a patient with PG on the lower extremities successfully treated with baricitinib. We also conducted a narrative review of the literature of PG patients treated with JAK inhibitors who were refractory to other treatments. [ABSTRACT FROM AUTHOR]

Sweet syndrome is an acute febrile neutrophilic dermatosis characterized by the infiltration of neutrophils into the skin. It may occur idiopathically or be linked to malignancies, inflammatory or autoimmune diseases. Leukocyte adhesion deficiency type I (LAD-I) is an inborn error immunity wherein leukocytes lack adhesion molecules necessary for migration to infection sites due to mutations in the CD18 gene encoding β2 integrins. We present a case of a 16-month-old female initially diagnosed and treated for Sweet syndrome based on histopathological findings with recurrent flare episodes. Subsequent workup revealed LAD-I, making this case the first documented association between Sweet syndrome and LAD-I. Moreover, we reviewed the pertinent literatures detailing the concurrence of neutrophilic dermatosis and immunodeficiency disorders. This case underscores the significance of comprehensive evaluation for Sweet syndrome patients who are refractory to conventional treatments. [ABSTRACT FROM AUTHOR]

PSTPIP1‐associated myeloid‐related proteinemia inflammatory (PAMI) syndrome is a rare autoinflammatory disorder often arising in pediatric patients. We present a case of an 18‐year‐old female with a past medical history of growth failure, immunoglobulin A nephropathy, and inflammatory arthritis who presented to a pediatric dermatology clinic with findings of acne, psoriasiform dermatitis, and hidradenitis suppurativa, whose clinical, genetic, and laboratory findings were most consistent with PAMI syndrome. We conducted a literature review to better characterize this rare condition in the context of dermatologic findings. Recognition of the distinctive skin findings seen in PAMI syndrome can help distinguish it from other inflammatory disorders, enabling expedited diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Introduction: Reactive angioendotheliomatosis (RAE) is a rare, benign, angioproliferative disorder with poorly understood aetiopathogenesis. It is characterised by vascular occlusion that occurs in patients with coexistent systemic or autoimmune disease. Case Presentation: A 60-year-old female presented with an 8-week history of a painful, non-healing, and non-traumatic ulcer on the left thigh. Her past medical history included smoking, peripheral vascular disease (PVD) and previously treated rectal squamous cell carcinoma. The diagnosis of pyoderma gangrenosum with superimposed cellulitis was considered and treatment with oral antibiotics was initiated. Following failure to improve, a biopsy was undertaken leading to the diagnosis of RAE. The patient was referred for urgent consideration of surgical correction of PVD, but was deemed unsuitable for surgical treatment due to a poor performance status. The patient was treated with conservative measures, but her condition rapidly deteriorated and she passed away a few weeks later. Conclusion: RAE is notorious for mimicking a wide spectrum of diseases. It is an important differential diagnosis to consider in patients with non-healing ulceration and underlying systemic or autoimmune disorders. Our case raises awareness of this rare condition and the mortality that it carries if left untreated. In an attempt to reverse disease progression and mortality, we urge clinicians to attempt surgical correction of PVD even when faced with multiple comorbidities and poor performance status. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum is a severe and uncommon neutrophilic dermatosis associated with certain rheumatologic, inflammatory, or malignant pathologies, but it can also occur in previously healthy individuals as a complication of surgery or trauma. More recently, an entity known as post-breast-surgery pyoderma gangrenosum has been described in the literature, which exhibits characteristic clinical features and rapid progression. A prompt diagnosis and early management are critical for optimizing treatment outcomes, with corticosteroids being the first-line treatment choice. Below, we present a clinical case illustrating this disease, including its diagnosis, progression, and management. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Epidermolysis bullosa acquisita (EBA) is a rare dermatosis of the mucous membrane and/or skin. Employing biologic treatment modalities, specifically rituximab (RTX), have become pivotal measure in treating patients with blistering diseases. This study aims to summarize the current evidence on the safety and efficacy of RTX in EBA. METHODS: An extensive search was performed in MEDLINE/PubMed, Embase, Scopus, and Web of Science databases until the end of August 19th, 2023. Two independent reviewers screened the papers, and collected data. Two hundred thirty-three studies were screened using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. RESULTS: Thirty-one studies were enrolled. The most common reason of RTX administration in patients with EBA was recalcitrant diseases. Clinical response and disease remission was recorded as 92.7 percent (63 patients) and 73.8 percent (45 patients) of the patients, respectively. A relapse rate of 39.5 percent (15 patients) in the mean follow-up of 23.0 months was reported in the studies. Of the patients, 28.2 percent (11 patients) experienced RTX-related side events, mostly mild and transient infusion reactions. CONCLUSION: The results of this systematic review demonstrated that RTX is safe and effective in patients with EBA. This biological treatment modality can be routinely used in managing EBA. [ABSTRACT FROM AUTHOR]

