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Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disease that mainly involves bones, joints, and skin, characterized by sterile osteitis and neutrophilic dermatosis. SAPHO syndrome and PG may be different variations of the spectrum of autoinflammatory diseases. [Extracted from the article]

Background: Adult-onset immunodeficiency (AOID) due to interferon-gamma autoantibody is a rare, acquired immunodeficiency disease. Reactive neutrophilic dermatoses (RND), predominantly Sweet syndrome (SS), and generalized pustular eruption have been reported repeatedly. Objectives: The aims of this study were to describe the cutaneous manifestations in AOID patients and determine the incidence of RND and associated factors using a larger population size than have been previously reported. Methods: A retrospective chart review of all confirmed AOID cases in Chiang Mai University Hospital from January 2006 to June 2020 was conducted. The demographics and characteristics of RND including type, onset, and laboratory information in every episode of cutaneous manifestations were collected. Generalized estimating equations of binary logistic regression were used to determine the indicators of RND. Results: A total of 146 patients with confirmed AOID were identified. Of these, 57 cases (39%) developed at least one episode of RND. Thirteen cases (23%) of the patients experienced RND twice during the follow-up period. All recurrence of RND displayed the same cutaneous phenotype, with the exception of 2 cases who had both SS and generalized pustular eruption. Finally, 49 episodes of SS and 22 episodes of generalized pustular eruption were included in the analysis. All patients with RND had concomitant active opportunistic infections, of which most were non-tuberculous mycobacterium (NTM) infection. NTM infection (prevalence odds ratio [POR] 2.87), lymphadenopathy (POR 3.30) as well as lower serum alkaline phosphatase (ALP) level (POR 0.71 for every 100-unit increment in ALP) were found to be significantly associated with RND occurrence. Conclusions: 39% of our AOID patients experienced RND once during the course of the disease. Notable factors associated with RND occurrence were concomitant NTM infection, lymphadenopathy, and lower level of ALP. [ABSTRACT FROM AUTHOR]

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare disease characterized by osteoarticular and cutaneous manifestations. SAPHO syndrome mostly involves the skin, mainly presented as palmoplantar pustulosis and severe acne. Sweet's syndrome (SS) is a neutrophilic dermatosis of unknown cause, which may be caused by autoinflammation. So far, SAPHO syndrome complicated with SS has been rarely reported worldwide. Here, we present a rare case diagnosed in our hospital with detailed clinical information. This patient presented pain and swelling in her right leg. Later, she developed red papules on her right lower eyelid and a skin biopsy showed diffuse lymphocytic and neutrophilic infiltration in the superficial dermis. She was diagnosed with SAPHO syndrome and SS according to medical history and examination. These two diseases share parts of autoinflammatory signaling pathways and might be different variations of the spectrum of autoinflammatory diseases. Through this case, we aim to provide a new horizon for the regulation of neutrophils in SAPHO syndrome and skin lesions like SS. [ABSTRACT FROM AUTHOR]

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Key Clinical Message Partial leukocyte adhesion deficiency type 1 (LAD‐1) deficiency is extremely rare condition with milder infectious manifestation and immune system imbalance leads to increased risks of autoinflammatory complications, such as pyoderma gangrenosum, that can be triggered by trauma or pregnancy. In patients with spice‐site ITGB2 variants, partial expression can occur due to different β2 integrin isophorms expression. Abstract LAD‐1, OMIM ID #116920 is a rare, autosomal recessive disorder that results from mutations in the ITGB2 gene that encodes the CD18 β2 integrin subunit. According to the CD18 expression, LAD‐1 is categorized as severe (30%). Here, we describe a 22‐year‐old female, who presented with inflammatory skin disease and oral cavity, as well as respiratory tract infections during the first year of life. LAD‐1 was diagnosed at the age of 2 years by low expression of CD18 (1%). Whole‐exome sequencing identified homozygous c. 59‐10C>A variant in the ITGB2 gene. Despite severe phenotype, the patient survived to adulthood without hematopoietic stem cell transplantation and became pregnant at the age of 20 years, with pregnancy complicated by a pyoderma gangrenosum‐like lesion. During her life, CD18 expression increased from 1% to 9%; at 22 years of age, 5% of neutrophils and 9% of lymphocytes were CD18+. All CD18+‐lymphocytes were predominantly memory/effector cytotoxic T cells. However, revertant mosaicism was not being established suggesting that CD18 expression variability may be mediated by other mechanisms such as different β2 integrin isophorms expression.

