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This article discusses a rare case of anaplastic large cell lymphoma (ALCL) presenting as pyoderma gangrenosum, a noninfectious neutrophilic dermatosis. The patient initially presented with painful nodules and ulcers that did not respond to conventional treatment for pyoderma gangrenosum. Further investigations revealed the presence of ALCL, and the patient was treated with chemotherapy, resulting in complete resolution of the ulcers. This case highlights the importance of considering paraneoplastic dermatoses in treatment-resistant conditions and emphasizes the need for thorough systemic investigations. [Extracted from the article]

Pyoderma gangrenosum is a rare dermatologic disorder that disrupts the skin barrier, requiring immunosuppressive therapy. We successfully used cefiderocol for the treatment of an extensively drug-resistant Pseudomonas aeruginosa bacteremia, and presumed osteomyelitis in a patient with severe pyoderma gangrenosum and associated immunosuppressive therapy while being medically optimized for skin grafting. We obtained bone and skin/subcutaneous tissue while the patient was on cefiderocol under an institutional review board-approved biologic waste recovery protocol. Cefiderocol concentrations in bone and skin/subcutaneous tissue were 13.9 and 35.9 mcg/g, respectively. The patient recovered from bacteremia and underwent autografting without further complications. Cefiderocol at approved dosing of 2 g IV (3-hour infusion) every 8 hours resulted in bone and skin/subcutaneous tissue concentrations adequate to treat extensively drug-resistant Gram-negative bacteria that remain susceptible to cefiderocol. [ABSTRACT FROM AUTHOR]

The article offers information on a rare case of pyoderma gangrenosum (PG) associated with aplastic anemia. Topics include the patient's clinical presentation, treatment with prednisolone and cyclosporine for aplastic anemia, unsuccessful management of the ulcer with minocycline and topical ointment, diagnosis of PG based on biopsy results, treatment with adalimumab, discontinuation due to cytomegalovirus infection, and ongoing wound growth due to discontinued immunosuppressive therapy.

This article discusses a case study of a 27-year-old Mexican man diagnosed with pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. PAPA syndrome is an autoinflammatory disease caused by genetic variants in the PSTPIP1 gene. The patient had a history of skin ulcers, cystic nodular acne, and episodes of arthritis. The study identified a specific pathogenic variant in the PSTPIP1 gene. The article also provides information on the clinical manifestations and treatment options for PAPA syndrome. [Extracted from the article]

This article is a systematic review that examines the association between pyoderma gangrenosum (PG) and autoimmune connective tissue disorders (AI-CTD). The review analyzed 78 articles and included 234 patients with PG and AI-CTD. The most common AI-CTD associated with PG was rheumatoid arthritis (RA), followed by systemic lupus erythematosus (SLE). The review also discussed the treatment options for PG, which often involve long courses of immunosuppressants. The authors propose an algorithm for diagnosing AI-CTD in patients with PG, particularly in females with RA. [Extracted from the article]