Atopic dermatitis (AD), a chronic inflammatory skin disease with T cell activation as a key feature, in which Th2 cell–mediated responses play a pivotal role. Regulatory T cells (Treg) are central immune cells that restrict autoimmunity and inflammation in the body. Patients with immune dysregulation, polyendocrinopathy, or enteropathy X-linked syndrome, an immune disease characterized by a deficiency in Treg, develop skin inflammation and allergic disorders, indicating that Treg play a crucial role in the development of allergic skin inflammation. we investigated the underlying mechanisms by which Treg control cutaneous allergic inflammation. An allergic skin inflammation mouse model was constructed using MC903, and Treg-depleted mouse model was constructed using diphtheria toxin. Neutralization of IFN-γ was constructed using anti-mouse-IFN-γ mouse antibody. Neutrophil infiltration was analyzed by flow cytometry and immunohistochemistry. Neutrophil extracellular traps (NETs), a process called NETosis, were detected using immunofluorescence. In vitro neutrophil stimulation and immunocytochemistry was conducted to demonstrate the effect of IFN-γ on NETosis. The depletion of Foxp3+ Treg led to significantly exacerbated AD-like skin inflammation, including increased recruitment of neutrophils and expression of Th1 cytokine IFN-γ. Neutrophil infiltrating in skin of Treg-depleted mice released more NETs than wild type. Neutralization of IFN-γ abolished neutrophil infiltration and NETosis in Treg-depleted mice. Neutrophils stimulated with IFN-γ were more prone to release NETs in vitro. Finally, Foxp3+ Treg control cutaneous allergic inflammation by regulating IFN-γ-driven neutrophilic infiltration and NETosis. Our results highlight the previously underestimated Treg-IFN-γ-neutrophil inflammatory axis. [Display omitted] ● Depletion of Treg exacerbates MC903-induced allergic skin inflammation. ● Neutrophil infiltration and NETosis were detected in Treg-depleted mice during allergic skin inflammation. ● Neutralization of IFN-γ abolished aggravated inflammation in Treg-depleted mice. ● IFN-γ is sufficient to induce NETosis in vitro. [ABSTRACT FROM AUTHOR]

Monoclonal Gammopathy of Undetermined Significance (MGUS) is a clonal plasma cell disorder that is considered preneoplastic, asymptomatic, and only requiring observation. However, MGUS may result in cutaneous complications, which are poorly understood, causing treatment delays and patient suffering. We present 30 patients with cutaneous findings associated with MGUS, characterizing clinical presentations, isoforms, treatments, and outcomes. These included: MGUS-associated 'rashes' (pruritic eczematous rashes), reactive and mucin-depositional conditions (pyoderma gangrenosum, scleromyxedema), M-protein-related deposition disorders (POEMS syndrome, Waldenstrom macroglobulinemia), and cutaneous lymphomas. Twelve of 30 (40%) patients received multiple myeloma drugs (MMDs). Eleven (92%) patients improved, and those not receiving MMDs rarely improved, suggesting that MMDs have efficacy for cutaneous manifestations of MGUS. Therefore, trialing MMDs may be warranted for patients with MGUS not responding to other therapies. Moreover, evaluation for monoclonal gammopathy in elderly patients with intractable pruritus or other chronic skin conditions that are non-responsive to skin-directed therapies should be considered. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Infections in infantile hemangiomas (IHs) are generally limited, and only few cases have been reported. The rapid expansion of an ulcerated IH should raise concern for possible complications to monitor and provide immediate therapeutic interventions. This case highlights the importance of prompt treatment in large segmental IHs to prevent ulceration and related complications, including bleeding and superinfection. IH is a benign tumor proliferating during early infancy. While many IHs spontaneously resolve, complications like ulceration, bleeding, and potential damage to vital organs can occur, leading to pain, infection, and scarring. A 6‐month‐old girl with a previously treated IH on her left leg developed a Klebsiella‐infected ulcer at the site. The ulcer resulted from non‐standard treatments used before admission. Upon hospitalization, she was initially treated with cefepime and propranolol, but a week later, the wound culture revealed Klebsiella pneumoniae, prompting a switch to piperacillin/tazobactam. After successfully managing the infection and bleeding, the child was discharged in good condition with orders to continue treatment with propranolol for at least a year. This case highlights the potential of IHs to become infected even with uncommon germs such as Klebsiella and the importance of receiving appropriate medical care to prevent further complications. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Inflammatory bowel disease (IBD) refers to a collection of chronic, idiopathic inflammatory/autoimmune disorders of the gastrointestinal tract characterized by relapsing and remitting episodes. In this case report, we will report a patient who has encountered ulcerative colitis related to mononeuritis multiplex as a rare clinical scenario. A 75‐year‐old male patient, with a prior medical history including long‐standing hypertension, recurring episodes of peripheral joint arthritis, leg skin lesions reminiscent of erythema nodosum, and persistent chronic diarrhea over the past 2 years, was recently hospitalized at the rheumatology department of Imam Reza Hospital in Tabriz. Throughout the patient's hospital stay, a series of diagnostic assessments were conducted, encompassing procedures such as colonoscopy, electromyography and nerve conduction studies, echocardiography, renal ultrasonography, and standard hematological analyses. The patient underwent the following treatment regimen, which resulted in a significant improvement in his condition. [ABSTRACT FROM AUTHOR]

