Pyodermatitis vegetans confined to the umbilicus: Report of 2 cases.

Pyodermatitis pyostomatitis vegetans is a rare variant of neutrophilic disorders, affecting the intertriginous areas such as axilla and groin, umbilicus, as well as the oral mucosa. We herein describe 2 female patients, in both of whom the umbilicus was restrictedly involved.

Postsurgical pyoderma gangrenosum after mastectomy with a familial component.

Postsurgical pyoderma gangrenosum (PSPG) is a rare, ulcerative skin condition that presents a diagnostic challenge due to its similar presentation to infectious etiologies in the postsurgical period-often leading to gratuitous and unnecessary surgery and antibiotic use. We report a 37-year-old female with breast cancer who received neoadjuvant chemotherapy and immunotherapy and underwent bilateral skin-sparing mastectomies who developed delayed bilateral mastectomy skin flap necrosis secondary to PSPG. This case had rare factors associated with the development of PSPG such as preoperative systemic therapy and a familial component. This case underscores the importance of early recognition of this rare disease and appropriate management of PSPG to prevent unnecessary interventions and ensure an optimal outcome.

Staphylococcus felis C4 exhibits in vitro antimicrobial activity against methicillin-resistant Staphylococcus pseudintermedius in a novel canine skin explant model.

BACKGROUND: Canine superficial pyoderma is a common bacterial skin infection of dogs, generally caused by Staphylococcus pseudintermedius. The C4 strain of Staphylococcus felis was recently discovered to have strong antimicrobial activity against S. pseudintermedius in mice. OBJECTIVES: We aimed to evaluate in vitro if this antimicrobial activity was maintained using a novel canine skin explant model. MATERIALS AND METHODS: Punch biopsies (8 mm) of skin from recently euthanised dogs were collected and placed into six-well plates on top of an agarose pedestal. RESULTS: Histological examination of the skin explants showed an intact dermal-epidermal organisation and a stratum corneum that was successfully colonised by S. pseudintermedius after topical application. The number of colony forming units of S. pseudintermedius showed a 2 log increase after 24 h colonisation, indicating that the explant supported bacterial growth. By contrast, co-treatment with S. felis C4 live bacteria and its sterile protein product significantly reduced the growth of a methicillin-susceptible (ST540, p = 0.0357) and a methicillin-resistant (MR) strain (ST71, p = 0.0143) of S. pseudintermedius. No detectable bacteria were recovered from or visualised on skin 24 h posttreatment with the S. felis C4 sterile protein product. CONCLUSIONS AND CLINICAL RELEVANCE: Using a novel canine explant model, we demonstrate that the S. felis C4 strain inhibits the growth of S. pseudintermedius and that it is a promising candidate for a new probiotic therapy to treat cutaneous infections caused by S. pseudintermedius, including MR strains.

Pyoderma Gangrenosum in an Ulcerative Colitis Patient on Vedolizumab.

Pyoderma gangrenosum is a rare neutrophilic dermatosis that presents as a tender, rapidly progressive ulcer with violaceous, undermined borders. Pathophysiology is multifactorial and has been suggested to involve neutrophil dysfunction, increased T-cell activation, and inflammatory mediator release, often in settings that are known to have autoimmune or genetic diseases. Some medications that modify the immune response have been described to trigger pyoderma gangrenosum. Vedolizumab is a monoclonal antibody used in the management of inflammatory bowel disease that has been shown to be effective in the treatment of pyoderma gangrenosum but, in some cases, has been shown to paradoxically induce pyoderma gangrenosum. Vedolizumab causes gut-selective inhibition of lymphocyte migration, which may lead to the activation of lymphocytes in other organ systems, such as the skin. In this report, we present a case of pyoderma gangrenosum in a patient treated with vedolizumab for ulcerative colitis and explore the possible mechanism behind vedolizumab-induced pyoderma gangrenosum.

Successful Treatment of Severe Pyoderma Gangrenosum and Ulcerative Colitis With Upadacitinib.

Pyoderma gangrenosum (PG) is a rare inflammatory and ulcerative skin disorder that is often associated with an underlying systemic disease, including inflammatory bowel disease. Many treatments used to treat inflammatory bowel disease are also used for the treatment of PG, including systemic therapies, immunomodulators, and tumor necrosis factor-α inhibitors; however, their efficacy in PG is limited. Novel treatments of PG are needed. We report the case of a 62-year-old woman with steroid-refractory PG and concomitant ulcerative colitis successfully treated for both conditions with upadacitinib, a selective Janus kinase inhibitor.

Clinicopathological Profile of Pyoderma Gangrenosum: A 10-Year Retrospective Study from a Tertiary Care Center in South India.