Cemiplimab has been approved for use in England for locally advanced, unresectable and metastatic cutaneous SCC (Squamous Cell Carcinoma). These cancers are usually morbid, have a detrimental impact on patient and carer quality of life and affect older adults, with limited treatment options. The aim of this study is to present a real world experience of the use, toxicity profile, and response rates of Cemiplimab. In this retrospective review, we present our regional experience of Cemiplimab (350 mg IV 3 weekly) in locally advanced and metastatic SCC, arising in the head and neck area. The electronic records of all patients who were exposed to at least one cycle of Cemiplimab were reviewed. Data was collected including comorbidities (ACE-27), performance status, and toxicity as well as response rates and overall survival. Our patient cohort includes 29 patients, treated between September 2019 and July 2023. Median Age was 76.5 years and 25 of the patients were male with 4 female patients. The median ACE-27 score was 2. All patients had a performance status of 0 or 1. The majority of patients had locally advanced disease 86% (25/29). Of our patient cohort, 83% (24/29) patients had undergone primary surgery following diagnosis of head and neck cutaneous SCC, with 54% (13/24) patients receiving adjuvant radiotherapy following surgery with doses of either 55Gy in 20# over 4 weeks or 60-66Gy in 30-33# over 6 to 6.5 weeks. Of the patients who failed following surgery and/or adjuvant radiotherapy, the mode of failure was locoregional in 79% (19/24). Median Time to failure from primary diagnosis was 6 months. Of the five patients who did not undergo surgery at diagnosis, 2 had locally advanced disease and 3 had metastatic disease. 3 of these 5 patients had palliative radiotherapy (20Gy/5#) after starting Cemiplimab and 1 of these patients received 20Gy/5# before starting Cemiplimab. When patients commenced Cemiplimab, response was assessed either clinically, via CT, PET-CT and/or MRI. The median number of cycles of Cemiplimab delivered was 7. The commonest side effects reported were fatigue (6 out of 29, 21%) and development of a new rash (5 out of 29, 17%). Only 3 of the 29 (10%) patients developed Grade 3 toxicity. All three patients discontinued treatment, one had developed hepatitis, the second patient developed pyoderma gangrenosum and it was unclear whether this was a direct toxicity from Cemiplimab, the 3rd patient developed colitis. All three required steroids and only 1 required hospitalisation. There were no toxicity associated deaths. Of the 3 patients who developed grade 3 toxicity, 2 have died of other causes and one is alive and disease free. The median time to the first radiological response assessment was 9.9 weeks. The response rate to treatment at first response assessment was 69% (20/29) and overall response rate at the end of the study period was 52% (15/29). Of the 3 patients who had complete response at first assessment this was maintained through the study period, and of the 17 patients who had a partial response at first assessment, 6 patients went on to develop complete response. All 9 patients who achieved complete response were able to maintain this throughout the study period. At the end of the study period median follow up was 7 months (0 to 37 months), with 12 patients still alive, 8 of whom have a complete response to treatment and 4 of whom have a partial response to treatment. Of the 29 patients in this study, 17 patients have died. 12 of disease progression and 5 of other causes. Median overall survival was 12 months. We note that our study population was overwhelmingly male, however in terms of demographics and toxicity profile was reflective of the populations presented in clinical trials. The small sample size reflects the difficult clinical decision making involved in managing this heterogenous group of patients. It is interesting that out of our patients who have achieved a complete response to treatment with Cemiplimab, none have progressed during the course of this study. Cemiplimab offers a safe and effective treatment option to locally advanced unresectable or metastatic cutaneous SCCs of head and neck primary origin, which overwhelmingly affect older adults. Our review highlights the importance of careful case selection. An interesting question is whether patients who achieve complete response to treatment, require a full 2 years of Cemiplimab immunotherapy. [ABSTRACT FROM AUTHOR]

This article discusses a rare case of concurrent vulvar Crohn's disease (CD) and pyoderma gangrenosum (PG). CD is a chronic inflammatory condition that affects the gastrointestinal tract, and vulvar involvement is a rare extraintestinal manifestation. PG is an autoinflammatory dermatitis that is infrequently seen in patients with CD. The article presents the case of a 33-year-old female with vulvar CD and PG, describing her symptoms, physical exam findings, and treatment. The article emphasizes the need for a multidisciplinary approach to care and mentions various treatment options for vulvar CD and PG. [Extracted from the article]

Pyoderma gangrenosum is a rapidly progressing rare systemic disease that is characterized by idiopathic chronic skin changes and can be seen with systemic diseases. In rapidly progressive lesions following surgery, it is vital to differentiate pyoderma gangrenosum from necrotizing soft tissue infections in which the primary treatment is debridement. In this report, we present a case of pyoderma gangrenosum with pulmonary involvement whose medical condition worsened after serial debridements were performed because of a misdiagnosis of necrotizing fasciitis. [ABSTRACT FROM AUTHOR]

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.

Abstract Background Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by chronic vasculitis involving small to medium sized arteries, granulomatous inflammation of the upper and lower respiratory tracts, pauci-immune necrotizing glomerulonephritis, as well as vasculitis of other organs. Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory syndrome characterized by sterile bone inflammation. Case presentation We report a case of CRMO that was doing well on non-steroidal anti-inflammatory drugs (NSAID for 6 years and then developed ANCA positive limited GPA presenting with pyoderma gangrenosum, persistent bilateral otalgia with serous otitis, otorrhea, then sensorineural hearing loss. Conclusion This is the first report of limited GPA initially presenting as pyoderma gangrenosum in a patient with underlying CRMO. It is unclear how the pathology of an autoimmune and an autoinflammatory condition can overlap.

Sarah L Becker,1,* Rose Velasco,2,* Alex G Ortega-Loayza1 1Department of Dermatology, Oregon Health & Science University, Portland, OR, USA; 2University of Illinois College of Medicine, Chicago, IL, USA*These authors contributed equally to this workCorrespondence: Alex G Ortega-Loayza, Department of Dermatology, Oregon Health & Science University, 3303 S Bond Ave Building 1, 16th Floor, Portland, OR, 97239, USA, Tel +1 503-418-3376, Fax +1 503-494-6968, Email ortegalo@ohsu.eduAbstract: Pyoderma gangrenosum (PG) is a rare, autoinflammatory disease leading to aseptic ulcers which carries a significant disease burden and is often difficult to treat, with many patients failing first-line treatment and requiring additional therapies. Such cases are typically referred to in the literature as “recalcitrant”, “refractory”, or “resistant”, though little is known about the clinical characteristics of such cases. We performed a narrative literature review to characterize patient demographics and clinical course associated with difficult to treat pyoderma gangrenosum cases in order to identify trends to guide future clinical management and therapeutic innovation. We identified 148 cases with clinical manifestations and associated patient demographics stratified by ulcer and patient features. Consistent with previous work, a greater prevalence of PG was observed among female patients and those with a history of inflammatory bowel disease, however interestingly despite an aggressive course to their PG, few patients had comorbidities complicating their disease course. Additionally, despite the requirement of three or more treatments for most patients’ disease to resolve, the majority healed within the typical window observed in previous clinical studies with low rates of recurrence. Biologics were the most common medication patients were on at time of remission. Collectively, our results suggest a potential benefit for a reduced threshold for biologic initiation in PG patients and a need for standardization of language in the field to facilitate treatment outcomes comparisons and interventions.Keywords: pyoderma gangrenosum, recalcitrant, refractory, resistant, biologics