Abstract Neutrophilic dermatoses are autoinflammatory disorders characterised by sterile cutaneous inflammatory neutrophilic infiltrates. This group of diseases is rare in infants and children, where sepsis is the diagnosis of exclusion. This report focuses on the clinical presentation, investigation and management of infants with idiopathic pyoderma gangrenosum. It should allow for a better appreciation of the disease and a more patient‐focused approach to its diagnosis and management.

Abstract Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with certain neoplasms, predominantly of haematological origin, and autoimmune diseases. Pemphigus vulgaris (PV) is a disease in which antibodies target crucial intercellular junction components. A few cases of PG associated with PV have been described. We present a woman with a high phototype presenting this rare association and highlight the diagnostic challenge of diagnosing rare diseases in patients with high phenotypes.

Abstract Pyoderma gangrenosum (PG) is a rare reactive noninfectious inflammatory dermatosis. More than 50% of PG patients have an associated systemic disease, which, in most cases, is an inflammatory bowel disease. PG affects more females than males and patients with active colitis. This case report describes a 12‐year‐old girl previously diagnosed with ulcerative colitis (UC) and on infliximab treatment. She came to our attention with a painful leg ulcer during admission for acute respiratory failure and fever. The lesion was classified as PG and a diagnosis of UC pulmonary involvement was made. Infliximab was substitute by ustekinumab and the girl is in remission after 1 year of treatment. To the best of our knowledge, this is the first case in the literature of a UC‐related paediatric PG treated with ustekinumab. Our data suggest that more studies are required as to the efficacy of ustekinumab in paediatric PG settings.

Treatment of pyoderma gangrenosum (PG) is challenging due to the absence of standardized guidelines and the lack of evidence-based, effective treatment options. Here, we performed a systematic review to summarize the use of biologics and their efficacy in the treatment of PG. We searched PubMed/MEDLINE, EMBASE, and Cochrane electronic databases from their inception to September 22nd, 2022, and included 82 peer-reviewed studies with a total of 108 patients. Infliximab, adalimumab, and etanercept were the most utilized biologic therapies in the treatment of PG in 64.8% (70/108), 16.7% (18/108), and 11.1% (12/108) of the cases, respectively. With respect to treatment response, 88.9% (96/108) of the patients achieved complete resolution of PG with biologic therapies. The average number of days to improvement and resolution of PG treated after starting biologic therapies was 30 and 161, respectively. PG recurred in 15.5% (11/71) of those reported the outcome. Our study suggests that biologic therapies may be an attractive therapeutic option for PG with an excellent efficacy. [ABSTRACT FROM AUTHOR]