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Background: Pyoderma gangrenosum is a rare neutrophilic dermatosis with very low incidence and varied clinical presentation associated with different comorbidities. It is also a diagnostic-therapeutic challenge. Clinical case: A 79-year-old woman presented 18 months of dermatosis evolution that began as a perianal pustule and progressed to a 20 cm in diameter ulcer. Due to her comorbidities, she received topic treatment with satisfactory evolution. Conclusions: Pyoderma gangrenosum is frequently associated with a systemic disease. Consequently, it must be approached from a systemic perspective with the support of a multidisciplinary team to provide the most appropriate treatment.

Key Clinical Message After the infection with COVID‐19, pyoderma gangrenosum worsened and further led to necrosis following pyogenic osteomyelitis. Infection is a major exacerbating factor in pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG is not fully understood; however, it is most commonly considered a disease in the spectrum of neutrophilic dermatoses. The treatment of PG remains challenging due to the lack of generally accepted therapeutic guidelines. Existing therapeutic methods focus on limiting inflammation through the use of immunosuppressive and immunomodulatory therapies. Recently, several reports have indicated the successful use of biologic drugs and small molecules administered for coexisting diseases, resulting in ulcer healing. In this review, we summarize the discoveries regarding the pathophysiology of PG and present treatment options to raise awareness and improve the management of this rare entity.

Neutrophilic dermatoses are a wide group of disorders encompassing indolent to severely disabling conditions. A co-existence of two such conditions, pyoderma gangrenosum (PG) and subcorneal pustular dermatosis, necessitates a thorough investigation for IgA dysglobulinemia. We report a middle-aged woman who developed PG following 18 years of (undiagnosed) subcorneal pustular dermatosis, along with rheumatoid arthritis, a known association of PG.

Neutrophilic dermatoses are a wide group of disorders encompassing indolent to severely disabling conditions. A co-existence of two such conditions, pyoderma gangrenosum (PG) and subcorneal pustular dermatosis, necessitates a thorough investigation for IgA dysglobulinemia. We report a middle-aged woman who developed PG following 18 years of (undiagnosed) subcorneal pustular dermatosis, along with rheumatoid arthritis, a known association of PG. [ABSTRACT FROM AUTHOR]

Keywords: Pyoderma gangrenosum; Pain; Diagnostic delay; Survey EN Pyoderma gangrenosum Pain Diagnostic delay Survey 2737 2739 3 09/25/23 20231101 NES 231101 Dear Editor, Pyoderma gangrenosum (PG) is among the most painful dermatologic conditions and pain is the most prominent symptom [[1], [4]]. This study describes patient experiences in reaching a PG diagnosis highlighting the impact of pain. While the impact of pain on PG treatment satisfaction has been studied, the impact of pain on other specific aspects of life has not been well-described [[3]]. [Extracted from the article]

Pyoderma gangrenosum triggered by COVID-19 vaccination in a patient with ulcerative colitis: a case report. Given the absence of any other significant medical history, the patient's relapse was believed to be triggered by the COVID-19 vaccination, and the patient chose not to receive the scheduled third dose. In this case report, we describe a patient with a history of PG, which had been in remission for several years prior to vaccination that experienced a recurrence of PG at the same sites as the previous involvement. [Extracted from the article]

The article presents a case study of a 38-year-old woman with hidradenitis suppurativa (HS) who, after being treated with the tumor necrosis factor α (TNF-α) inhibitor adalimumab, experienced two distinct paradoxical drug reactions psoriasis and pyoderma gangrenosum. It also provide insights into paradoxical drug reactions associated with TNF-α inhibitors, emphasizing the importance of early recognition and appropriate management of such reactions in patients with HS.