Qianqian Zhou,1 Shengru Zhou,1 Huizi Xiong,1 Jianqiu Yang,1 Ziliang Yang,2 Naihui Zhou,2 Jinzhu Mao,2 Min Li1,2 1Department of Dermatology, Dushu Lake Hospital Affiliated to Soochow University (Medical Center of Soochow University, Suzhou Dushu Lake Hospital), Suzhou, People’s Republic of China; 2Department of Dermatology, The First Affiliated Hospital of Soochow University, Suzhou, People’s Republic of ChinaCorrespondence: Min Li, Department of Dermatology, Dushu Lake Hospital Affiliated to Soochow University (Medical Center of Soochow University, Suzhou Dushu Lake Hospital), No. 9, Chongwen Road, Suzhou, 215123, People’s Republic of China, Tel +86-18936140383, Email lm@suda.edu.cnIntroduction: Psoriasis is an immune-mediated chronic inflammatory skin disease. As our understanding of the pathogenesis of psoriasis has improved, biologic agents have become increasingly important in the treatment of psoriasis. However, the use of biologic agents is associated with cutaneous side effects. A new type of side effect called paradoxical reactions is an emerging threat arising from the increasing use of biologic agents.Case: Here, we present a case of paradoxical skin reactions – pyoderma gangrenosum (PG) and eczema – induced by biologic therapy. The case was successfully and eventually treated with baricitinib.Discussion: PG is a rare inflammatory disease characterised by painful and necrotic ulcerations containing neutrophils. It has been associated with autoimmune diseases such as inflammatory bowel disease (IBD). TNF (tumor necrosis factor) -α inhibitors can effectively treat refractory PG, while IL (interleukin) − 17A inhibitors may worsen IBD symptoms. The cause of PG in this case was believed to be secukinumab, not adalimumab. The patient was diagnosed with eczematous dermatitis due to TNF-α inhibitors, and baricitinib was added to treat eczematous dermatitis.Conclusion: Paradoxical reactions are unpredictable events that may occur during treatment with biologics at anytime. They need further research in order to formulate personalised treatment.Keywords: biology drugs, pyoderma gangrenosum, eczema, adalimumab, secukinumab, baricitinib

Abstract Pyoderma gangrenosum (PG) belongs to the group of classical neutrophilic dermatoses and is characterised by painful ulcerations. While the clinical features have been well described, the underlying pathological mechanisms are incompletely understood and adequate treatment remains challenging. A 63‐year‐old male patient had suffered from recalcitrant ulcerations on the back, groins and legs for over 20 years. Due to suspicion of PG, a skin biopsy of the flank was obtained, which was confirming the suspected diagnosis. Following several frustrating attempts to treat, another skin biopsy was carried out and a cytokine profile by quantitative reverse transcription polymerase chain reaction was performed. Compared to healthy skin, a notable upregulation of interleukin (IL)‐17A and IL‐8 was detected. Subsequently, treatment with the anti‐IL17 antibody secukinumab was started. After 3 months of treatment, the PG area was 0 cm2. Due to the discontinuation of the drug by the patient typical PG lesions reappeared, all of which disappeared again within a few weeks of restarting secukinumab therapy. At month 23, the PG area was again 0 cm2 and this complete response was maintained under secukinumab therapy to this day (76 months after the onset of secukinumab therapy). This case of a patient with therapy‐resistant PG and subsequent long‐term responsiveness to anti‐IL‐17 therapy supports the potential importance of the Th17 axis in PG. Collectively, treatment of PG using IL‐17 antagonists seems to be very promising.

Major aphthae are usually located on the dorsum of the tongue, the mucosal surface of the lips and the palate. They are large, round or oval ulcers, with a whitish-grey bed, well-defined borders and erythematous halo. They are very often accompanied by severe pain. Major aphthae can take up to four months to heal, often with a scar. Relapses are possible. We present a case of “giant” major aphtha that was previously diagnosed as squamous cell carcinoma.

