Ulcerative Pyoderma Gangrenosum Research Articles

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.

Diagnosis and management of peristomal pyoderma gangrenosum: A systematic review.

Peristomal pyoderma gangrenosum (PPG) is an uncommon subtype of pyoderma gangrenosum. PPG is a challenging condition to diagnose and treat; no evidence-based guidelines exist. We sought to identify important clinical features of PPG and effective treatments available for its management. A systematic literature review of PPG was performed using PubMed, Medline, and Embase databases. We describe 335 patients with PPG from 79 studies. Clinical features include a painful, rapidly progressing ulcer with undermined, violaceous borders with a history of ostomy leakage and local skin irritation or trauma. Systemic steroids are first-line therapy; infliximab and adalimumab provide concomitant control of active inflammatory bowel disease. Combination local and systemic therapy was commonly used. Wound dressings, vehicle selection, and appropriate ostomy devices to minimize leakage, irritation, and pressure-induced ischemia can improve healing. Distinct from classic ulcerative pyoderma gangrenosum, surgical approaches, such as stoma closure and resection of active inflammatory bowel disease, have an effective role in PPG management. PPG is a rare disease lacking randomized trials or diagnostic guidelines. Treatment duration and follow-up time among studies are variable. Key clinical characteristics of PPG are highlighted. Several treatments, including a moreprominent role for surgical intervention, may be effective for PPG treatment.

Pyoderma gangrenosum and underlying diseases in Japanese patients: A regional long-term study.

Pyoderma gangrenosum (PG) is a chronic inflammatory disease of unknown cause that presents as an inflammatory and ulcerative disorder of the skin. PG is often associated with an underlying systemic disease. However, the frequencies of the underlying diseases are unclear in Japanese patients. In this retrospective, observational study, all patients diagnosed with PG who visited dermatology departments of nine regional hospitals in and around Ibaraki Prefecture were collected from 1982 to 2011 or 2014. The diagnoses of PG were based on the characteristic clinical and histological appearances and ruling out of infection. Sixty-two PG patients, including 29 males and 33 females, were identified. The ages of onset were 16-89 years, and the mean age was 50.2 years. Fifty (80%) of the 62 patients presented with an ulcerative PG, and the lower leg was the most common site (74%). Forty-six (74%) PG patients had underlying diseases. The most frequent was ulcerative colitis (32%), followed by myelodysplastic syndrome (11%), rheumatoid arthritis (6%) and aortitis syndrome (5%). For treatment, 54 cases (87%) received systemic corticosteroids and 10 received additional treatment with cyclosporin. There was no significant correlation between underlying diseases and response to the initial treatment. Multivariate analysis revealed that the number of affected sites negatively correlated with successful initial treatment. Fifteen (24%) of the 62 cases relapsed. In conclusion, ulcerative colitis and hematological disorders were frequently associated with PG while approximately a quarter of the cases were idiopathic.

Pyoderma gangrenosum: A clinico-epidemiological study.

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown etiology, with inconstant systemic associations and a variable prognosis. To study the clinical features and systemic associations of pyoderma gangrenosum and its response to treatment. All patients diagnosed to have pyoderma gangrenosum at the dermatology department of the Government Medical College, Kozhikode, from January 01, 2005 to December 31, 2014 were included in this prospective study. During the 10-year study period, 61 patients were diagnosed to have pyoderma gangrenosum. A male predilection was noted. The most common clinical type was ulcerative pyoderma gangrenosum (90.2%). More than 60% of patients had lesions confined to the legs; 78.7% had a single lesion and 27.9% had systemic associations. Most patients required systemic steroids. Patients with disease resistant to steroid therapy were treated with intravenous immunoglobulin G and split-thickness skin grafts under immunosuppression induced by dexamethasone pulse therapy. All except one patient attained complete disease resolution. The main limitation of our study was the small sample size. The male predilection documented by us was contrary to most previous studies. We found split-thickness skin graft to be a useful option in resistant cases. More prospective studies may enable the formulation of better diagnostic criteria for pyoderma gangrenosum and improve its management.

Multiple Pyoderma Gangrenosum Ulcers Associated with Cocaine Abuse.

Multiple Pyoderma Gangrenosum Ulcers Associated with Cocaine Abuse.