Https://doi.org/10.1093/bjd/ljad235 DearEditor, Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that is challenging to diagnose and manage. In this initial exploratory study, we used patient interviews to define QoL domains in PG that will inform this conceptual framework. At the time of the interview, three patients were in remission post-treatment, one was in remission on treatment and three had active disease. Our exploratory data endorse an interdisciplinary systematic approach to PG care, including pain management, psychological care and patient advocates. [Extracted from the article]

Introduction: Systemic inflammation leading to various types of disorders with mainly cutaneous manifestations is not uncommon. Hidradenitis suppurativa (HS) and pyoderma gangrenosum (PG) may be the consequences of such situation and develop concomitantly in a single patient. Case Presentation: We present herewith a case of HS along with PG which was managed with Adalimumab biosimilar. Discussion: HS and PG are both uncommon inflammatory skin conditions linked to systemic inflammatory disorders. Adalimumab, a tumor necrosis factor-alpha antagonist, is used to treat a variety of inflammatory disorders. Biosimilars are biologics that are extremely similar to an already-approved reference biological product but are produced by a distinct company. Several biosimilar variants of adalimumab have been developed and approved by regulatory authorities in various countries. They can be used as alternatives to the reference product, resulting in potential cost-savings and increased treatment accessibility. Conclusions: Biosimilars of adalimumab have an important role in the management of HS and PS considering their favorable outcome. [ABSTRACT FROM AUTHOR]

Scurvy is a clinical syndrome, resulting from ascorbic acid deficiency. Prevalence of the condition is now extremely low in the Western population and its diagnosis can be challenging without a high index of suspicion. When cases do present, they are often misdiagnosed initially. Therefore, a thorough history, physical exam, and laboratory evaluation are key to showing this now rare but extremely well-known disease. We report a case of scurvy manifesting as persistent non-healing lower-extremity ulcerations, initially mistaken for pyoderma gangrenosum. The patient responded to appropriate replacement therapy, but ulcers were slow to heal. As was the case in our patient, symptom reversal may require additional nutritional replacement. We encourage physicians to consider nutritional deficiencies in their differential diagnoses and highlight the incidence of malnutrition in the proper clinical setting to avoid diagnostic delay.

Granuloma annulare (GA) is a fairly common inflammatory skin condition with a range of clinical subtypes. We describe an unusual case of unilateral GA confined to the thigh on a previously amputated limb. A man in his 80s with a past medical history of below-knee amputation of the left leg owing to severe leg ulcers from pyoderma gangrenosum, chronic lymphocytic leukemia, and dyslipidemia developed a slowly spreading eruption on the distal stump spreading proximally. On physical examination, he had numerous non-scaly violaceous papules and annular plaques from the stump to the lateral, medial, and anterior thigh. Histology confirmed a diagnosis of GA. The extensive, chronic lesions make this presentation of GA very unusual in that it shares features of both localized and generalized forms. Moreover, the temporal and spatial association with pyoderma gangrenosum is unique and may reflect a related inflammatory pathway.

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Biopsy revealed a dense dermal infiltrate of neutrophils with negative tissue cultures consistent with pyoderma gangrenosum (PG). He was treated with systemic, intralesional, and topical steroids, as well as doxycycline. Three weeks after the diagnosis of PG, he was found to have persistent anemia and leukopenia. Bone marrow aspiration analysis was consistent with hypocellular myelodysplastic syndrome and genetic testing was consistent with Fanconi anemia. There is a well-known association of PG with hematological disorders. Fanconi anemia is a rare genetic hematologic disorder with congenital defects leading to bone marrow failure and malignancy in long-standing disease. In our patient, we consider his development of PG a paraneoplastic sign associated with the onset of his hypocellular myelodysplastic syndrome.

Pyoderma gangrenosum (PG) is a rare condition characterized by the development of aseptic, non-healing skin ulcers. Any skin trauma, such as a surgical incision, can trigger an outbreak of lesions. Our case and literature review show that a physician should consider PG in every event of a non-healing, aseptic wound after surgery. The treatment of PG requires collaboration within a multidisciplinary team and immunosuppressive therapy is the first line of treatment, while surgical interventions should be avoided in the active stage of PG.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis with unknown origin. Genetic factors, neutrophil dysfunction, complement system dysregulation and aberrant cytokine secretion play important parts in its pathogenesis. This article will make a review of the pathogenesis of the pyoderma gangrenosum from the aspects mentioned above.