AbstractThe purpose of the article Pyoderma gangrenosum (PG) is an ulcerating neutrophilic dermatosis with an incidence of 3-10 patients per million. PG equally affects patients of both sexes and of any age. Of these patients, 50-75% are associated with auto-immune disease. The lower extremities are the most commonly affected body parts. Minor trauma to the skin may result in the development of new lesions. Patients complain of chronic, nonhealing ulcers with associated pain. Treatment starts with systemic or intralesional corticosteroids, however, no official treatment protocol currently exists. Recent success has been found with biologic agents such as TNF-a inhibitor, although the treatment efficacy in these reports is limited. As for the pregnant patient, the drug selection is difficult. In this report, we want to assess the efficiency of certolizumab in the pregnant patient.Results We report a case of a patient with PG, who responded well to certolizumab, 400 mg as a booster dose, followed by 200 mg biweekly for 8 weeks. The lesions gradually resolved and followed up for 5months without side effect. In addition, we reviewed the literature and compared the current treatment efficiency in the treatment of PG.Conclusion Certolizumab may be a promising therapeutic option for patients with severe PG.

Objective: The objective of the study was to describe the epidemiological, clinical, therapeutic and evolutionary aspects of systemic diseases (SD) with cutaneous expression associated with HIV infection and to identify the circumstances of their occurrence. Patients and methods: This were a cross-sectional, descriptive, and analytical study of the records of patients hospitalized or followed up at the Voluntary Counselling and Testing Unit (VCT) of the Dermatology Department of the University Hospital of Treichville, from January 2005 to December 2014. Results: We recorded 63 patients with MS during this period, 13 of whom were HIV positive (20.6%). MS of HIV positive patient was more frequent after 24 years (mean age: 49.5 years) and a male predominance (58.3%). HIV 1 was involved in all cases with a mean CD4 count of 190/mm3 [18-521] at diagnosis of MS. HIV-associated MS were psoriasis (7 cases), cutaneous lymphoma (2 cases), Goujerot-Sjögren syndrome (1 case), pyoderma gangrenosum (1 case), systemic scleroderma (1 case) and sarcoidosis (1 case). They were circumstances of discovery of HIV infection in 7 cases, expression of therapeutic failure in 3 cases and expression of an immune restoration syndrome in 3 cases. The 13 patients received concomitant antiretroviral treatment and the specific treatment of the systemic pathology associated with an antiinflammatory in some cases. Conclusion: The association of MS and HIV infection is rare. Their etiologies are dominated by psoriasis in Abidjan. A well-conducted interrogation to determine the chronology of events, assisted by biological examinations, allows the circumstance of occurrence to be identified. [ABSTRACT FROM AUTHOR]

Background: Immune-related cutaneous adverse events (ircAEs) are frequent and may reduce quality of life and consistent dosing. IL12/23 has been implicated in psoriasis, which is reminiscent of the psoriasiform/lichenoid ircAE phenotype. We report the use of ustekinumab as a therapeutic option. Methods: Patients at Memorial Sloan Kettering Cancer Center, New York, who received immune checkpoint inhibitors and were treated with ustekinumab or had the keywords "ustekinumab" or "Stelara" in their clinical notes between 1 March 2017 and 1 December 2022 were retrospectively identified via a database query. Documentation from initial and follow-up visits was manually reviewed, and response to ustekinumab was categorized into complete cutaneous response (CcR, decrease to CTCAE grade 0), partial cutaneous response (PcR, any decrease in CTCAE grade exclusive of decrease to grade 0), and no cutaneous response (NcR, no change in CTCAE grade or worsening). Labs including complete blood count (CBC), cytokine panels, and IgE were obtained in a subset of patients as standard of care. Skin biopsies were reviewed by a dermatopathologist. Results: Fourteen patients with psoriasiform (85.7%), maculopapular (7.1%), and pyoderma gangrenosum (7.1%) ircAEs were identified. Ten (71.4%) receiving ustekinumab had a positive response to treatment. Among these 10 responders, 4 (40%) demonstrated partial cutaneous response and 6 (60%) demonstrated complete cutaneous resolution. Six patients (42.9%) experienced interruptions to their checkpoint inhibitor treatment as a result of intolerable ircAEs, and following ircAE management with ustekinumab, two (33.3%) were successfully rechallenged with their checkpoint inhibitors. On histopathology, patients primarily had findings of interface or psoriasiform dermatitis. No patients reported an adverse event related to ustekinumab. Conclusions: Ustekinumab showed a benefit in a subset of patients with psoriasiform/lichenoid ircAEs. No safety signals were identified. However, further prospective randomized controlled trials are needed to confirm our findings. [ABSTRACT FROM AUTHOR]