Pyoderma gangrenosum : two case reports

Pyoderma gangrenosum is a rare, often destructive, neutrophilic dermatosis characterized by painful, necrotic superficialulceration, central suppurative exudate and a peripheral undermined border. Five clinical variants are recently recognized asfollows: classic(ulcerative), bullous, pustular, vegetative and peristomal types. Pyoderma gangrenosum is often a diagnosis ofexclusion, as there are no specific laboratory or histopathologic findings to confirm the diagnosis. Accordingly, it is difficult todiagnose, and often requires a work-up for underlying systemic diseases. Mimickers include infection, vascular insufficiency,systemic vasculitides, autoimmune disease, skin cancer or exogenous tissue injury. Herein, we present two cases of ulcerativepyoderma gangrenosum occurring on the legs, along with a comprehensive review.

Use of Becaplermin for nondiabetic ulcers: pyoderma gangrenosum and calciphylaxis

Large difficult to heal ulcers of various etiologies carry a high morbidity and mortality rate. Becaplermin is a recombinant platelet-derived growth factor approved for treatment of diabetic ulcers. In this two-case series, we report the use of becaplermin in the treatment of ulcers due to (i) calciphylaxis, an often fatal condition resulting from systemic calcification and thrombosis of vessels and (ii) pyoderma gangrenosum (PG), a neutrophilic dermatosis. We also report that topical collagenase worsened PG ulcers, consistent with pathergy. Becaplermin can be used to help treat ulcers resulting from calciphylaxis and PG. These encouraging results lend support for the utilization of becaplermin in the treatment of nondiabetic chronic ulcers of various etiologies.

Recurrent and treatment-resistant pyoderma gangrenosum, secondary to hidden underlying breast cancer

Pyoderma gangrenosum (PG) is a rare ulcerative disorder of the skin associated with underlying diseases, such as rheumatoid arthritis, inflammatory bowel disease and hematological malignancies. However, there have been very few reports of association of PG with solid organ malignancies. We report here the case of a 37-year-old lady with a chronic non-healing ulcer of the thigh, who repeatedly underwent multiple debridements, followed by two failed grafts, which was later diagnosed as PG. Initially, she failed to respond to oral steroids, but responded well with intravenous methylprednisolone pulse. However, she later presented with a breast mass with bone metastasis and recurrence of PG ulcer. Diagnosis and treatment of the underlying occult malignancy along with that of PG at an initial stage would have prevented the recurrence and avoided a serious consequence like metastasis. The case study underscores the association of PG with serious conditions, such as malignancy, and the necessity to conduct prompt and thorough analysis in such cases.

Ulcérations extensives chez l’enfant : penser au pyoderma gangrenosum

Pyoderma gangrenosum is an amicrobial neutrophilic dermatosis of unknown cause with a chronic course. We report a case in a 30-month-old child who presented with progressive and painful skin ulcers. Lesions were quickly extensive, refractory to local and systemic antibiotic therapy. Histopathology of the skin biopsies confirmed the diagnosis of ulcerative pyoderma gangrenosum. Only 4% of the cases reported in the literature are in children below the age of 4 years. This neutrophilic dermatosis is associated in half of the cases with a systemic disease. The treatment is based on corticosteroids. Immunosuppressant drugs such as tacrolimus, cyclosporine, and mycophenolate mofetil may be effective for pyoderma gangrenosum refractory to corticosteroids. Currently, anti-TNF is a promising treatment for refractory PG.

Pyoderma gangrenosum localized on the breast

Breast ulceration is usually associated with breast cancer. However, it is important to know other factors that may be involved in its etiology. In this case report, we presented a very rare cause of pyoderma gangrenosum (PG) in the breast of a female patient without any prior history of breast tissue trauma or surgical intervention. More than one-half of PG cases develop in association with an underlying systemic condition including inflammatory bowel disease (IBD), hematologic disorder and arthritis. In contrast to other extraintestinal manifestations, cutaneous and ocular disorders occur at equal frequency in both Crohn’s disease and ulcerative colitis. PG has been detected in 0.75% of IBD patients. It usually is related to the activity of colitis. The classic lesion begins as erythematous pustules or nodules. The patients with ulcerative PG have had an associated disease such as IBD, arthritis, monoclonal gamapathy, and internal malignancy. Lesions may be single or multiple. It can be resolved by treatment of the underlying colitis. For mild localized cases, topical corticosteroid or topical tacrolimus should be considered as the first choice. Severe cases can require systemic glucocorticoids, immunosuppressants or anti-TNF therapy. This case differs from others reported in the literature because in addition to breast, hand and foot lesions consistent with pyoderma gangrenosum were also present in this patient.