Heng Zhang, Yifang Sun, Kun Li, Jianzhong Zhang, Xue Chen Department of Dermatology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of ChinaCorrespondence: Xue Chen, Department of Dermatology, Peking University People’s Hospital, # 11. Xizhimen South St, Beijing, 100044, People’s Republic of China, Tel +86 10 88325472, Fax +86 10 88325474, Email rmpkcx@163.comAbstract: Pyoderma gangrenosum (PG) is an inflammatory dermatosis characterized by the rapid progression of a painful, necrolytic ulcer with an irregular and undermined border. The prevalence of PG in patients with Crohn’s disease (CD) has been estimated to be 0.7%. Here, we report a case that presented various painful skin lesions, including erythema, vesicles, plaques, and ulcers, one week before the fourth infliximab infusion for CD. PG was diagnosed and the lesions subsided after a 390-mg ustekinumab infusion for one month. It suggests that different lesions of PG may occur concomitantly in CD patients, and the therapy should be re-evaluated on time.Keywords: pyoderma gangrenosum, Crohn’s disease, cutaneous manifestation

Ulcerative colitis (UC) may be associated with different extra-intestinal manifestations (EIM), which are often difficult to diagnose and treat, and may complicate the course of the disease. EIM are a multidisciplinary problem encountered by doctors of various specializations. However, many incidences of EIM in patients with UC remain unknown. The coexistence of UC and aseptic nasal abscess (ANA) is uncommon. Here, we describe two cases of ANA in young female patients with UC. ANA run in parallel with intestinal disease activity and led to necrosis of the septal cartilage. Moreover, pyoderma gangrenosum was described in one of them. Aseptic abscess syndrome should be kept in mind when a microbial factor is not identified, the diagnosis is not clear or a patient is not improving on appropriate antibacterial therapy. ANA can present as a rare complication of UC.

Liang Joo Leow,1– 3 Nicolas Zubrzycki1 1Aesthetic Dermatology, Sydney, NSW, Australia; 2Department of Dermatology, St Vincent’s Private Hospital, Sydney, NSW, Australia; 3St Vincent’s & Mater Clinical School, University of Notre Dame Australia, Sydney, NSW, AustraliaCorrespondence: Liang Joo Leow, Aesthetic Dermatology, 69 Burton Street, Darlinghurst, NSW, 2010, Australia, Tel +61 2 9188 2080, Email drleow@aestheticdermatology.com.auAbstract: Elevated levels of inflammatory mediators—including the interleukin IL-23—are implicated in the pathogenesis of pyoderma gangrenosum (PG), an autoinflammatory neutrophilic dermatosis characterized by rapidly enlarging, suppurative ulcers and cribriform scarring. Here, we present the first case report of significant response of isolated ulcerative PG with tildrakizumab, a biologic agent directed against the p19 subunit of IL-23, in an elderly woman with extensive treatment-refractory PG on her left leg. Tildrakizumab (100 mg subcutaneously at weeks 0 and 4, then every 8 weeks, and eventually increased in frequency to every 6 weeks), combined with acetic acid soaks each morning and chemical debridement every evening with 3% hydrogen peroxide, resulted in progressive decrease in ulcer size and depth, re-epithelialization, and recovery of sensory perception. This report describes the dramatic clinical response of ulcerative PG on the leg with tildrakizumab.Keywords: interleukin-23, leg ulcers, pyoderma gangrenosum, biological drug, wound dressing

Aortoiliac occlusive disease (AIOD) is non-inflammatory obstructive vasculopathy commonly affecting patients with advanced atherosclerosis, diabetes mellitus, or elevated cholesterol levels, and subjects with other risk factors such as cigarette smoking.Two Caucasian patients (a 55-year-old woman and a 56-year-old man), with ulcerous cutaneous lesions of AIOD are reported. In both cases, medical history comprises initial lower limb claudication, multiple painful ulcers along the legs and absence of superficial femoral artery pulse. Severe obstruction of both infrarenal aorta and iliac arteries on the left side was demonstrated by contrast angiography and Doppler ultrasonography.The evolution of the disease showed some characteristic findings, including pyoderma gangrenosum-like ulcerations as the initial cutaneous manifestation of AIOD, multiple painful ulcers along the lower extremities, and aorto-iliac occlusive disease due to atherosclerosis.Early diagnosis and surgical reconstruction of vessels in patients with AIOD improved quality of life and limb salvage rates.