Malakoplakia is a rare chronic inflammatory condition that most commonly involves the urogenital tract. Cutaneous malakoplakia is extremely rare and many patients diagnosed with skin involvement are immunosuppressed. While the clinical presentation of cutaneous malakoplakia is variable, the histopathologic features are quite distinct and include sheets of closely packed dermal histiocytes with foamy‐appearing cytoplasm and Michaelis–Gutmann bodies that are positive with certain immunohistochemical stains. While the exact pathogenesis of malakoplakia is unknown, it has been associated with certain bacterial infections. Treatment generally involves a combination of surgery and antimicrobial agents and/or modulation of immunosuppressant therapy if appropriate. Herein, the authors report a unique case of cutaneous malakoplakia arising in a patient on chronic immunosuppressive therapy for the management of pyoderma gangrenosum. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The simultaneous occurrence of pyoderma gangrenosum and systemic lupus erythematosus is exceedingly rare and poses diagnostic challenges due to the similarity of skin manifestations in both conditions. The exact relationship between the two conditions remains unclear; however, it is hypothesized that immune dysregulation and neutrophilic infiltration may play a role in the development of pyoderma gangrenosum in SLE patients. Clinicians should be vigilant in recognizing such uncommon associations to ensure prompt and appropriate management. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) is a chronic neutrophilic disorder characterized by recurrent painful ulcers. Aseptic inflammation by neutrophils plays an essential role, and neutrophil extracellular traps (NETs) formation can contribute to the pathogenesis of PG. Seventy‐five patients were diagnosed as having PG in our department, among which 58 ulcerative, 4 bullous, 3 pustular and 10 vegetative type. We examined the 20 skin biopsy specimens (11 ulcerative, 3 bullous, 2 pustular and 4 vegetative type), and local NETs formation in various types of PG was compared among each type. The biopsied specimens were double labelled for myeloperoxidase, citrullinated histone H3. Immunofluorescent images indicated that the histopathologic location and depth of NETs formation in PG varied by the clinical subtypes. In ulcerative PG, NETs formation was observed in the upper to deep dermis. In bullous PG, NETs formation was mainly observed in the epidermis. Pustular type showed NETs formation in the epidermis near the pustules, and in vegetative type, showed NETs formation mainly in the upper dermis. These results indicate that NETting neutrophils play an important role in the pathogenesis of various forms of PG, although the location and depth of NETs formation in the skin lesion of PG differ depending on each type. Further studies are necessary to examine what factors identify different clinical features of PG. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum is a rare neutrophilic dermatosis characterized by painful skin ulcers with necrotic, undermined margins. In severe cases, particularly in pediatric patients, work‐up for an associated autoimmune, inflammatory, malignant, or genetic disorder should be considered based on the clinical presentation. We report a unique case of pediatric pyoderma gangrenosum with a leukemoid reaction, secondary to an autosomal recessive leukocyte adhesion deficiency type 1. [ABSTRACT FROM AUTHOR]

Refractory pyoderma gangrenosum associated with rheumatoid arthritis successfully treated with upadacitinib. A significant number of cytokines rely on JAK1 and JAK2, while the dependency of cytokines on JAK3 is more limited.[6] IL-6 activates intracellular signaling pathways primarily involving JAK1 and STAT3. The successful treatment of these patients with JAK inhibitors suggests the interference on the inflammatory signaling mediated by IL-6. [Extracted from the article]

Objective: Rare disease Background: Septic arthritis of the facet joint (SAFJ) has been considered a rare type of spinal infection. However, because of the aging of the population, the increase in compromised hosts, and the increase in MRI use in recent years, the number of reports has been increasing. We report the clinical progress of a rare case of septic arthritis of the cervical facet joint (SACFJ) with some imaging considerations, and we compare our findings with existing reports of SACFJ. Case Report: A 73-year-old Japanese woman presented with fever, paralytic symptoms, and paresthesia of the upper limbs. Here, we report a case of SACFJ in which MRI findings allowed early diagnosis, and a favorable course was obtained by conservative treatment with antibiotics. Although MRI performed 93 days after the initiation of treatment showed a slight residual signal change in the facet joints, no symptoms had recurred by the sixth month after hospital discharge. Conclusions: If a patient develops neurological symptoms such as paralysis with fever and increased inflammatory response, the physician must consider the possibility of pyogenic spondylitis, including SACFJ, and order an MRI. Epidural abscess is almost inevitable in SACFJ, and surgical treatment, including abscess drainage, is required if spinal cord or paralytic symptoms progress. For patients with SACFJ, as well as pyogenic spondylitis, MRI may not be useful in determining treatment efficacy. [ABSTRACT FROM AUTHOR]

