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.

Pyoderma gangrenosum complicated with hematological malignancies: Two case reports.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic skin disease. The diagnosis of PG is mainly based on clinical manifestations. Therefore, the clinical features of PG are important for confirming the diagnosis of this disease. Herein, the clinical data of 2 young males with PG complicated with hematological malignancies were reported, and the literature were reviewed. CASE PRESENTATION: The first case was a 22-year-old male who was admitted due to a systemic rash, headache, and fever. Physical examination showed black scabs on the skins of the extremities, trunk, scalp, and face. Biopsy of the skin lesion showed epidermal edema, spongy formation, neutrophil infiltration, acute and chronic inflammatory cell infiltration in the dermis, showing purulent inflammation with epidermal erosion. The bone marrow biopsy showed obviously active proliferation of nucleated cells, granulocytes at various stages, abnormal morphological neutrophils, and occasionally observed young red blood cells. The diagnosis of PG and chronic myelomonocytic leukemia (CMML-0) was made. The second case was a 28-year-old male who presented a swollen, painful right calf following injury and then developed ulcers on skin and soft tissues. Bone marrow biopsy showed obviously active nucleated cell proliferation, suggesting a myeloid tumor. He was also diagnosed with PG and hematological malignancies. They both received hormone and antiinfection therapy. After treatment, their body temperature, infection, and skin lesions were improved. However, both of them were readmitted and had a poor prognosis. CONCLUSIONS: PG may be associated with hematological malignancies. For patients with typical skin lesions and obvious abnormal blood routines, it is necessary to investigate the possibility of PG with hematological malignancies.

Doxycycline monotherapy for pyoderma vegetans: a case report.

Background: Pyoderma vegetans (PV) is a rare neutrophilic dermatosis of unknown etiology. Currently, there are no treatment guidelines for PV. Systemic steroids are often used as first-line therapy, but recurrence upon discontinuation or tapering is common.Materials and methods: We tested the efficacy of doxycycline at a dose of 200 mg/d to treat resistant PV.Results: After 4 weeks of treatment we noticed a significant improvement in the clinical appearance of PV.Conclusions: Our case demonstrates the potential utility of doxycycline as a systemic steroid-sparing agent in the treatment of PV.

A Rare Coexistence: Cutaneous Lobular Carcinoma with Pyoderma Gangrenosum.

BACKGROUND Pyoderma gangrenosum (PG) is a neutrophilic inflammatory disease associated with inflammatory and autoimmune conditions and malignancies. Previously identified links between PG and cancer have included hematological malignancies, solid-organ tumors such as gastric adenocarcinoma, and breast cancer. While specific histologic subtypes of breast cancer such as ductal carcinoma have been associated with PG of the extremities, the literature is limited on the association between PG and cutaneous metastatic lobular carcinoma. CASE REPORT We describe the case of an 84-year-old woman with recurrent cutaneous metastatic lobular carcinoma of the left anterior chest with concurrent pyoderma gangrenosum on her bilateral lower extremities. The patient was initially diagnosed with lobular carcinoma of the breast and underwent a left breast mastectomy and was in remission. One year later, she developed 2 lower-extremity ulcerations, which at the time were attributed to an injury and underlying venous stasis. She was referred to a wound care clinic, where the lesions worsened with surgical debridement. Six years later, she presented to the dermatology clinic with a rash on her chest wall and worsening of the ulcerations on her ankles bilaterally. Biopsies revealed lobular carcinoma metastatic to the skin of her anterior chest wall and histopathology consistent with pyoderma gangrenosum on her ankles. CONCLUSIONS This case demonstrates a unique presentation of worsening pyoderma gangrenosum due to metastatic malignancy in conjunction with a cutaneous manifestation of lobular carcinoma.

Concurrent pyoderma gangrenosum and Takayasu arteritis in an infant: diagnostic challenges and treatment considerations.

BACKGROUND: Takayasu arteritis (TA) is an uncommon chronic inflammatory and autoimmune disease primarily affecting large vessels, particularly the aorta and its branches. Skin manifestations have been documented in association with TA. Pyoderma gangrenosum (PG) is a chronic neutrophilic dermatosis characterized by destructive, necrotizing, and painful ulcers, predominantly found on the lower extremities. The coexistence of PG and TA is extremely rare, with most reported cases involving adult patients. Interestingly, the association between PG and TA appears to be more common in Japan compared to North American and European populations. Childhood TA (c-TA) accompanied by PG is exceptionally rare, with only 10 cases reported in the literature thus far. CASE REPORT: We present the case of a 7-month-old patient initially diagnosed with PG. Despite aggressive immunosuppressive therapy, the patient`s high acute phase reactants remained elevated. Although the abdominal ultrasound was normal, advanced imaging was performed due to severe abdominal pain. Contrastenhanced computerized tomography angiography of the aorta and its branches revealed extensive vascular involvement consistent with TA. CONCLUSION: In this report, we highlight an infantile case of PG that was subsequently diagnosed as infantile TA. Recognizing the rare association between PG and TA is important. Thorough evaluation and prompt diagnosis of TA in infants with PG can guide further investigations and prevent vascular complications.

Perioperative management and clinical outcomes of peristomal pyoderma gangrenosum.

Peristomal pyoderma gangrenosum is an uncommon subtype of pyoderma gangrenosum mainly affecting stoma sites of patients with inflammatory bowel disease. While surgical treatments are often used to assist healing, little is known about the relationship between surgical interventions and the rate of recurrence of peristomal pyoderma gangrenosum. The aim of this study was to identify patient and clinical factors associated with peristomal pyoderma gangrenosum recurrence following surgical intervention. A multi-institutional retrospective case series and literature review was conducted to evaluate patient characteristics and perioperative treatment. Patients of any age with peristomal pyoderma gangrenosum undergoing surgical operations related to their pyoderma gangrenosum or due to another comorbidity were included. Descriptive statistics were used to characterize demographic information. Associations were evaluated using Wilcoxon's rank-sum test for continuous variables and Fisher's exact test for categorical data. Thirty-seven cases were included, 78.3% of which had a history of inflammatory bowel disease. Overall, 13 (35.1%) cases experienced recurrence at 30 days. There was no significant association identified between patient demographics, stoma location, surgical intervention, or perioperative treatment with rate of recurrence at 30 days post-operation. While no clinical risk factors or treatments were associated with recurrence, our work underscores the importance of a multidisciplinary approach to this disease to address gastrointestinal, dermatologic, and surgical components of 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.

Pyoderma Gangrenosum: Diagnostic Criteria, Subtypes, Systemic Associations, and Workup.

Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis with variable clinical features. The classic presentation is an ulceration with an erythematous to violaceous undermined border. Extracutaneous manifestations may occur. Associated systemic diseases include inflammatory bowel disease, inflammatory arthritides, and hematologic disorders. The pathophysiologic mechanism of disease is not completely known but likely related to the cumulative impact of inflammation, immune-mediated neutrophilic dysfunction, and genetic predisposition. Incidence is between 3 and 10 people per million but may be greater due to under recognition. In this article, we will discuss the diagnostic criteria, disease subtypes, systemic associations, and workup.

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.