Letter to the Editor Pyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Romańska-Gocka K, Cieścińska C, Zegarska B, et al. Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2015;32(2):137-141. doi:10.5114/pdia.2014.40974. APA Romańska-Gocka, K., Cieścińska, C., Zegarska, B., Schwartz, R., Cieściński, J., & Olszewska-Słonina, D. et al. (2015). Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 32(2), 137-141. https://doi.org/10.5114/pdia.2014.40974 Chicago Romańska-Gocka, Krystyna, Czanita Cieścińska, Barbara Zegarska, Robert A. Schwartz, Jakub Cieściński, Dorota Olszewska-Słonina, and Rafał Czajkowski. 2015. "Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review". Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii 32 (2): 137-141. doi:10.5114/pdia.2014.40974. Harvard Romańska-Gocka, K., Cieścińska, C., Zegarska, B., Schwartz, R., Cieściński, J., Olszewska-Słonina, D., and Czajkowski, R. (2015). Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 32(2), pp.137-141. https://doi.org/10.5114/pdia.2014.40974 MLA Romańska-Gocka, Krystyna et al. "Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review." Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, vol. 32, no. 2, 2015, pp. 137-141. doi:10.5114/pdia.2014.40974. Vancouver Romańska-Gocka K, Cieścińska C, Zegarska B, Schwartz R, Cieściński J, Olszewska-Słonina D et al. Letter to the EditorPyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2015;32(2):137-141. doi:10.5114/pdia.2014.40974.

潰瘍性大腸炎治療経過中に生じた頭部の潰瘍型壊疽性膿皮症

潰瘍性大腸炎治療経過中に生じた頭部の潰瘍型壊疽性膿皮症

Genital Ulcerative Pyoderma Gangrenosum in Behçet's Disease: A Case Report and Review of the Literature.

Behçet's disease (BD), first described by Hulusi Behcet, is a multisystemic disease characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis and vascular disease. Pyoderma gangrenosum (PG) is a rare, chronic, sterile pustular and progressive ulcerative process of unknown cause; sometimes can participate in the differential diagnosis of Behcet's ulceration. A 33-year-old woman complained a severe genital ulcer. She had a purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm. A punch biopsy at ulcer margin showed that the lymphocytic panniculitis was extending to the subcutaneous fat tissue without fibrin deposition or necrotic changes in the vessel wall. Based on the clinical and histological findings, she was diagnosed as genital ulcerative PG, which occurred during the exacerbation of BD.

Use of dermal injection of activated protein C for treatment of large chronic wounds secondary to pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a systemic disease that presents with cutaneous necrotizing ulceration, producing deep necrotic ulcers, usually with a raised, undermined, violaceous border. Treatment typically involves high dose immunosuppressive drugs, but more recently anti-tumour necrosis factor and monoclonal antibodies have been used. Activated protein C (APC) stimulates wound healing in patients with treatment-refractory skin ulcers, possibly by stimulating angiogenesis and re-epithelialization, and preventing inflammation. To investigate whether APC may be beneficial as a treatment for ulcers related to cutaneous PG. Two patients were recruited with a clinical history and physical and histopathological evidence of acute PG. A total of 400μg (1.0mL) of APC was injected subcutaneously into the dermal edge of necrotic PG ulcers weekly for a total treatment period of 6weeks. Photographs were taken, and clinical progress, ulcer size and pain score were monitored during this period and after the cessation of treatment, at weeks 8and 12. Over the 12weeks of the trial, APC led to a reduction in wound size from 3.8cm(2) to 0.8cm(2) in patient 1 (78.9% decrease) and from 41cm(2) to 16cm(2) in patient 2 (70.0% decrease, respectively), and a reduction in pain scores from 10 to 0 (100% decrease) in both patients. Although this study has limited because of its small sample size and lack of a true placebo group, it does indicate that APC has potential as a therapeutic option for patients with chronic skin ulcers from PG.

