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.

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.

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.

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.

Autoimmune, Autoinflammatory Disease and Cutaneous Malignancy Associations with Hidradenitis Suppurativa: A Cross-Sectional Study.

BACKGROUND: Hidradenitis suppurativa (HS) is a debilitating cutaneous disease characterized by severe painful inflammatory nodules/abscesses. At present, data regarding the epidemiology and pathophysiology of this disease are limited. OBJECTIVE: To define the prevalence and comorbidity associations of HS. METHODS: This was a cross-sectional study of EPICTM Cosmos© examining over 180 million US patients. Prevalences were calculated by demographic and odds ratios (OR) and identified comorbidity correlations. RESULTS: All examined metabolism-related, psychological, and autoimmune/autoinflammatory (AI) diseases correlated with HS. The strongest associations were with pyoderma gangrenosum [OR 26.56; confidence interval (CI): 24.98-28.23], Down syndrome (OR 11.31; CI 10.93-11.70), and polycystic ovarian syndrome (OR 11.24; CI 11.09-11.38). Novel AI associations were found between HS and lupus (OR 6.60; CI 6.26-6.94) and multiple sclerosis (MS; OR 2.38; CI 2.29-2.48). Cutaneous malignancies were largely not associated in the unsegmented cohort; however, among Black patients, novel associations with melanoma (OR 2.39; CI 1.86-3.08) and basal cell carcinoma (OR 2.69; CI 2.15-3.36) were identified. LIMITATIONS: International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy. CONCLUSION: This is the first study to identify correlations between HS with melanoma and basal cell carcinoma (BCC) among Black patients as well as MS and lupus in all patients with HS.

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.

Mortality and Autopsy Findings in Patients with Pyoderma Gangrenosum: A Multi-Institutional Series.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with an increased risk of mortality compared to the general population. The causes of this increased risk are not well understood. Misdiagnosis is common in PG, and many studies are limited by the inclusion of misdiagnosed cases. The goal of this study was to review autopsy findings, identify causes of death, and identify factors that may worsen outcomes among deceased patients confirmed to have PG. METHODS: Data was retrospectively reviewed from the electronic medical records at five academic hospitals. A search was conducted for deceased patients with a diagnosis of PG who had an autopsy performed between 2010 and 2020. We report a descriptive analysis of 11 patients and their clinical characteristics, causes of death, and autopsy findings. RESULTS: The average age of death was 62.9 years. Seven patients had at least one underlying condition known to be associated with PG including inflammatory bowel disease, inflammatory arthritis, or a hematologic disorder. The most common cause of death was infection (n = 6, 54.5%), followed by pulmonary embolism (n = 3, 27.3%), and myelodysplastic syndrome (n = 2, 18.2%). Six patients (54.5%) were taking systemic steroids at the time of death. CONCLUSION: The development of PG may shorten life expectancy among those with underlying conditions associated with PG, and common treatments for PG may contribute to the risk of fatal complications. Awareness of the risk of infection, thrombosis, and malignancy among those with PG is necessary for proper management. Further research is needed to explore the relationship between PG and thromboembolism.

Prevalence of Extraintestinal Manifestations in Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.

BACKGROUND: Inflammatory bowel disease (IBD) is a multisystem disease impacting various body systems including musculoskeletal, ocular, skin, hepatobiliary, pulmonary, cardiac, and haematological systems. The extraintestinal manifestations of IBD are frequent, common in both ulcerative colitis (UC) and Crohn's disease (CD), and impact the morbidity and mortality of patients. METHODS: The Embase, Embase classic, and PubMed databases were searched between January 1979 and December 2021. A random effects model was performed to find the pooled prevalence of joint, ocular, and skin extraintestinal manifestations of UC and CD. RESULTS: Fifty-two studies were included that reported on 352 454 patients. The prevalence of at least 1 joint, ocular, or skin extraintestinal manifestation in all IBD, UC, and CD was 24%, 27%, and 35% respectively. The prevalence between UC and CD were similar for pyoderma gangrenosum and axial joint manifestations. Ocular manifestations were found to be more common in CD than in UC. Peripheral joint manifestations and erythema nodosum were found to be more common in CD than UC. DISCUSSION: To our knowledge, this is the first meta-analysis that reports on the prevalence of at least 1 joint, ocular, or skin extraintestinal manifestation in IBD. Our results are largely consistent with figures and statements quoted in the literature. However, our findings are based on significantly larger cohort sizes. Thus, our results have the potential to better power studies and more accurately counsel patients.

