Clinical efficacy of crushed prednisolone and hydrocolloid powder in the primary treatment of peristomal pyoderma gangrenosum and correlation to in vitro drug release data.

We evaluated the primary application of crushed prednisolone combined with hydrocolloid powder for clinically diagnosed peristomal pyoderma gangrenosum (PPG). We present our data on this cohort and follow-up of our previous patients. Of the 23 patients who were commenced on this regime, 18 healed (78%). Twenty-two patients commenced on this regime as the primary treatment for their PPG, and for one, it was a rescue remedy after failed conventional therapy. Four patients with significant medical comorbidities failed to heal and one had their stomal reversal surgery before being fully healed. The proposed treatment regime for PPG is demonstrated to be effective, inexpensive and able to be managed in the patient's usual home environment. In vitro drug release analysis was undertaken, and data are presented to provide further insights into the efficacy of this regime.

Successful treatment of PASS syndrome with IVIG and anti-IL-1 treatment: A case report.

PASS syndrome is a rare autoinflammatory disease characterized by acne vulgaris, hidradenitis suppurativa, pyoderma gangrenosum, and ankylosing spondylitis. Unlike other autoinflammatory disorders such as PAPA and PASH syndrome, there is no documented gene mutation link. Although there are no established treatment guidelines due to the rarity of these diseases, systemic corticosteroids, biologics, and immunosuppressive drugs are used currently. In our report, we presented a case of PASS syndrome who was unresponsive to adalimumab and in whom we observed improvement in both skin and joint manifestations with intravenous immunoglobulin (IVIG) and anti-IL-1 treatment.

Deep Vein Thrombosis and Healing Outcomes in Patients With Pyoderma Gangrenosum.

This single-center prospective case-control study assessed the association between deep vein thrombosis and healing outcomes in patients with pyoderma gangrenosum.

Postoperative and Peristomal Pyoderma Gangrenosum: Subtypes of Pyoderma Gangrenosum.

Postoperative pyoderma gangrenosum and peristomal pyoderma gangrenosum are 2 subtypes of pyoderma gangrenosum. The diagnosis is made as a clinicopathologic correlation when assessing a rapidly progressing ulcer with irregular and undermined borders following a surgical procedure, trauma, or the creation of a stoma. Familiarity with the associated risk factors and distinguishing features of these disorders can facilitate prompt recognition, proper diagnosis, and the initiation of treatment. Management usually involves the use of corticosteroids and steroid-sparing agents as immunomodulators to shift the inflammatory neutrophilic dermatoses to chronic noninflammatory wounds and eventual healing.

The Neutrophilic Dermatoses, or the Cutaneous Expressions of Neutrophilic Inflammation.

Acute febrile neutrophilic dermatosis, or Sweet syndrome, has been described in 1964 and is now considered as a prototypical condition of the group of the neutrophilic dermatoses. Since this time, many clinical conditions have been included in this group and a clinical-pathological classification in 3 subgroups has been proposed. Neutrophilic infiltrates can localize in all internal organs. This defines the neutrophilic disease, which induces difficult diagnostic and therapeutic problems. Autoinflammation is the main pathophysiological mechanism of the neutrophilic dermatoses. There is a special link between myeloid malignancies (leukemia and myelodysplasia) and the neutrophilic dermatoses.

Treatment of Pyoderma Gangrenosum.

Pyoderma gangrenosum is a rare neutrophilic dermatosis that results in painful cutaneous ulcers and is frequently associated with underlying hematologic disorders, inflammatory bowel disease, or other autoimmune disorders. Pathogenesis involves an imbalance between proinflammatory and anti-inflammatory mediators, leading to tissue damage from neutrophils. First-line treatment options with the greatest evidence include systemic corticosteroids, cyclosporine, and tumor necrosis factor alpha inhibitors. Other steroid-sparing therapies such as dapsone, mycophenolate mofetil, intravenous immunoglobulin, and targeted biologic or small molecule inhibitors also have evidence supporting their use. Wound care and management of underlying associated disorders are critical parts of the treatment regimen.

Pediatric Neutrophilic Dermatoses.