Valéria Ferreira Martinelli,1,2 Pedro Martinelli Barbosa,3 Lucila Samara Dantas de Oliveira,1 Luísa de Andrade Lima Vieira de Melo,1 João Manoel Casa Nova,1 Carlos Alexandre Antunes de Brito2,4,5 1Department of Gastroenterology, Hospital das Clínicas, Federal University of Pernambuco, Recife, Pernambuco, Brazil; 2Department of Gastroenterology, Member of Organização Brasileira de Doença de Crohn e Retocolite – GEDIIB, São Paulo, Brazil; 3Department of Internal Medicine, Medical Sciences Center, Pernambucana of Health College, Recife, Pernambuco, Brazil; 4Department of Internal Medicine, Center of Medical Sciences of Federal University of Pernambuco, Pernambuco, Brazil; 5Department of Immunology, Autoimune Research Institute, Recife, Pernambuco, BrazilCorrespondence: Carlos Alexandre Antunes de Brito, Department of Immunology, Autoimune Research Institute, Avenue Rui Barbosa 715, Recife, Pernambuco, 52011-040, Brazil, Tel +55 81 31480101, Email cbritoc@gmail.comBackground: Cutaneous involvement is the second-most frequent extraintestinal manifestation of inflammatory bowel disease, with pyoderma gangrenosum (PG) a particularly relevant form because of its frequency, morbidity, and recurrence. The limited number of clinical trials involving PG increases the challenge to gastroenterologists in the management of this condition.Case Presentation: Four cases of atypical presentations of PG are reported. A 25-year-old patient with ulcerative colitis presented an extensive chronic ulcerative lesion on her left leg that was associated with significant bleeding; the intestinal disease was in remission under the use of azathioprine. The patient was on long-term use of 60 mg corticosteroid with no improvement in the skin disease; however, initiation of cyclosporine induced remission. In the second case, a 52-year-old woman was a carrier of Crohn’s disease, with a history of partial colectomy. The patient’s skin condition had evolved with a cutaneous lesion localized in the perineal region, buttocks, and colostomy pouch, simulating a case of impetigo, and this had been treated with antibiotic cycles without improvement. Lesion biopsy suggested a diagnosis of PG. Consequently, the patient was started on biological therapy with infliximab, and the PG regressed. In the third case, a 38-year-old woman with a history of pancolitis presented a picture of PG with an extensive and deep ulcerative lesion in the right breast. The lesion regressed after treatment with oral corticosteroid. The final case was a 44-year-old woman with Crohn’s disease suffering from Crohn’s disease pancolitis. The patient’s condition evolved with a mixed pattern with pustules, bullae, and ulcerative lesions in the vulva, oral cavity, gluteus, right auricular region, scalp, and left flank, and was resolved by administration of adalimumab.Conclusion: PG is an important and frequent manifestation of inflammatory bowel disease, with a spectrum of clinical variants, significant morbidity, and requiring a variety of therapeutic approaches.Keywords: inflammatory bowel disease, pyoderma gangrenosum, Crohn’s disease, ulcerative colitis, extraintestinal manifestations

The use of levamisole as the most frequent adulterant of cocaine has merged in previously unknown toxicities, notably a disease entity called cocaine/levamisole-associated autoimmune syndrome (CLAAS). Clinically, CLAAS can manifest with diverse cutaneous and extracutaneous features sharing common laboratory findings (neutropenia, autoantibody patterns). We report the case of a cocaine-abusing female patient with relapsing episodes of painful ulcers, worsening and expanding over a three-year period. The case exhibited all features of a drug-induced, skin-limited, ANCA-associated vasculitis, evolving over time to PG-like findings. In both disease stages, the patient responded well to the cessation of cocaine exposure and systemic glucocorticosteroids. This case demonstrates the continuous nature of cutaneous CLAAS manifestations in a single patient. CLAAS has become a major public health issue in the at-risk group of cocaine users, and clinicians should be alert of this condition when treating cocaine users presenting with single or multiple skin ulcerations.