The prevalence of joint, ocular, or skin extraintestinal manifestations in IBD, UC, and CD was 24%, 27%, and 35% respectively. Ocular manifestations were more common in CD. Peripheral joint manifestations and erythema nodosum were more common in CD.

New treatment of pyoderma gangrenosum and hidradenitis suppurativa: A review.

Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are stubborn inflammatory skin diseases categorized as neutrophilic hypodermal dermatoses. These conditions exhibit connections with other autoinflammatory disorders driven by immune responses. Their pathogenesis is complex, rooted in significant imbalances in both innate and adaptive immune systems, particularly featuring elevated levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-8, IL-17, and IL-23. Studies involving skin tissue pathology and serology have indicated that targeting specific cytokines can bring therapeutic benefits. Indeed, many patients in clinical settings have responded positively to such interventions. Yet, given the diverse cytokines in play, focusing on a single one with antibody therapy might not always be effective. When resistance to biologics emerges, a combined approach targeting multiple overactive cytokines with immunosuppressants, for example cyclosporine and Janus kinase inhibitors, could be an option. In the current review, we explore recent therapeutic developments for PG and HS.

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.

Pyoderma gangrenosum and impact on quality of life: A narrative review.

Pyoderma gangrenosum (PG) is an autoinflammatory disorder typically characterized by progressive ulcers with dense neutrophilic infiltrates in the absence of infectious causes. The chronic nature of this disease significantly impacts the patients' quality of life (QoL). Yet there is currently a dearth of information in the literature regarding standardised treatment guidelines and the impact of PG on patients' QoL. We conducted a literature search on PubMed using the terms "pyoderma gangrenosum" AND "quality of life." We identified nine relevant articles that provide insight into which domains are affected and what treatment can improve QoL. The most common domains involved are physical, emotional, and psychological. Patients tend to feel depressed/anxious, isolated, and embarrassed secondary to PG manifestations. Comorbidities such as Crohn's disease, monoclonal gammopathy of dermatologic significance, and ulcerative colitis can worsen the impact on these patients' QoL. Pain is also a significant contributor to decreasing patients' QoL. Treatments such as topical steroids, adalimumab, and canakinumab may help improve QoL scores. We believe this information can help clinicians guide the care of patients with PG and highlight the need for more studies and clinical trials focusing on PG treatments' impact on QoL.

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.

Pyoderma gangrenosum following reduction mammoplasty: Systematic review of the literature and case report.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis that can develop at a surgical site. Diagnosis can be challenging at its presentation causing delays in appropriate treatment. The aim of this study is to review the current literature as well as to describe the clinical presentation, diagnostic pathway, and treatment of PG after reduction mammaplasty in order to define a standardized multidisciplinary diagnostic and therapeutic approach. In the future, this may ease early identification and prompt treatment, and eventually minimize severe morbidity and long-term sequelae. METHODS: The entire PubMed/Medline database was screened following the PRISMA guidelines to identify studies describing PG that have occurred after reduction mammoplasty. RESULTS: Twenty-eight articles including 31 patients reported a PG after breast reduction surgery between January 1988 and March 2022. Twenty-one (68%) patients presented with skin ulcerations, 14 (45%) with erythema, and 5 (16%) with vesicles. Out of the 30 cases that underwent bilateral surgery, 18 (60%) developed PG bilaterally. In 12 out of 31 patients, nipple-areolar complex (NAC) involvement was evaluated, though in 10 patients (83%) the NAC was spared. Of the 20 patients (65%) who underwent skin biopsies for histopathological examination, 18 (90%) showed neutrophilic infiltration of the dermal layers. All 31 patients (100%) showed rapid clinical improvement after the introduction of immunosuppressive therapy. CONCLUSIONS: PG can result in devastating skin alterations also after reduction mammoplasty, if misdiagnosed. However, it presents with constant yet unspecific local and general signs and symptoms that can be recognized to early initiate an appropriate pharmacological treatment.