The term neutrophilic dermatosis encompasses a heterogeneous group of diseases, often associated with an underlying internal noninfectious disease, with an overlapping histopathologic background characterized by perivascular and diffuse neutrophilic infiltrates in one or more layers of the skin; extracutaneous neutrophilic infiltrates may be associated. Neutrophilic dermatoses are not frequent in children and, when they appear in this age group, represent a diagnostic and therapeutic challenge. Apart from the classic neutrophilic dermatoses such as pyoderma gangrenosum, Sweet syndrome, and Behçet disease, a neutrophilic dermatosis can be the presentation of rare genetic diseases of the innate immune system, such as autoinflammatory diseases.

The Pathophysiology and Treatment of Pyoderma Gangrenosum-Current Options and New Perspectives.

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.

Relapse of Ulcerative Colitis with Immune Thrombocytopenia and Pyoderma Gangrenosum Subsequent to Receiving COVID-19 Vaccination.

This case illustrates the complex interactions of the immune responses after vaccination and highlights their potential connections to various autoimmune conditions. A 22-year-old man with quiescent ulcerative colitis (UC) presented with abdominal pain, rectal bleeding, and thrombocytopenia 7 days after receiving the third coronavirus disease 2019 mRNA vaccination. Laboratory data confirmed the diagnosis of immune thrombocytopenia. High-dose intravenous immunoglobulin administration boosted the patient's platelet count. Simultaneously, colonoscopy revealed that his UC had relapsed. Although salazosulfapyridine briefly improved his symptoms, his stool frequency worsened one week later. The patient also developed pyoderma gangrenosum. Subsequent treatment with infliximab notably improved both pyoderma gangrenosum and UC.

Pediatric pyoderma gangrenosum with pulmonary involvement: A case report.

Pyoderma gangrenosum is a rare disease in childhood. Extra-cutaneous manifestations are uncommon in pyoderma gangrenosum, even more so in children, with only a few cases reported in the literature. We present the case of a pediatric patient with pyoderma gangrenosum and associated pulmonary involvement. In this case, the diagnosis was delayed leading to late initiation of therapy, emphasizing the importance of maintaining a high level of suspicion for this diagnosis.

Tofacitinib in pyoderma gangrenosum - A case series.

Pyoderma gangrenosum (PG) is a rare autoinflammatory disorder falling under the spectrum of neutrophilic dermatosis, characterized by distinctive skin ulceration which is non-infective, non-neoplastic and usually with no primary vasculitis. PG lesions are notorious for relapse and hence require multiple trials of medications often with prolonged and concomitant use of steroids. Due to lack of evidence-based studies on effective treatment options for PG, we have presented three isolated biopsy-proven PG cases who were successfully treated with Tofacitinib, a Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway inhibitor, without relapse in follow up.

Evaluation of Inclusion and Exclusion Criteria for Pyoderma Gangrenosum in Clinical Research: A Systematic Review.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, inflammatory dermatologic disease that, as a diagnosis of exclusion with nonspecific histologic features, is difficult to diagnose. As pharmaceutical interest in potential treatments for PG increases, the need for standardized diagnostic criteria to ensure reproducibility, comparability, and external validity of PG research is required. In this study, we aim to characterize the inclusion and exclusion criteria used in the diagnosis of PG in clinical research studies as well as the eligibility of PG in clinical trials. METHODS: A systematic review was conducted to characterize the PG inclusion and exclusion criteria in research studies. An additional search of the USA and international clinical trials databases was conducted as well to capture eligibility criteria for PG trials. RESULTS: Our study revealed a broad range of inclusion and exclusion criteria used to establish the presence or absence of PG. Based on eight distinct categories used to characterize inclusion criteria for research studies, diagnosis by a dermatologist (n = 25, 31.6%), no inclusion criteria listed (n = 21, 26.6%), and clinical and histopathologic features consistent with PG (n = 20, 25.3%) were most common. For current clinical trials, six categories were used to characterize inclusion criteria, of which clinical and histopathologic features consistent with PG (n = 5, 31.3%), identification based on diagnosis of PG (n = 4, 25.0%), and clinical features consistent with PG (n = 3, 18.8%) were the most common. CONCLUSION: This systematic literature review highlights the range of heterogeneity in diagnostic and eligibility criteria used in PG-directed clinical research and current clinical trials and illustrates the need for the development of consensus guidelines and a rigorous framework to enable high-quality future trials for PG.