Case: A 61-year-old woman with lower back and radicular leg pain underwent minimally invasive spinal fusion at L5-S1. By postoperative day 6, she exhibited erythema, wound dehiscence, and necrotic changes. Although a necrotizing infection was initially suspected, multiple debridements and antibiotic therapy failed to improve her condition. The patient was eventually diagnosed with pyoderma gangrenosum (PG) and was managed with immunosuppressants and extended wound care. Conclusion: PG is a rare dermatosis that is often misdiagnosed, leading to inappropriate treatment, debridements, and additional complications. Prompt identification and multidisciplinary collaboration are key to preventing unnecessary interventions and achieving the best outcomes. [ABSTRACT FROM AUTHOR]

Inflammation is a physiologic defense mechanism against an out-side attack. Usually, it resolves after the removal of noxious causes, but systemic autoinflammatory disorders (SAIDs) have recurrent or repeated acute inflammation through uncontrolled gene function, which can present as gain-of-function or loss-of-function of a gene during inflammation. Most SAIDs are hereditary autoinflammatory diseases and develop by dysregulation of innate immunity through various pathways including inflammasomes, endoplasmic reticulum stress, nuclear factor-κB dysregulation, and interferon production. The clinical manifestations include periodic fever with various skin findings such as neutrophilic urticarial dermatosis, or vasculitic lesions. Some SAID cases stem from immunodeficiency or allergic reactions related to monogenic mutation. The diagnosis of SAIDs is based on clinical findings of systemic inflammation and genetic confirmation, and have to exclude infections or malignancies. Moreover, a genetic study is essential for clinical features to be suspect SAID with or without a family history. Treatment is based on understanding the immunopathology of SAID, and targeted therapy to control disease flares, reduce recurrent acute phases and prevent serious complications. Diagnosing and treating SAID requires understanding its comprehensive clinical features and pathogenesis related to genetic mutation. [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis (RA) is a chronic, systemic autoimmune disorder characterized by inflammatory arthritis and periarticular structural damage. Available evidence suggests that RA results from complex interactions between genetic susceptibility (e.g., HLA-DRB1), environmental factors (e.g., smoking), and immune dysregulation. Alongside joint-related symptoms, individuals with RA may also experience a wide array of skin issues, including the development of nodules, neutrophilic dermatoses, vasculitis, and vasculopathy. Treatment strategies for these manifestations vary but routinely involve corticosteroids, disease-modifying anti-rheumatic drugs, and biologics, with individualized approaches guided by disease severity. In this review, we provide comprehensive insights into the skin-related issues associated with RA, outlining their clinical characteristics and histopathological findings. Our aim is to facilitate early diagnosis and personalized treatment to improve the quality of life of affected individuals. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) after breast-conserving surgery is rare, and its diagnosis is often delayed because of the similarity to wound infection and the broad differential diagnosis for PG, making it a diagnosis of exclusion. A 60-year-old woman who underwent breast conserving surgery and sentinel lymph node biopsy for invasive breast carcinoma presented with increasing erythema, fever and serosanguinous discharge in the lower outer quadrant of the right breast at the site of tumour excision on postoperative day (POD) 9. Fever persisted despite antibiotics and the patient was noted to have leucocytosis (0.9 × 109/L), neutrophilia (37.8 × 109/L) and elevated C-reactive protein levels (136 µg/mL) on POD 16. Microbiology and blood culture results were negative but the breast ulcer continued to expand at a rate of 1-2 cm a day. The patient underwent surgical debridement on POD 21 to rule out necrotising soft tissue infection. Persistent ulcer progression, despite debridement and antibiotics, led to clinical suspicion of PG and the patient was started on prednisolone and cyclosporin. A rapid response was seen with treatment and an optimum healing process was noted over the subsequent three-month follow-up period. Early suspicion, careful macroscopic evaluation of disease progression and appropriate use of immunosuppressive therapy are important for the management of PG. Prompt initiation of immunosuppressive therapy may avoid unnecessary treatment and aggravation of the surgical wound. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with underlying disorders. The association between PG and solid organ tumors (SM), including gynecologic cancers, has been previously reported. Here, we report a case of a 61‐year‐old woman with pyoderma gangrenosum on the posterior auricular region associated with an underlying fallopian tube carcinosarcoma: a rare and aggressive gynecologic malignancy. The patient's ulcer responded favorably to treatment, and surgical resection of the tumor was performed. The patient was then referred for further cancer management. No new lesions or recurrences were found over the 18 months of routine follow‐up. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful ulcers with undermined edges and peripheral erythema. The global incidence of PG is estimated to be 3 to 10 cases per million people per year, affecting individuals aged between 25 to 54 years. We here present a case report of a 34-year-old female with deep painful non-healing ulcers on both legs which first appeared during her pregnancy. Prolonged consumption of propylthiouracil (PTU) to treat hyperthyroidism was found. Gastrointestinal symptoms were denied. Histopathological examination revealed intense neutrophilic infiltration of the dermis in the skin biopsy and chronic ulcerative inflammation in the biopsy of ileum and colon. The result of p-ANCA was positive. The patient was diagnosed with drug-induced pyoderma gangrenosum associated with PTU use and inflammatory bowel disease. Combination therapy of systemic corticosteroid, two split-thickness skin graft (STSG) surgeries, oral dapsone, appropriate wound care, and management to eliminate predisposing factors resulted in a favorable outcome. Pyoderma gangrenosum has been considered a challenging disease to diagnose and treat. Clinicians need to enhance their knowledge of diagnosis and management of PG to give proper management and yield a better prognosis. [ABSTRACT FROM AUTHOR]