Background: Pyoderma gangrenosum (PG) is a reactive neutrophilic inflammatory dermatosis with a varied clinicopathologic presentation. It commonly manifests as rapidly progressive painful ulcers, mimicking varied conditions including infections, vasculitis, and malignancies, and is a diagnosis of exclusion. There are scarce data on PG from the Indian subcontinent. Aim and Objectives: The aim of the study was to study the clinicopathologic profile of patients with PG and their underlying systemic associations. Materials and Methods: A retrospective observational study was done between 2011 and 2021, and patients diagnosed as PG based on the diagnostic tool proposed by Maverakis et al. were recruited and their demographic, clinical, and histological findings were obtained. Results: Among 54 patients with suspected PG, 17 patients (eight males and nine females) fulfilled the diagnostic criteria, and the mean age of disease onset was 32.1 years (range: 3-60 years). Ulcerative variant was the most common type (9/17, 52.9%), and 29.4% had systemic associations including autoinflammatory syndromes. The onset at atypical sites such as face and hand were noted in one patient each. Histopathology revealed a polymorphous dermal infiltrate with neutrophilic predominance in the majority (94.1%). Systemic steroids (dose ranging from 0.5-1 mg/kg prednisolone equivalent) were used in 11/17 (64.7%) patients. The commonly used alternative drugs included clofazimine (47%), minocycline (29%), thalidomide (23.5%), adalimumab and mycophenolate mofetil in 17.6% each, dapsone and ciclosporine in 11.7% each. Remission was achieved between two weeks and three months in 10 (58.8%) patients after treatment initiation and two mortalities (11.7%) were recorded. Conclusion: PG can affect any age group and may be localized to rarer, atypical sites. The possibility of underlying autoinflammatory conditions should be considered in addition to the evaluation of other disorders like inflammatory bowel disease, hematological disorders, and rheumatological disorders.

A complex case of PASH syndrome: pyoderma gangrenosum, acne, suppurative hidradenitis, and Crohn's disease in a 36-year-old smoker.

Introduction and importance: PASH syndrome, is autoinflammatory condition driven by immune system dysfunction, resulting in elevated interleukin 1 levels and subsequent production of proinflammatory cytokines and chemokines. The clinical progression of PASH typically starts with acne conglobate in adolescence, followed by hidradenitis suppurativa, and pyoderma gangrenosum. Diagnosis relies on recognizing these hallmark features, but treatment remains a challenge despite current understanding. Conventional immunosuppressive therapies have shown limited efficacy in managing PASH syndrome. Case presentation: The authors present a 36-year-old man with a complex combination of pyoderma gangrenosum, acne, suppurative hidradenitis, obesity, and Crohn's disease. The patient's symptoms began in adolescence with acne and recurrent furuncles, evolving into painful skin ulcers and fistulas over time. Histological examination confirmed the diagnosis of pyoderma gangrenosum. Despite various treatment modalities, including isotretinoin, cyclosporine, azathioprine, and adalimumab, the patient experienced only partial improvement until receiving Infliximab, which led to remarkable improvement. Discussion: PASH syndrome, a rare neutrophilic dermatosis linked to autoinflammatory conditions like Braun Flaco, is characterized by Pyoderma gangrenosum, acne, and suppurative hidradenitis. This clinical entity presents diagnostic challenges due to its unique features and association with obesity and bowel diseases, such as Crohn's disease. Treatment options, including TNF-α blockers like Infliximab, have shown promising results in controlling cutaneous manifestations. Our case study underscores the complexity of treating PASH syndrome and highlights the importance of personalized therapeutic approaches for optimal outcomes. Conclusion: PASH syndrome presents significant diagnostic and treatment challenges due to its complex symptomatology and associations with conditions like Crohn's disease. The case of a 36-year-old man demonstrates the partial efficacy of conventional therapies and highlights the promising results of infliximab. This underscores the need for personalized treatment strategies and ongoing research to improve outcomes for patients with this rare and intricate syndrome.

Multiple Pyoderma Gangrenosum Overlying AV Fistula Treated With Colchicine: A Case Report.