This article discusses a case report of a 34-year-old female with chronic pyoderma gangrenosum (PG) and multi-drug-resistant pseudomonas infection. The patient had tried various topical and systemic treatments, including antibiotics, without success. Maggot debridement therapy (MDT) was then used to remove nonviable tissue and address microbial colonization. MDT resulted in a significant improvement in the wound, and subsequent negative pressure wound therapy (NPWT) further enhanced healing. The authors suggest that MDT can be considered as a safe and effective option for debridement in non-healing PG wounds with bacterial colonization or necrotic tissue. [Extracted from the article]

The article describes the case of a 40-year-old male with a 13-year history of stricturing colonic and perianal Crohn disease (CD). Topics discussed include his medical history, basis of the decision to use four weekly subcutaneous dosing, and finding on colonoscopy performed 24 months after commencing ustekinumab therapy.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory disease, clinically characterized by chronic and recurrent episodes of osteoarticular inflammation, that generally presents in children and adolescents. From a dermatological point-of-view, CMRO can be associated with skin rashes mainly including psoriasis, palmoplantar pustulosis and acne. Pyoderma gangrenosum (PG) is a rare immune-mediated inflammatory skin disease classified within the spectrum of neutrophilic dermatoses that, in some cases, has been reported as cutaneous manifestation in CMRO patients. This paper presents a 16-year female patient diagnosed with CMRO, who presented PG lesions located on the lower leg, that arose after the administration of the tumour necrosis factor (TNF)-α inhibitor adalimumab. Cases of PG have been reported in patients being treated with certain medications, including TNF-α antagonists, leading to classified them in a setting aptly termed “drug-induced PG.” In this paper, we discuss the co-occurrence of PG and CRMO, in the light of recent evidence on the pathogenesis of both diseases and giving ample space to a literature review on drug induced PG. In our case, it is plausible that PG could be considered a cutaneous manifestation of CRMO, although the mechanisms underlying this intriguingly relationship remain to be fully unraveled.

Introduction/Aims/Background: Pyoderma gangrenosum is an immune-mediated illness that can be caused by several affections, such as inflammatory bowel disease, rheumatoid arthritis, and drug use. We present a rare case of pyoderma gangrenosum induced by levamisole-adulterated cocaine. There have been few cases of this disease reported in the world. Levamisole is an anthelmintic drug used to adulterate cocaine to boost its effect. It also has immune-modulating effects causing, among others, vasculitis and dermatological problems. Materials and Methods: Clinical case of a 46-year-old man admitted to the hospital University Marqués de Valdecilla in Santander, Spain, in August 2022. We diagnosed pyoderma gangrenosum based on clinical, analytical, and histological parameters. Results: We report a case of pyoderma gangrenosum induced by consumption of levamisole-adulterated cocaine. Discussion: This patient suffered from a rare and extensive immune-mediated affection with characteristic primary lesions in the form of suppurative ulcers that responded to immunosuppressive treatment. Behind pyoderma gangrenosum there may be underlying conditions such as inflammatory bowel disease, or pyoderma gangrenosum may be secondary to identifiable causes such as cocaine use as in this patient.

Pyoderma gangrenosum (PG) is an auto-inflammatory dermatosis characterized by lesions that often cause ulcers. We present a case of successful ustekinumab treatment for acute general PG in a 31-year-old woman with coexisting Crohn's disease (CD). For a month, the patient suffered from skin ulcers, two of them deep and necrotic; a histopathological examination revealed PG. Treatment included: methylprednisolone, azathioprine, betamethasone, gentamicin and zincic ointments, antiseptic compresses and adalimumab therapy. Due to resistance to the implemented treatment, the patient was enrolled in a clinical trial that included the administration of a novel anti-cytokines drug - ustekinumab. Subsequently, a significant reduction was observed in the severity of symptoms of PG and CD, with no relapse. The use of ustekinumab in patients with PG who have an inadequate response to current treatment or cannot receive first-line treatment can be considered. This applies especially to patients with accompanying autoimmune diseases such as CD.

