Ulcerative Pyoderma Gangrenosum Research Articles

Ulcerated, tender nodules of the lower extremities

Ulcerated, tender nodules of the lower extremities

Pyoderma gangrenosum: examination and treatment experience

BACKGROUND: Pyoderma gangrenosum is a rare inflammatory skin disease that currently belongs to the group of neutrophilic dermatoses.
 AIMS: Development of epidemiological, clinical, laboratory characteristics of patients with pyoderma gangrenosum, as well as the treatment of this disease.
 MATERIALS AND METHODS: The study revealed 30 patients, 16 (53%) were women, the average age at the time of diagnosis was 5916.3 years. The most common localization of rashes was the lower leg 20 patients (67%), lesions of the skin of the face, genitals, and breast rarely noted, in one case, respectively. In 14 patients (47%), two or more ulcers were detected at the same time (max. 9 ulcers). The pathergy phenomenon was positive in 23 patients (77%) of 30. Ulcerative pyoderma gangrenosum occurred in 25 (83%), and extracutaneous pyoderma gangrenosum with lung involvement was identified in one patient. The most common associated disease was rheumatoid arthritis in 6 patients (20%), there were also rare associated diseases, such as hepatitis C two cases, multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, non-Hodgkins lymphoma by one observation. Histological examination revealed neutrophilic infiltration of the dermis in 30 patients (100%), and the presence of leukocytoclastic vasculitis in 16 (53%). Complete scarring during the treatment was observed in 22 patients (73%). Relapses during the observation period were observed in 12 patients (40%), the number of deaths was two cases.
 CONCLUSIONS: One of the largest case series of pyoderma gangrenosum to date is presented. During the examination of patients, it was found that neutrophilic infiltration of the dermis is a characteristic sign of the disease. Rare comorbidities (multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, hepatitis C, non-Hodgkins lymphoma) have been found. A sufficiently large percentage of relapses indicates the need for further research to develop an additional examination method for the purpose of modern diagnostics and justification for the timely prescription of targeted therapy.

Clinical manifestations and treatment outcomes of pyoderma gangrenosum following rituximab exposure: A systematic review

Clinical manifestations and treatment outcomes of pyoderma gangrenosum following rituximab exposure: A systematic review

Health-related domains of quality of life in pyoderma gangrenosum: A qualitative analysis

Health-related domains of quality of life in pyoderma gangrenosum: A qualitative analysis

Treatment of pyoderma gangrenosum: A multicenter survey‐based study assessing satisfaction and quality of life

Pyoderma gangrenosum (PG) lacks consensus regarding treatment, and no prior studies assess treatment satisfaction in PG. The objective of this study was to determine patient-reported satisfaction in the treatment of PG, and associations with satisfaction. Methodology was a multicenter cross-sectional survey for patients who received systemic medication(s) to treat PG. Thirty-five patients completed the survey (mean age: 54.0 years, 65.7% female, response rate: 81.4%). Mean (± SD) SATMED-Q score was 75.0 (±16.2, range: 67.6-85.3). Older patients (72.6 ± 23.6 for 18-39 years, 74.4 ± 16.1 for 40-59, 77.1 ± 11.6 for 60+), plus those with higher incomes (72.9 ± 20.3 for $0-49 000; 74.0 ± 17.6 for $50 000-99 000; 79.0 ± 14.6 for $100 000+) and education status (69.4 ± 14.3 for high school equivalent, 72.9 ± 15.9 for undergraduate, 91.7 ± 10.6 for graduate), were more satisfied with treatment. Ulcerative PG had higher SATMED-Q scores (79.0 ± 13.2) than other subtypes (66.2 ± 19.3). For local therapy, wound care, or pain control, 63.2%, 100%, and 75% were satisfied, respectively. The mean DLQI was 8.6 (±7.6, range: 0-29), and higher DLQI was associated with decreased satisfaction. Satisfaction with providers was positively correlated with global satisfaction (Pearson's r = 0.638). The presence of pain and/or depression influenced both SATMED-Q (72.8 ± 18.8 with pain, 78.3 ± 11.2 without; 68.2 ± 18.8 with depression, 80.1 ± 12.2 without) and DLQI scores (12.1 ± 8.1 with pain, 3.9 ± 3.4 without; 10.3 ± 7.1 with depression, 7.4 ± 8.0 without). To optimize the patient experience, non-modifiable associations should be individually considered, and potentially modifiable associations such as satisfaction with specific providers, pain, and depression, may be targeted for management.