Rationale: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis which gives rise to painful ulcers. Pyoderma gangrenosum can be triggered by trauma, a phenomenon called pathergy. Here, we report the first case of PG arising from pathergy due to needle insertion overlying an arteriovenous fistula (AVF). This case report seeks to inform nephrologists about PG, this yet unreported presentation, and management in the context of hemodialysis. Presenting concerns: A 69-year-old woman presented to dermatology clinic for erythemato-violaceous plaques with central ulceration at the site of needle insertion overlying her AVF. The patient was known for chronic renal insufficiency secondary to C3 glomerulonephritis, for which she received hemodialysis. After an accidental burn which lead to appearance of a painful ulcer, following each needle insertion for hemodialysis, she would develop an erythematous papule that progressed to a painful ulcer with erythematous-violaceous borders. Diagnosis: Pyoderma gangrenosum was clinically diagnosed and both clinical and paraclinical evaluation did not reveal any secondary cause of PG. Intervention: Dialysis via AVF was suspended due to the risk of triggering more PG and was temporarily pursued by central venous catheter. The patient was initially treated with prednisone and topical corticosteroids. Furthermore, owing to the high recurrence rate of PG, colchicine was initiated in prevention to avoid resorting to immunosuppressive or long-term corticotherapy. Outcomes: The patient's lesions improved on prednisone, which was then tapered over 1 month. Following prednisone taper and continuing improvement of PG on colchicine and topical corticosteroids alone, the decision was taken to recommence dialysis via AVF after performing a negative pathergy test. Topical corticosteroids were ceased due to the risk of cutaneous atrophy and were replaced by pimecrolimus ointment. The patient has continued dialysis via AVF ever since, without recurrence. Novel Finding: This is the first case reported of PG arising from pathergy due to needle insertion overlying an AVF. Colchicine may be a safe and effective therapy for long-term treatment of PG in the context of hemodialysis.

Contexte: Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare qui provoque des ulcères douloureux. Le PG peut être déclenché par un traumatisme, un phénomène appelé pathergie. Nous rapportons ici le premier cas de PG résultant d'une pathergie au site d'insertion de l'aiguille recouvrant une fistule artérioveineuse. Ce rapport de cas vise à informer les néphrologues sur le PG, sur cette présentation non encore rapportée et sur sa prise en charge dans le contexte de l'hémodialyse. Présentation du cas: Une femme âgée de 69 ans s'étant présentée à une clinique de dermatologie pour soigner des plaques érythémateuses violacées avec ulcération centrale au site d'insertion de l'aiguille recouvrant sa fistule artérioveineuse (FAV). La patiente était connue pour une insuffisance rénale chronique secondaire à une glomérulonéphrite à C3, pour laquelle elle recevait des traitements d'hémodialyse. À la suite d'une brûlure accidentelle ayant entraîné l'apparition d'un ulcère douloureux, la patiente développait une papule érythémateuse qui évoluait vers un ulcère douloureux aux bords érythémateux violacés après chaque insertion d'aiguille pour l'hémodialyse. Diagnostic: Le Pyoderma gangrenosum a été diagnostiqué cliniquement et l'évaluation, tant clinique que paraclinique, n'a révélé aucune cause secondaire de PG. Intervention: En raison du risque accru de déclenchement de PG, la dialyse par la FAV a été suspendue et remplacée temporairement par la dialyse par cathéter veineux central. La patiente a d'abord reçu un traitement de prednisone et de corticostéroïdes topiques. En raison du taux élevé de récidive de PG, un traitement préventif à la colchicine a été initié pour éviter le recours à une corticothérapie immunosuppressive ou à long terme. Résultats: Les lésions de la patiente se sont améliorées avec le traitement à la prednisone, celui-ci a ensuite été réduit progressivement sur une période d'un mois. Après la réduction progressive de la prednisone et en raison de l'amélioration continue du PG avec le traitement par colchicine et corticostéroïdes topiques seulement, la décision a été prise de recommencer la dialyse par la FAV après un test de pathergie négatif. Un risque d'atrophie cutanée a justifié l'arrêt des corticostéroïdes topiques qui ont été remplacés par une pommade de pimecrolimus. Depuis, la patiente a poursuivi sa dialyse par FAV, sans récidive. Principales observations: Il s'agit du premier cas signalé de PG résultant d'une pathergie au site d'insertion de l'aiguille recouvrant une fistule artérioveineuse. La colchicine peut s'avérer un traitement sûr et efficace pour le traitement à long terme du PG dans le contexte de l'hémodialyse.

Hospital admissions for pyoderma gangrenosum in Spain (1999-2021): Epidemiological and clinical characteristics, temporal trends, and factors associated with poor prognosis and higher cost.