Objective To characterize the demographic and the cutaneous features of pyoderma gangrenosum in large series of patients. Methods This is a case series cross sectional descriptive study with therapeutic trial where 53 patients with pyoderma gangrenosum were seen during the period from September 2013-January 2023.All patients were fully investigated and diagnosed as cases of pyoderma gangrenosum. Still full history and examination were carried out to characterize the clinical pictures. All patients were fully investigated to exclude any associated disease in doubtful diagnosis. Skin biopsy was performed when needed as exclusive measure. Triple therapeutic trial was tried using oral azathioprine, oral dapsone and oral prednisolone. Results Fifty-three patients were included, their ages ranged from 10-60 years with a mean 45 years, with 39 (73.58%) males and 14 (26.41%) females with ratio 2.78:1. The duration of lesions ranged from 4-12 weeks. The location of these lesions was mostly lower limbs in 31(58.49%) cases. For the rest of the cases, lesions appeared on the trunk including the groin and neck in 14 (26.41%), breast of the female in 3 (5.66%) cases, upper limbs in 2 (3.77%) cases, male genitalia in 2 (3.77%) cases and face in one (1.88%) case. The lesions were single in 36 (67.9.21%) patients while multiple in 17 (32%) cases. The rash had mostly painful ulcerative yellowish surface with undermined edges. The ulcerative subtype was the most frequently recorded in 48 (90.56%) patients. No associated underlying triggering pathologies were noticed during follow up except in seven (13.2%) patients. The therapeutic response was noticed after two weeks but complete clearance of ulcers took several weeks to months according to the size of ulceration. Conclusion This study showed that pyoderma gangrenosum is a disease of middle-aged males with no well-defined etiological triggering factors except in 13.2% of cases that have been associated with miscellaneous underlying diseases. Accordingly, the etiopathogenesis of pyoderma gangrenosum remains idiopathic, still long follow up might discover many other concealed etiologies. Effective triple therapy was tried and proved its effectiveness. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum is a rare neutrophilic dermatosis that leads to exceedingly painful ulcerations of the skin. Although the exact pathogenesis is not yet fully understood, various auto-inflammatory phenomena with increased neutrophil granulocyte activity have been demonstrated. Despite the limited understanding of the pathogenesis, it is no longer a diagnosis of exclusion, as it can now be made on the basis of validated scoring systems. However, therapy remains a major multidisciplinary challenge. Various immunosuppressive and immunomodulatory therapies are available for the treatment of affected patients. In addition, concomitant topical pharmacologic therapy, wound management and pain control should always be addressed. Corticosteroids and/or cyclosporine remain the systemic therapeutics of choice for most patients. However, in recent years, there has been an increasing number of studies on the positive effects of biologic therapies such as inhibitors of tumour necrosis factor-α; interleukin-1, interleukin-17, interleukin-23 or complement factor C5a. Biologics have now become the drug of choice in certain scenarios, particularly in patients with underlying inflammatory comorbidities, and are increasingly used at an early stage in the disease rather than in therapy refractory patients. [ABSTRACT FROM AUTHOR]

Background: Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS. Case presentation: Through a multidisciplinary approach, this patient was also found to have very early onset inflammatory bowel disease (VEO-IBD) and Mevalonate kinase-associated disease (MKAD). While prior case studies have characterized an association between VEO-IBD and MKAD, there is no literature describing the association of all three diagnoses this case: H-SS, VEO-IBD and MKAD. Initiation of canakinumab in this patient resulted in successful control of the disease. Conclusions: This case highlights the importance of a multidisciplinary approach to rare diagnoses, and collaboration during cases with significant diagnostic uncertainty. [ABSTRACT FROM AUTHOR]

The article outlines an expert panel consensus on the practical applications and clinical benefits of small intestine submucosa extracellular matrix (SIS-ECM), including appropriateness in wound indications, promotion of cell migration and tissue formation relevant to wound healing, management of wound indications in dermatological diseases, efficacy and cost-effectiveness.