Piyoderma Gangrenozum Ülserinde Trombositten Zengin Fibrin Etkili midir?

Piyoderma gangrenozum, kronik nonenfeksiyöz ülserlerle karekterize nötrofilik bir dermatozdur. Tedavisinde standart bir tedavi ya da algoritma bulunmamaktadır. Tedavi seçimi; ülserin tipine, büyüme hızına, hastanın genel durumuna ve altta yatan hastalığa bağlı olarak değişiklik gösterir. Trombositten zengin fibrin, içerisinde trombosit ve lökosit bulunan, otolog membran olup, yara örtüsü olarak kullanılabilmektedir. Burada, kullandığı sistemik tedavilere rağmen ülserinde iyileşme olmayan, 56 yaşında ve piyoderma gangrenozumu bulunan kadın hasta, trombositten zengin fibrin ile ülserinde anlamlı düzelme olması üzerine sunuldu. Trombositten zengin fibrin tedavisinin kolay hazırlanıp uygulanabilir olması, otolog özellikte olması ve aynı zamanda ucuz olmasını vurgulamak amacıyla olgu sunuldu.

Cutaneous ulcer resembling pyoderma gangrenosum in a patient with antiphospholipid syndrome

The present case presented with abrupt appearance of rapidly enlarging ulcerations over his legs that resembled PG with no atypical histopathological findings such as dermal infiltrate showing numerous neutrophils. The pathogenesis of PG-like cutaneous ulcer in the present case could be related to some abnormalities in the coagulation/fibrinolysis pathways and an abnormal immune condition that involves antiphospholipid antibodies. Cutaneous manifestations of antiphospholipid syndrome (APS) vary from livedo reticularis to cutaneous necrosis, with the appearance of associate cutaneous ulcers with sharp margins, typically over the legs.1 Classic pyoderma gangrenosum (PG), also known as ulcerative type, is characterized by rapidly progressive ulcers with violaceous indurated borders. The diagnosis remains challenging due to the lack of a gold standard diagnostic test.2 A 39-year-old man presented with a reddish necrotic and erosive lesion and cutaneous ulcers all over his legs that had appeared one month earlier. Physical examination revealed various sized necrotic ulcers include papules, vesicles, and pustules over both his legs, along with dense brown pigmentation (Figure 1A). Microscopic findings showed dermal fibrosis, intravascular hyalinized material of fibrin-containing thrombi with cell inflammation and capillary dilatation in the upper to middle dermis (Figure 1B). Antibiotic treatment alone was insufficient, and the lesion continued to gradually increase in size. He had abrupt appearance of rapidly enlarging and hemorrhagic ulcerations on his right leg. Cutaneous ulcers in the lateral femoral region of the right leg were found with undermined, overhanging, dusky purple edges, and surrounding induration and erythema (Figure 1C). Histopathological examination revealed a severe dermal infiltrate comprised of neutrophils and lymphocytic cells and capillary dilatation and thrombi in the dermis to subcutaneous fat tissue (Figure 1D). The clinical features of the cutaneous ulcer suggested a diagnosis of PG. In contrast, histopathological findings showed thrombi and vascular damage in the dermal capillary vessels with neutrophilic infiltrate. Laboratory examinations showed increased anti-cardiolipin antibodies (112 U/mL; normal range, <9 U/mL) and anti-β2 glycoprotein I antibodies (>125 U/mL; normal range 0-3.4 U/mL), and the lupus anticoagulant was detected. Abdominal contrast-enhanced computed tomography revealed thrombi, contraction, and occlusion of the abdominal aorta and iliac arteries (Figure 1E). Ultrasonography of his veins showed thrombi in the femoral veins, popliteal veins, and flounder veins. Based on these findings, he was diagnosed with APS. He responded to anticoagulation treatment with warfarin alone and his cutaneous ulcer that had initially been diagnosed as PG improved. Classic ulcerative PG is characterized by a peripheral erythematous inflammatory halo and the edges are erythematous, raised, and sometimes necrotic, with an undermining border, the size of which determines how rapidly the ulcer edge will evolve.2, 3 The present case presented with abrupt appearance of rapidly enlarging ulcerations over his legs that resembled PG with no atypical histopathological findings such as dermal infiltrate showing numerous neutrophils. There have been other reports of PG-like ulcers in patients with APS.3, 4 Recent studies have also suggested that lupus anticoagulant and anti-cardiolipin antibodies could be an important risk factor for venous ulcers of the leg.5 These antiphospholipid antibodies potentially cause repeated thrombi which lead to chronic damage of the skin and eventually to poorly healing venous ulcers over the long term. We propose that the pathogenesis of PG-like cutaneous ulcer in the present case could be related to some abnormalities in the coagulation/fibrinolysis pathways and an abnormal immune condition that involves antiphospholipid antibodies. The authors declares no conflict of interest. Approval of the research protocol and Informed Consent were performed by Tohoku Medical and Pharmaceutical University, Sendai, Japan. Registry and the Registration No. of the study/trial: N/A. Animal Studies: N/A