Background and Aims: Pyoderma gangrenosum (PG) is a neutrophilic inflammatory dermatosis that can be idiopathic or associated with other diseases. The aim was to analyze the epidemiological and clinical characteristics, temporal trends, risk factors for poor prognosis, and admission costs associated with PG in Spain. Methods: We conducted a retrospective study, based on the Hospital Discharge Registry of the Spanish National Health System in Spain from 1999 to 2021. Results: Of 82,161,670 admissions during the study period, 4901 were for PG (hospitalization rate of 59.7/1,000,000 admissions). PG hospitalizations increased from 28.8/1,00,000 in 1999 to 91.9/1,000,000 in 2021. PG was a primary cause of admission in 60.5% of cases, and 58.4% of patients were women. The main PG-related comorbidities were inflammatory bowel disease (15.7%) and neoplasms (10%). There was a significant increase over the years in admissions for inflammatory bowel disease, monoclonal gammopathy of undetermined significance, and lymphoma, as well as an increase in diseases unrelated to PG, such as hypertension, diabetes, and chronic kidney disease. The hospital mortality rate was 5.6%. Death was associated with PG being a primary diagnosis, older age, leukemia, neoplasms, diabetes, and chronic kidney disease. The cost of treatment increased over the years and was higher in older people. Conclusion: PG cases in the inpatient setting in Spain over the past 23 years make up a tiny proportion of all hospital admissions, although the rate of hospitalization for PG has increased in the last two decades.

Pyoderma Gangrenosum Associated With Iatrogenic Interleukin 17A Blockade: A Report of Two Cases and a Review of the Literature.

Pyoderma gangrenosum (PG) is a rare necrotizing neutrophilic dermatosis driven by monokines and cytokines elaborated by monocytes and autoreactive T cells, respectively. Th1-mediated autoimmune disorders and myeloproliferative disease are among the potential disease associations. More recently, certain medications were implicated, including TNF-alpha inhibitors, rituximab, and IL-17A inhibitors, such as secukinumab, where the development of PG is held to represent a cutaneous immune adverse effect. We present two patients who developed an autoinflammatory syndrome resembling PG in the setting of drug therapy with agents exhibiting an IL-17A inhibitory effect. The drugs were erunumab in one and secukinumab in the other. One patient received the anti-calcitonin gene-related peptide targeted therapy, erenumab, for migraine prophylaxis. While this drug has not been previously implicated in the development of PG, it can cause IL-17A blockade. The other patient was on secukinumab, a monoclonal antibody that selectively targets IL-17A. We documented a microenvironment enriched in IL-17A, emphasizing that the blockade impacts the functionality of the receptor as opposed to a quantitative reduction in IL-17A production by T cells. Qualitative functional IL-17A blockade could result in a paradoxical increase in IL-23, a pro-inflammatory cytokine that may contribute to the influx of neutrophils pathogenetically implicated in PG.

Recurrent Vegetative Pyoderma Gangrenosum of the Face: A Case Report.

A 46-year-old male presented with an erythematous papule progressing into a vegetative plaque on the right cheek that resolved with cribriform scarring. Eight months after, a similar looking erythematous papule appeared on his left cheek. This papule rapidly progressed into a vegetative plaque within a week, and was associated with a pain score of 7 out of 10. Histopathology of the second lesion revealed suppurative dermatitis with diffuse dense infiltrates composed mostly of neutrophils. Cultures revealed few colonies of Enterobacter cloacae which was inconclusive. Pathergy test was negative. High dose systemic corticosteroids were started, with an observed rapid reduction of pain, inflammation, and ultimately resolution of the lesion with formation of cribriform scarring, confirming a case of vegetative pyoderma gangrenosum. It is important to note that not all inflamed and purulent lesions are infectious-neutrophilic dermatoses should always be considered.

Combination Doxycycline and Topical Corticosteroids in the Treatment of Ulcerative Pyoderma Gangrenosum: A Case Report.

Pyoderma gangrenosum (PG) is a chronic inflammatory neutrophilic dermatosis often presenting as a solitary enlarging painful ulcer with red to violaceous undermined borders. This report delves into the role of doxycycline in the treatment of PG in a 44-year-old male COVID-19 positive patient who has concomitant active tuberculosis infection and end-stage kidney disease, for which both first-line treatments (systemic corticosteroid and cyclosporine) are contraindicated. After three months on doxycycline and topical corticosteroids, there was resolution of the ulcers and no note of recurrence up to three months from completion of the treatment regimen.

Decoding the Histopathology of Pustular Pyoderma Gangrenosum: A Multidisciplinary Approach to Diagnosis.

This article discusses the rare neutrophilic dermatosis pustular pyoderma gangrenosum (PG), characterized by necrotizing skin lesions. It highlights the importance of thorough histological examination in diagnosing this variation, which resembles other pustular dermatoses. The case study of a 54-year-old female highlights the unique histological aspects of PG, including epidermal erosion, neutrophilic infiltration, sterile abscesses, and no vasculitis. The article emphasizes the need for differential diagnosis and clinical correlation, emphasizing the importance of collaboration between physicians and pathologists for accurate diagnosis.

Assessing the role of wound debridement in pyoderma gangrenosum-A retrospective cohort study.

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.