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. 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. Certolizumab may be a promising therapeutic option for patients with severe PG. [ABSTRACT FROM AUTHOR]

In dermatology, biologics that block signaling pathways of TNF‐α, IL‐4/IL13, IL‐17s, and IL‐23 are widely used for the treatment of several inflammatory skin diseases, such as atopic dermatitis and psoriasis. They have shown excellent efficacy with an acceptable safety profile. However, these biologics targeting pathogenic cytokines and their receptors could modulate immunological balance, leading to the development of other inflammatory or autoimmune skin diseases in some cases. In this study, we present a patient who suffered pemphigus vegetans and showed an exacerbation of pemphigus foliaceus after secukinumab loading for the treatment of complicated generalized pustular psoriasis and pyoderma gangrenosum. [ABSTRACT FROM AUTHOR]

Background: Acute febrile neutrophilic dermatosis (Sweet syndrome) is a potentially fatal multiorgan inflammatory disease characterized by fever, leukocytosis, and a rash with a neutrophilic infiltrate. Disease pathophysiology remains elusive, and current dogma suggests Sweet syndrome is a "reactive" process to an unknown antigen. Corticosteroids and steroid-sparing agents remain front-line therapies, but refractory cases pose a clinical challenge.Methods: A 51-year-old woman with multiorgan Sweet syndrome developed serious corticosteroid-related side effects and was refractory to steroid-sparing agents. Blood counts, liver enzymes, and skin histopathology supported the diagnosis. Whole genome sequencing, transcriptomic profiling, and cellular assays of patient's skin and neutrophils were performed.Results: We identified elevated IL-1 signaling in lesional Sweet syndrome skin caused by a PIK3R1 gain-of-function mutation specifically found in neutrophils. This mutation increased neutrophil migration towards IL-1β and neutrophil respiratory burst. Targeted treatment with an IL-1R1 antagonist in the patient resulted in a dramatic therapeutic response and enabled tapering of corticosteroids.Conclusions: Dysregulated PI3K-AKT signaling is the first signaling pathway linked to Sweet syndrome and suggests Sweet syndrome may be caused by acquired mutations that modulate neutrophil function. Moreover, integration of molecular data across multiple levels identified a distinct subtype within a heterogenous disease that resulted in a rational and successful clinical intervention. Future cases will benefit from efforts to identify potential mutations. The ability to directly interrogate diseased skin allows this method to be generalizable to other inflammatory diseases and demonstrates a potential personalized medicine approach for challenging patients.FUNDING Berstein Foundation, NIH, VA, Moseley Foundation, and H.T. Leung Foundation. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum is an inflammatory skin disease characterized by the formation of deep, painful ulcers that may heal with prominent scarring. This condition is classified as a neutrophilic disorder, which means it involves an exaggerated response from immune cells called neutrophils. Next, we will describe a clinical case of pyoderma gangrenosum that illustrates the typical characteristics of this disease. For its diagnosis, it was important to perform a biopsy of the lesions and their treatment. It is important to keep in mind that the disease can affect not only the physical health of the patient, but also her mental and emotional health. [ABSTRACT FROM AUTHOR]

Background: Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum. Aims: We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum. Methods: In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999–2019. Results: Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0–9.0months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence. Limitations: This study was a single-centre study with a retrospective design. Conclusion: Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum. [ABSTRACT FROM AUTHOR]

Introduction: Pyoderma gangrenosum is a rare dermatosis characterised by a rapid course and uncertain prognosis. It poses a major therautic challenge in the daily dermatological practice, also to experienced medical practitioners. Pyoderma gangrenosum may coexist with autoimmune diseases, but it can also a paraneoplatic diseease associated with cancers of internal organs and myeloproliferative diseases. Case report: We present a 78-year-old woman with pyoderma gangrenosum hospitalised in the Clinical Department of Dermatology and to review the literature on the coexistence of pyoderma gangrenosum and neoplastic deseases of internal organ cancers. In our patient pyoderma gangrenosum was associated with duodenal thickening observed on computer tomography combined with a positive test for occult blood in the stool. It led to the suspicion of gastrointestinal malignancy. However the patient refused further diagnostic procedures. Conclusions: Multiple case studies and review articles point to a statistically significantly increased frequency of neoplatic diseases (both hematologic malignanciues and solid tumours) in patients with pyoderma Thus, a reasonable strategy is to perform cancer screening in patients with pyoderma gangrenosum. Not all patients consent to expand the diagnostic procedures to confirm or exclude the suspicion of a neoplastic disease. [ABSTRACT FROM AUTHOR]