Management of classic ulcerative pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with unclear etiology and is associated with notable morbidity. Due to the rarity of PG, there are limited large, multicentered, randomized trials to guide management. We aim to highlight best practices in PG management through survey responses from expert medical dermatologists.

PG-TIME: A practical approach to the clinical management of pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis which may be rapidly progressive. Standard guidelines for local treatment are lacking. Through our experience, we suggested a local treatment algorithm based on the tissue, infection, moisture balance, and epithelization (TIME) concept. The clinical and histopathological features of 52 patients with PG, the duration, and the evolution of the lesions were retrospectively evaluated. Systemic therapies, local treatments, and standard wound treatments were reported. We observed ulcerative PG in the majority of the patients (82.6%), followed by the pustular form (9.6%), the peristomal type (3.8%), the vegetative form (1.9%), and the bullous type (1.9%). The lower leg was the most commonly area affected (90.4% of cases). Pathergy was reported in 15.3% of cases. The first-line treatment was the use of oral glucocorticosteroids (GCSs). We observed healing after 3 weeks of GCS in 17.3% of cases. In 25% of all cases, we obtained complete healing with long-term low doses (<0.5 mg/kg) of GCS in the range of 2 to 6 months. We used second-line treatments in 57.6% of patients. Local, systemic treatment, and correct wound management can be associated with better clinical results. We suggest a new local therapeutic algorithm in both the inflammatory and noninflammatory healing phases.

CASE REPORT OF ULCERATIVE PYODERMA GANGRENOSUM OF THE FOREARM: A CHALLENGING DIAGNOSIS

Pyoderma gangrenosum (PG) is an uncommon disease associated with significant morbidity, characterized by the rapid development of a painful, necrolytic ulcer with undermined, irregular margins. Various clinical or histological subtypes have been illustrated in the literature. The diagnoses of various subtypes of PG are difficult due to its uneven presentation, clinical overlap with other conditions and not have specific diagnostic laboratory tests or classical histopathological findings. PG is frequently misdiagnosed as infection, results in delay or inappropriate treatment of the same, which leads to devastating results such as amputation of limbs and even demise. We report here the rare case of a 64-year-old woman who had initially presented with tender papules over the right forearm, later developed expanding ulcers and underwent serial debridements, which resulted in extensive ulcerative PG at the same site. This case highlights the challenges being faced while diagnosing ulcerative PG, emphasizes the key clinical and histopathological findings to aid in the final diagnosis, and also the clinical consequences of delayed diagnosis of this condition.