History of Present Illness: The patient is a 57-year-old woman who presented to the emergency department with increasing cough and shortness of breath over several days. She has a history of ulcerative colitis complicated by toxic megacolon with subsequent colectomy. Past Medical History, Family History and Social History • Ulcerative colitis with history of toxic megacolon (4 years prior), s/p total colectomy • History of recent respiratory failure thought secondary to ustekinumab (Stelara). The respiratory failure responded well to steroid therapy. • She has a history of latent Tb treated with rifampin • Anxiety Medications • Clonazepam 1.0 mg daily at bedtime • Gabapentin 300 mg TID • Pantoprazole 40 mg BID • Prednisone 5 mg daily Physical Examination • Mild-moderate respiratory distress • Afebrile. SpO2 87% on room air. Oxygen saturation 94% on 2 lpm supplemental oxygen. • Chest: crackles noted at left base • Cardiovascular: regular rhythm, no murmur • Extremities: scarring and erythema on both ankles consistent with resolving pyoderma …

We report a case of a 54-year-old female diagnosed with HIV and antiretroviral therapy (ART) for the same. Seven years ago, she suffered from fever, cough and weight loss, was diagnosed with pulmonary tuberculosis and also seropositive for HIV. She suffered from Herpes Zoster infection, after which her ART regimen was changed to TLD (tenofovir, lamivudine and dolutegravir). The patient presented with two episodes of pyoderma gangrenosum (PG), which were biopsy-proven, corresponding to a rise in CD4 counts above 500. She responded to glucocorticoids, both systemic and topical.

Key Clinical Message: Administering azathioprine or infliximab for UC and AS treatment carries a significant risk of adverse reactions. Here, we present the case diagnosed with UC and AS, who received treatment with azathioprine and infliximab for 10 months, and subsequently developed drug‐induced myopathy affecting the right vastus medialis muscle. Drug‐induced myopathy is an uncommon form of muscle injury that can arise in patients without preexisting muscle conditions when exposed to therapeutic doses of certain medications. Administering azathioprine or infliximab for ulcerative colitis (UC) and ankylosing spondylitis (AS) treatment carries a significant risk of adverse reactions, including drug‐induced myopathy and increased susceptibility to opportunistic infections. However, occurrences of myopathy induced by the combination of azathioprine and infliximab are rarely reported in clinical practice. Here, we present the case of a 37‐year‐old male patient diagnosed with UC and AS, who received treatment with azathioprine and infliximab for 10 months. Despite the resolution of symptoms and improvement in intestinal mucosal inflammation observed via endoscopy, the patient subsequently developed drug‐induced myopathy affecting the right vastus medialis muscle. [ABSTRACT FROM AUTHOR]

Background: GPP is a rare, chronic, neutrophilic skin disease, with limited real‐world data characterizing patients with flares and the impact of flares on disease progression and morbidity. Objective: Describe the clinical characteristics of patients with GPP, comorbidities, disease epidemiology and frequency and severity of flares, and compare patients with GPP with a matched severe psoriasis population. Methods: In this population‐based real‐world cohort study an algorithm was developed to identify patients with GPP flares. Three cohorts were identified using the Système National des Données de Santé (SNDS) database covering almost the entire French population; a prevalent cohort (2010–2018), an incident cohort (2012–2015). A severe psoriasis cohort was compared with the GPP incident cohort using propensity score matching. Results: The prevalent and incident cohorts comprised 4195 and 1842 patients, respectively. In both cohorts, mean age was 58 years; 53% were male. Comorbidities were significantly more common in the incident cohort versus matched psoriasis cohort, respectively, including hypertension (44% vs. 26%), ischaemic heart disease (26% vs. 18%) and hyperlipidaemia (25% vs. 15%). In the incident cohort, the flare rate was 0.1 flares/person‐year and 0.4 flares/person‐year among the 569 out of 1842 patients hospitalized with flares. These patients had a mean (±SD) stay of 11.6 ± 10.4 days; 25% were admitted to the intensive care unit. In 2017, the cumulative incidence and cumulative GPP age–sex standardized prevalence were 7.1 and 45.2 per million, respectively. Conclusions: Patients with GPP had a distinct comorbidity profile compared to patients with severe psoriasis, and GPP flares were associated with long hospitalizations. [ABSTRACT FROM AUTHOR]