Hidradenitis suppurativa (HS) is a clinically heterogeneous disease with a broad spectrum of clinical features. Attempts to classify HS into distinct clinical phenotypes could lead to a better understanding of the condition and the development of individualized treatment protocols. We summarize some of the existing phenotype classifications and present our experience with 250 patients and their many clinical presentations. We have emphasized the pathophysiologic and clinical overlap between HS and pyoderma gangrenosum. The more severe presentations can include erosive and ulcerative lesions, sometimes associated with vegetative changes leading to diagnostic quandaries. We propose a new phenotype of pyoderma gangrenosum–like HS in which painful ulcerative or vegetative lesions appear in sites affected by HS, their activity coincides with the flareups of classic inflammatory manifestations of HS, and they heal with cribriform or atrophic scars. [ABSTRACT FROM AUTHOR]

Drainage from chronic wounds can significantly negatively impact a patient's quality of life. Change in severity of wound drainage is an important measure of treatment efficacy for wounds. This study reviews existing tools used to assess wound drainage. Qualitative drainage tools are overall less burdensome, and however, differences in user interpretation may reduce inter-rater reliability. Quantitative drainage tools enable more reliable comparisons of drainage severity and treatment response between patients but sometimes require equipment to administer, increasing responder burden. Gaps in the current wound drainage measurement landscape are highlighted. Many of the existing scales have not been validated in robust studies. There is also a lack of validated global drainage measurement tools for patients with chronic inflammatory skin disorders with drainage, such as hidradenitis suppurativa or pyoderma gangrenosum. Development of a succinct drainage measurement tool for inflammatory skin diseases where drainage is a prominent symptom will improve monitoring of meaningful treatment response. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) is a neutrophilic dermatological disease, whose pathogenesis is still poorly clarified. Because of the lack of validated criteria for diagnosis and response, PG treatment is still challenging and should be differentiated in the inflammatory and non-inflammatory phases. Our study aimed to provide a new semi-quantitative approach for PG diagnosis and monitoring, identifying ultra-high-frequency ultrasound (UHFUS) early biomarkers associated with the transition between the two phases. We enrolled 13 patients affected by painful PG lesions evaluated during the inflammatory phase (T0) and during the non-inflammatory phase (T1): pain was measured by the Visual Analogue Scale (VAS); clinical features were recorded through digital photography; epidermis and dermis ultrasound (US) characteristics were evaluated by UHFUS examination with a 70 MHz probe (Vevo MD® FUJIFILM VisualSonics). In T1 UHFUS examination, the presence of hyperechoic oval structures was lower compared to T0 (p value < 0.05). An hyperechogenic structure within the oval structure, suggestive of a hair tract, was evident in T0 and absent in T1 (p value < 0.05). In T0, blood vessels appear as U-shaped and V-shaped anechoic structures with a predominance of U-shaped vessels (p value < 0.05) compared to the more regular distribution found in T1. Finding early biomarkers of the transition from the inflammatory to the non-inflammatory phase could provide new insight in terms of therapeutic decision making and response monitoring. The differences found by this study suggest a potential use of UHFUS for the development of an objective standardized staging method. Further investigations will be necessary to confirm our preliminary results, thus providing a turning point in PG early detection, differential diagnosis and treatment monitoring. [ABSTRACT FROM AUTHOR]

Background: Neutrophilic eccrine hidradenitis (NEH) is a benign neutrophilic dermatosis not well recognized beyond an association with malignancy. Although the disease is commonly reported in adults with malignancy, this association is uncommon in children. The diagnosis of NEH is predominantly based on histologic findings to exclude alternative diagnoses for adults, but biopsy is not usually required for children. Methods: A retrospective study was performed of adult and pediatric patients diagnosed with NEH at three Mayo Clinic sites from January 1, 1992, to January 1, 2022. The aim of this study was to elucidate risk factors for NEH and its clinical characteristics, treatment options, and natural course. Clinical information and pathologic results were collected from health records. Available pathologic slides were reviewed with a dermatopathologist. Results: Of 47 patients identified, 33 had either histologic or clinical confirmation of the diagnosis; 21 were adults (64%), and 12 were children (36%). Most adults (16/21; 76%) had underlying malignancy and received chemotherapy. Five adults (24%) were classified as having idiopathic NEH, and they were younger and had higher NEH recurrence rates than the other adults. Only one pediatric patient (8%) had underlying malignancy. For 10 children (83%), NEH was preceded by strenuous activity. Initial findings of idiopathic NEH were palmoplantar eruptions for both adult and pediatric patients, whereas malignancy‐associated NEH commonly involved the face and axillae. Conclusions: Among adults, NEH is commonly associated with malignancy and chemotherapy. Among children, idiopathic NEH occurs primarily after overexertion, and malignancy is highly unlikely to be the cause of NEH. [ABSTRACT FROM AUTHOR]