Therapeutic efficacy of the combination therapy of corticosteroids and 5-aminosalicylic acid for treatment of pyoderma gangrenosum with ulcerative colitis

Background:Pyoderma gangrenosum (PG) in inflammatory bowel disease (IBD) is a rare cutaneous condition and its treatment remains controversial due to limited data.Aims and Objectives:The purpose of this study was to investigate the characteristics and treatment response to specific therapies of IBD-associated PG.Methods:In this retrospective study, we reviewed a series of cases of IBD-associated PG patients who presented at our institution, and collected clinical data, such as demographics, characteristics, subtype, and disease activity of IBD and specific therapies used and their treatment response.Results:In total, 42 cases were identified: 69% female and 92.9% with ulcerative colitis (UC). At PG diagnosis, 93% had active and 7% inactive IBD. PG ulcers occurred predominantly on the legs (33.3%); 14.3% had multiple lesions. In total, 20/39 UC patients received the combination therapy of systemic corticosteroids and 5-aminosalicylic acid (5-ASA) with a good response in 19 patients (95%). Seven patients received the monotherapy of 5-ASA with a response rate of 43%. Five patients were successfully treated with systemic corticosteroids alone. Other patients were treated with intravenous immunoglobulin, infliximab, or cyclophosphamide alone or in combination with corticosteroids and all showed a good response.Conclusion:Our study indicates the therapeutic efficacy of corticosteroids in combination with 5-ASA, which may be considered as the first-line therapy for UC-associated PG.

溃疡型坏疽性脓皮病合并皮肤真菌感染一例

溃疡型坏疽性脓皮病合并皮肤真菌感染一例

Pyoderma gangrenosum in hematologic malignancies: A systematic review

Pyoderma gangrenosum (PG) is a devastating neutrophilic dermatosis that may be associated with trauma or systemic diseases. The associations, characteristics, and temporal relationship of PG with hematologic malignancies are not well understood. We performed a systematic review of PG associated with hematologic malignancies using data from case reports, case series, and retrospective studies. We searched MEDLINE, EMBASE, Scopus, and Web of Science from each database's inception to December 12, 2018. Two reviewers independently selected studies and extracted data. Two hundred seventy-nine publications met the inclusion criteria (340 cases). Myelodysplastic syndrome (MDS) was the most commonly reported hematologic malignancy associated with PG, followed by monoclonal gammopathy of undetermined significance and acute myeloid leukemia. The mean age of patients was 56.5years, with males being more common. There was a predominance of the ulcerative PG subtype and multifocal distributions across all hematologic malignancies. The majority of MDS cases preceded PG, which was reversed for MGUS. The data were limited by reporting bias because PG subtypes rely on the rendered diagnosis reported. In addition, the classification for hematologic malignancies has evolved since 1978. Patients with PG should be evaluated for hematologic malignancies, with MDS being the most common.

Paraneoplastic pyoderma gangrenosum in solid organ malignancy: a literature review.

Pyoderma gangrenosum (PG) is a rare destructive, ulcerative, and inflammatory cutaneous disease. PG can be associated with inflammatory bowel disease (IBD), arthritis, autoinflammatory syndromes, and hematological malignancies. Multiple reports in the literature have found an association between PG and solid organ tumors. This article provides a summary and review of PG in patients with solid organ malignancies. We performed a PubMed search using the terms pyoderma gangrenosum, paraneoplastic pyoderma gangrenosum, cancer, malignancy, tumor, and solid organ malignancy. Out of 529 papers screened, 19 relevant cases were included that reported patients above the age of 12years old with antecedent, coincident, or subsequent occurrence of PG in association with a solid organ malignancy. The most common malignancies associated with PG were found in the breast (n=6, 31.6%). In a majority of the cases, the site of PG was found to be the lower extremities (n=12, 63.2%). Almost all cases were presented with ulcerative PG subtype (n=18, 94.7%). Moreover, 78.9% of cases (n=15) were reported to have PG prior to tumor diagnosis. PG lesions resolved in 100% of patients after either tumor or PG-specific treatment. We identified a strong temporal relationship between ulcerative PG and its associated solid organ malignancy. Other associations with breast cancer and lower extremity location exist but are not as strong.