Background: Ustekinumab is an interleukin 12/23 inhibitor, approved by the US-FDA for the management of moderate-to-severe plaque psoriasis and psoriatic arthropathy. Purpose: This review aims to describe the dermatological implications and applications of ustekinumab. Methodology: PubMed and Google Scholar were searched for scholarly articles related to ustekinumab and its utility in dermatology using the search terms "Ustekinumab" AND "Psoriasis" AND "dermatological diseases". Studies utilising Ustekinumab in psoriasis and other dermatological indications were analysed and formulated into a systematic review discussing the utility of the drug in psoriasis, as well as other dermatological conditions. Results: Ustekinumab is a valuable biologic agent for the management of psoriasis and psoriatic arthropathy, as well as other dermatological disorders like hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris, Behcet's disease, lupus erythematosus, alopecia areata and pyoderma gangrenosum. Conclusion: Ustekinumab's usage is not limited to psoriasis. Its benefit extends to many more dermatological conditions. It is considered to be the biologic of choice in childhood psoriasis and adult psoriatic patients with concurrent Crohn's disease. Besides, it has an acceptable safety profile. [ABSTRACT FROM AUTHOR]

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow‐up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post‐surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied. [ABSTRACT FROM AUTHOR]

This letter discusses a bibliometric analysis of research on pyoderma gangrenosum, a cutaneous condition, from 1930 to 2023. The study aimed to identify research trends, primary contributing countries, and prevalent topics in the field. The analysis found that there has been a steady increase in publications on pyoderma gangrenosum over the years, with a focus on treatment challenges, diagnosis obstacles, and connections to underlying diseases. The findings have important implications for clinical practice and can guide future research efforts to address knowledge gaps and improve patient outcomes. [Extracted from the article]

The article discusses the understanding of the skin condition pyoderma gangrenosum (PG) and its association with multi-kinase inhibitors (MKIs). PG is a chronic ulcerative skin condition characterized by dysregulated immune responses. The lack of validated animal models has hindered the understanding of PG's etiology, but drug-induced PG can provide insights into the molecular actions of causative medications. The article reports a case of MKI-associated PG-like ulcers and discusses the spectrum of cutaneous side effects of MKIs. The study suggests that clinicians should consider PG or PG-like ulcers as a differential diagnosis in MKI-treated patients and highlights the need for further research to understand the molecular mechanisms of PG. [Extracted from the article]

Background: Hidradenitis suppurativa (HS) is a debilitating cutaneous disease characterized by severe painful inflammatory nodules/abscesses. At present, data regarding the epidemiology and pathophysiology of this disease are limited. Objective: To define the prevalence and comorbidity associations of HS. Methods: This was a cross-sectional study of EPICTM Cosmos© examining over 180 million US patients. Prevalences were calculated by demographic and odds ratios (OR) and identified comorbidity correlations. Results: All examined metabolism-related, psychological, and autoimmune/autoinflammatory (AI) diseases correlated with HS. The strongest associations were with pyoderma gangrenosum [OR 26.56; confidence interval (CI): 24.98–28.23], Down syndrome (OR 11.31; CI 10.93–11.70), and polycystic ovarian syndrome (OR 11.24; CI 11.09–11.38). Novel AI associations were found between HS and lupus (OR 6.60; CI 6.26–6.94) and multiple sclerosis (MS; OR 2.38; CI 2.29–2.48). Cutaneous malignancies were largely not associated in the unsegmented cohort; however, among Black patients, novel associations with melanoma (OR 2.39; CI 1.86–3.08) and basal cell carcinoma (OR 2.69; CI 2.15–3.36) were identified. Limitations: International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy. Conclusion: This is the first study to identify correlations between HS with melanoma and basal cell carcinoma (BCC) among Black patients as well as MS and lupus in all patients with HS. [ABSTRACT FROM AUTHOR]

Subcorneal pustular dermatosis (SPD) is a rare neutrophilic dermatosis characterized by pustules on the trunk and intertriginous areas. While oral dapsone is the first-line treatment for SPD, alternative options are necessary for patients with glucose-6-phosphate dehydrogenase deficiency, drug hypersensitivity reactions, or refractory disease. To date, no consensus exists regarding next-best agents for SPD. In this report, we present a patient with significant SPD who developed dapsone hypersensitivity syndrome and then was successfully treated with colchicine and adalimumab. We propose that colchicine should be considered as a second-line treatment for SPD and present a therapeutic algorithm for clinicians to utilize when patients are not candidates for dapsone, have side effects requiring drug discontinuation, or have refractory disease. [ABSTRACT FROM AUTHOR]