Background: Pyoderma gangrenosum (PG) is a reactive, noninfectious, neutrophilic dermatosis. Diagnosis of PG is based on exclusion, due to lack of availability of a confirmatory test. PG is not caused by infection or gangrene. Misdiagnosis or delayed diagnosis of PG can lead to devastating results. Case Presentation: In this report, we present a patient with a delayed diagnosis of PG lesion on right hand. Despite initial surgical treatment, the wound was aggravated, and amputation was considered; however, it was eventually treated successfully with an autologous split thickness skin graft. Conclusions: Knowledge of the PG is essential to actively consider PG in early stage to help facilitate immediate treatment and avoid unnecessary interventions that may worsen the outcome. [ABSTRACT FROM AUTHOR]

Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune/inflammatory condition. Acute myeloid leukaemia was the most common malignancy (16/93) followed by myelodysplastic syndrome (7/93). Patients with acute myeloid leukaemia presented isolated flares, marked cytopaenia and rapid response to treatment, whereas myelodysplastic syndrome followed a chronic-recurrent course. The most frequent associated medications and inflammatory disorders were filgrastim and hydroxyurea (n=2); and inflammatory bowel disease (n=4). In addition, 3 patients were diagnosed with VEXAS syndrome. Male sex (p=0.006), fever (p=0.034), increased erythrocyte sedimentation rate (p<0.001), anaemia (p<0.001), and thrombocytopaenia (p<0.001) were associated with malignancy. Histologically, patients were classified as classic (60%), histiocytoid (22.5%) or subcutaneous (15%), with pain (p=0.011) and nodules (p<0.001) being associated with subcutaneous-Sweet syndrome. Sweet syndrome in the context of cytopaenia should alert the presence of malignancy. An acquired autoinflammatory condition should be explored in relapsing Sweet syndrome with myelodysplastic syndrome. A minimum follow-up of 6 months is recommended. [ABSTRACT FROM AUTHOR]

The early clinical manifestations of pyoderma gangrenosum are not characteristic, it resembles other infectious skin lesions and is difficult to identify in the early stages. The exploratory surgery for direct observation of the superficial fascia of the suspected infection site for signs of infection, together with the collection of tissue samples for bacterial culture, combined with histopathological biopsy and clinical manifestations, was of significant value in the differential diagnosis of this disease and infectious skin lesions with similar manifestations. We introduce a patient who was admitted to hospital with redness and swelling of the left calf and foot for 7 days. The local appearance of the lesion resembled a carbuncle or early-stage necrotizing fasciitis. We performed surgical exploration for a definitive diagnosis but found no signs of infection, therefore, infection could be preliminarily ruled out. The disease was finally diagnosed as pyoderma gangrenosum, which was treated with hormone therapy and recovered after dressing change. [ABSTRACT FROM AUTHOR]

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

With advances in drug development and our understanding of the pathophysiology of skin disease, biologic medications have emerged as powerful management tools for dermatologists. While biologics have most often been used in the management of psoriasis, they are being used off-label for the management of a variety of other immune-mediated skin diseases with overlapping molecular targets. This narrative review focuses on the novel and off-label use of biologic medications for the management of hidradenitis suppurativa (HS), pyoderma gangrenosum (PG), lichen planus (LP), and seborrheic dermatitis (SD). Review of the literature revealed that IL-17, IL-23, and tumor necrosis factor (TNF) inhibitors were being used across a variety of immune-mediated skin pathologies with variable efficacy, among other targeted biologics. While biologics were generally safe in the treatment of primary immune-mediated skin disorders, paradoxical disease eruptions were noted with biologic use and were theorized to occur owing to immune dysregulation and cytokine imbalance. While numerous case reports show promise for the use of biologics in immune-mediated skin pathologies, the variable efficacy and safety reported warrants more thorough investigations of the role of these targeted medications in comprehensive disease management. [ABSTRACT FROM AUTHOR]