Ulcerative pyoderma gangrenosum in inflammatory bowel disease

Ulcerative pyoderma gangrenosum in inflammatory bowel disease

Clinical factors influencing the response to intravenous immunoglobulin treatment in cases of treatment-resistant pyoderma gangrenosum

Background: Pyoderma gangrenosum (PG) is a neutrophilic disorder which classically presents as chronic, painful ulcers on the lower extremities. There is evidence supporting a potential role for intravenous immunoglobulin (IVIG) as adjuvant therapy for treatment-resistant cases; however, it is unclear which patients will most benefit from this modality of treatment – an especially important consideration given the cost per infusion ($5000–$10,000). Thus, we sought to identify the clinical characteristics of patients with refractory PG lesions who demonstrated complete healing when IVIG was incorporated into the therapeutic plan.Methods: We performed a literature search of PubMed/MEDLINE and Embase using the keywords ‘pyoderma gangrenosum’ and ‘IVIG’. We also added four institutional cases. Descriptive statistics were used to analyze the data. Significance was set at p < .05.Results: We discovered a total of 45 cases. Twenty-three patients with treatment-resistant PG had complete healing, 22 had partial or unhealed PG ulcers. Patients with one ulcer were 4.1 (95% CI: 1.1–18.5) times more likely to achieve complete healing than patients with more than one ulcer, when IVIG was added (p = .041).Conclusion: There is increased efficacy of IVIG as a treatment for patients with a solitary treatment-resistant PG lesion compared to patients with multiple refractory lesions.

A 40-Year-Old Woman with Asymmetric Arthritis and Skin Lesions

A 40-year old woman with recent asymmetric arthritis and fever was evaluated in our clinic. NSAIDs were recommended, but a few days later she was admitted to our hospital because of worsening arthritis along with the appearance of new skin lesions in both feet. Although she was treated with antibiotics and high dosages of steroids, her arthritis did not improve. The skin lesions progressed from bullous initially to ulcerative pyoderma gangrenosum, so we suggested endoscopic examination of the colon which revealed Crohn’s disease. The patient received I.V. treatment with infliximab resulting in a remarkable response. Some patients with Crohn’s disease may present with extraintestinal manifestations well before the bowel disease is manifested and diagnosed.

Successful experience in treating primary cutaneous anaplastic large cell lymphoma occuring with common lesions of the skin and lung tissue

The aim of the study is to present a successful case in treating primary cutaneous anaplastic large cell lymphoma (PCALCL) occurring with common lesions of the skin and lung tissue.Materials and methods. For the verification of the diagnosis in a patient with three types of skin elements (spot, thin plaque with and without ulceration), differential diagnosis was performed between ulcerative pyoderma gangrenosum, PCALCL, large-cell transformation of mycosis fungoides, and secondary skin lesions under the nodal ALK-negtaive ALCL. A complex of studies, including histological, immunohisto - chemical, cytogenetic studies of skin tumor biopsy, allowed the verification of the PCALCL diagnosis. For the treatment of the patient, intensive induction chemotherapy was used followed by high-dose consolidation and autologous transplantation of hematopoietic stem cells.Results. The selected treatment tactics allowed a long-term complete remission of the disease to be achieved in a patient from the poor prognosis group.Conclusion. An algorithm for the differential diagnosis and tactics of treating is presented for a patient with primary anaplastic large cell lymphoma with a widespread skin lesion and extradermal foci.