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder typically characterized by the clinical triad including a sudden onset of fever, painful skin lesions, and neutrophilia. The histopathological findings are a dense neutrophilic infiltrate and oedema of the dermis and epidermis without evidence of a vasculitis. Besides treatment of the underlying cause, sweet syndrome is typically treated with high‐dose corticosteroids leading to a relapse‐free response in 70% of patients. However, if left unrecognized or untreated, the condition may lead to serious complications. Here, we report on the case of a 38‐year‐old patient in whom, under the assumption of the presence of necrotizing fasciitis, exarticulation of the right arm was performed. In the absence of pathogen detection and insufficient response to anti‐infective therapies, the diagnosis of a sweet syndrome was assumed and, later, confirmed by an excellent response to high‐dose administration of systematic glucocorticoids. The case emphasizes the need to be aware of this rare syndrome, which can be easily misdiagnosed due to its close resemblance to infection and stresses the need of further research to define distinct diagnostic tools. [ABSTRACT FROM AUTHOR]

A 54-year-old woman presented to our emergency department two months after the removal by Video-Assisted Thoracoscopic Surgery (VATS) of her middle lung lobe affected by severe inflammation and fibrosis. She was experiencing pain from a large ulcer (diameter 13 cm) starting from the surgical scar. She suffered from Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) syndrome, and hypothyroidism. The laboratory results showed White Blood Cell count (WBC) 13,600/mm3 (N 76%), C-Reactive Protein (CRP) 12 mg/L (nv

Introduction: Guselkumab is an interleukin 23p19 inhibitor, and the first in this group, to be approved by the US Food and Drug Administration (FDA) for the management of moderate to severe psoriasis. Apart from its utility in psoriasis, there are a number of other dermatologic conditions where guselkumab has demonstrated value. Objectives: The aim of this narrative review is to describe the utility of guselkumab in psoriasis as well as its implication in off-label dermatologic disorders. Methods: Pubmed, Google Scholar, Scopus and ResearchGate were searched for scholarly articles related to guselkumab and its utility in dermatology using the search terms “Guselkumab” AND “Psoriasis” AND “other dermatological disorders”. Results: Guselkumab is a valuable biologic agent for the management of psoriasis and psoriatic arthropathy. It has also been used successfully for other dermatologic disorders like hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris and pyoderma gangrenosum. Recently, its utility in Stewart-Treves angiosarcoma (STA) has been exemplified. Conclusion: Guselkumab’s usage is not limited to psoriasis. Its benefit extends to many more dermatologic conditions. Its utility in STA could open an avenue for its application in the field of oncology. Furthermore, it has an acceptable safety profile.

BackgroundHematologic malignancies (HMs) are well-known underlying comorbidities of pyoderma gangrenosum (PG). However, studies quantifying the likelihood of PG after HMs are yet to be performed.ObjectiveTo investigate the bidirectional association between PG and several HMs, namely acute leukemia, chronic leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma, and multiple myeloma.MethodsA population-based retrospective cohort study was conducted to study the risk of HMs in patients with PG (n = 302) as compared to age-, sex-and ethnicity-matched control subjects (n = 1,799). A case–control design was used to estimate the likelihood of PG in individuals with a preexisting history of HMs. Adjusted hazard ratios (HRs) and adjusted odds ratios (ORs) were estimated by Cox regression and logistic regression, respectively.ResultsThe prevalence of preexisting HM was higher in patients with PG than in controls (6.7% vs. 0.9%, respectively). The likelihood of having PG was significantly greater among patients with a history of HM (adjusted OR, 7.88; 95% CI, 3.85–16.15; p

Facial neutrophilic dermatosis is a relatively recent and rare entity. Herein, we report an original case with a clinical appearance of crusty pyoderma gangrenosum and limited face involvement, classifying it as neutrophilic dermatosis (ND) of the face. The latter is an infrequent entity and forms part of the ND spectrum. The few reported cases in the literature are characterized by a restricted distribution on the face, as in our case. Nevertheless, this entity remains controversial: some consider it a variant of Sweet's syndrome; others see it as a truly independent entity. Matthews et al. reported an association between ND of the face, as in the case of our patient with a crusty appearance, and ulcerative colitis. Our observation and that of Matthews et al. underline the wide and varied potential for the clinical presentation of this entity. [ABSTRACT FROM AUTHOR]

This document is a scientific study or review on the use of intravenous immunoglobulin (IVIg) as a treatment for refractory pyoderma gangrenosum (PG). The authors conducted a systematic review and meta-analysis of studies on patients with PG treated with IVIg. They found that IVIg was effective and safe, with over half of the patients experiencing complete or near-complete resolution of PG. However, the authors note that the data may be impacted by publication bias and that further research is needed. They also highlight the need for caution in interpreting the results, as most patients received concurrent steroid treatment. [Extracted from the article]