Allogenic Mesenchymal Stem Cell Transplantation For Refractory Severe Pyoderma Gangrenosum

Background: Pyoderma Gangrenosum (PG) is a rare and an idiopathic, inflammatory ulcerative condition, which is a diagnosis of exclusion and the treatment are empirical and based on small series or local experience. Mesenchymal stem cells (MSCs) have immunomodulatory and tissue-repairing effects in autoimmune diseases. Objectives: This study aimed to assess the efficacy of UC-MSCT and define whether there is remission of wounded skin in refractory severe PG patient. Methods: Twenty nine year old male PG patient was recuited. UC-MSCs were prepared by the Stem Cell Center of Jiangsu Province (Beike Bio-Technology). The patient underwent two times of UC-MSCT on June 13th, 2012 and June 27th, 2012. The protocol was approved by our Ethics Committee and informed consent was signed from the patient. Results: The PG patient presented with pustules over the lower limbs and was refractory to oral prednisolone (initially 60 mg per day, tapered in 8 weekly steps to 5 mg per day) , pulse intravenous cyclophosphamide (0.6 g per month for 6 months) and he also underwent two times unsuccessful of skin grafting for the lower extremity ulcers. There are no adverse events during the UC-MSCT treatment. After one week, he was free of intense pain and the exudation significantly reduced. Auto-skin grafting was given 4 weeks after UC-MSCT and the grafts came from his back and inner thigh. His ulcers significantly healed with complete resolution of pain 2 months after UC-MSCT. Maintenance therapy followed transplantation included prednisone 5 mg per day and intravenous cyclophosphamide 0.6 g per month. Conclusions: This is the first reported case of successful allogenic UC-MSCT for refractory severe PG. Although additional studies are needed to confirm this finding, we believe that UC-MSCT may be considered a therapeutic option in large area ulcerative PG patients unresponsive to conventional treatments.

A case of pyoderma gangrenosum with ulcerative colitis treated with combined approach: Infliximab and surgery

Pyoderma gangrenosum (PG) is an ulcerating noninfectious disease of the skin seen in 1–2% of patients with inflammatory bowel disease (IBD). The pathogenesis of PG has yet to be determined, but may be related to abnormal T cell responses and the production of TNF-α, a pathway also involved in IBD pathogenesis. Infliximab, a chimeric monoclonal antibody to TNF-α, is used to treat moderate to severe IBD and several case reports and studies suggest the efficacy of infliximab in the treatment of PG. The surgical approach to PG is reserved to a few selected cases. We report here the case of a patient with ulcerative colitis (UC) and PG localized on the left breast, treated with a simultaneous combined medical and surgical approach.

Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients

Pyoderma gangrenosum (PG) is an uncommon and challenging disease, highly associated with comorbidities, but poorly characterized from a diagnostic and therapeutic perspective. To describe the epidemiology of PG in a hospital-based retrospective review, focusing on demographics, comorbidities and treatments. We conducted a retrospective chart review. Patient data were taken from the Research Patient Data Repository of Brigham and Women's Hospital and Massachusetts General Hospital from 1 January 2000 to 31 December 2007. We identified and confirmed 103 cases of PG, and collected data on anatomical location, number and size of the PG lesions, patient demographics, comorbidities, mortality rate and treatments. Of the 103 patients, 78 (76%) were female, and only 7% had a biopsy suggestive of PG. The lower leg was the most common location with 78% of PG ulcers occurring there, and 67 (65% of patients) had two or more ulcers at some point. Thirty-five individuals (34%) had inflammatory bowel disease (IBD), 21 (20%) had haematological disorders, 14 (14%) had major depression, 20 (19%) had seronegative arthritis, 11 (11%) had psoriasis, and nine (9%) had hepatitis. Therapy was generally multimodal. The mortality rate during the 8-year study period was 16%. We present one of the largest PG case series to date. In our study, we found that biopsy of a PG lesion rarely yielded characteristic features of the disease and tissue pathology should not be used to exclude a PG diagnosis. We also found a female predominance and associations with IBD and haematological disorders. Patients with PG in this series had high rates of depression and hepatitis. Further work is needed to establish the mechanism(s) underlying these findings.

Pyoderma Gangrenosum of Abdominal Wall

Pyoderma Gangrenosum of Abdominal Wall

Ulcerative pyoderma gangrenosum in mixed connective tissue disorder: A rare association and role of azathioprine in the management

Ulcerative pyoderma gangrenosum in mixed connective tissue disorder: A rare association and role of azathioprine in the management

Improvement of ulcerative pyoderma gangrenosum with hyperbaric oxygen therapy

Pyoderma gangrenosum (PG) is a rare noninfectious destructive neutrophilic dermatosis of unknown origin affecting the skin and occasionally the subcutaneous fat. In this report, we present the results of intensive hyperbaric oxygen (HBO) therapy in a 62-year-old Greek woman who had been diagnosed with ulcerative PG two years ago, but had been resistant to other therapies.