Improvement of Ulcerations in Treatment-Resistant Chronic Scarring in a Patient with Pyoderma Gangrenosum After Improving Vascular Insufficiency, Gently Removing Necrotic Debris, and Decreasing Wound Fluid.

Patient: Male, 44Final Diagnosis: Pyoderma gangrenosumSymptoms: Pain • ulceration • scarring • exudate • necrosisMedication: Clobetasol 0.5% ointment • Cadexomer iodine • PentoxifyllineClinical Procedure: Compression stocking application • leg elevation at nightSpecialty: Family MedicineObjective:Rare diseaseBackground:Classical pyoderma gangrenosum is a rare, inflammatory, neutrophilic dermatosis that commonly presents with severe ulcerations on the lower extremities and is often misdiagnosed and mistreated. Delay in treatments can lead to worsening of the ulcerations and allows for multiple comorbid factors. Pyoderma gangrenosum is most commonly treated with immunosuppressants or anti-inflammatory agents and is often worsened by surgical procedures due to the presence of pathergy. In acute cases, a course of anti-inflammatory treatments works well in alleviating symptoms and reducing ulcerations and residual scarring. However, in chronic cases with the presence of severe scarring and necrotic ulcerations, the simple implementation of systemic immunosuppressants is frequently ineffective alone. Although not mentioned in most case reports on pyoderma gangrenosum, the chronicity of its inflammatory component can lead to necrosis and scarring and subsequent vascular insufficiency.Case Report:We present a severe case of chronic ulcerative pyoderma gangrenosum in a patient who had treatment-resistant ulcerations and cribriform edematous scarring with subsequent vascular insufficiency of the right lower extremity. This patient, while receiving topical clobetasol, had marked improvement in the healing of his ulcerations only after starting a novel course of cadexomer iodine, compression stockings, and pentoxifylline.Conclusions:The efficacy of non-anti-inflammatory treatments indicates that chronic pyoderma gangrenosum with extensive scarring is commonly associated with the comorbid factors of vascular insufficiency, necrotic debris, and extensive wound fluid. In cases of ulcerations in chronic pyoderma gangrenosum that are resistant to anti-inflammatory treatments alone, one should identify and address other compounding factors that may inhibit wound healing.

Classic Ulcerative Pyoderma Gangrenosum Is a T Cell-Mediated Disease Targeting Follicular Adnexal Structures: A Hypothesis Based on Molecular and Clinicopathologic Studies.

Pyoderma gangrenosum (PG) is a debilitating ulcerative skin disease that is one of the most common associated diseases seen in patients with inflammatory bowel disease and rheumatoid arthritis. Although PG is classified as a neutrophilic dermatosis, its pathophysiology is poorly understood. Use data obtained from patient-reported histories, immunohistochemistry, and gene expression analysis to formulate a hypothesis on PG pathophysiology. Ten PG patients participated and answered questions about new ulcer formation. Skin biopsies of healed prior ulcers and adjacent normal skin were obtained from four patients for immunohistochemistry. Scars from healthy patients and patients with discoid lupus were used as additional controls. New onset PG papules were analyzed using immunohistochemistry and gene expression analysis via quantitative real-time PCR. All PG patients reported that healed sites of previous ulceration are refractory to re-ulceration. Simultaneous biopsies of healed and uninvolved skin triggered ulceration only in the latter. On immunohistochemistry, healed PG scars showed complete loss of pilosebaceous units, which were present in normal skin, and to a lesser extent in control scars, and discoid scars. Early PG papules showed perivascular and peripilosebaceous T cell infiltrates, rather than neutrophils. These early inflammatory events were dominated by increased gene expression of CXCL9, CXCL10, CXCL11, IL-8, IL-17, IFNG, and IL-36G and transcription factors consistent with Th1 phenotype. Small sample size was the main limitation. We put forth the hypothesis that PG is a T cell response resulting in the destruction of pilosebaceous units.