Pyoderma Gangrenosum In Association Research Articles

Pyoderma gangrenosum with Marfan syndrome and Crohn’s disease: A rare association

A 22-year-old man presented with multiple, painful ulcers on the thighs, legs, and face of 10-day duration and diarrhea and bleeding per rectum of 2-year duration. The ulcers were tender and had a peripheral violaceous border. General examination revealed tall stature, long extremities, kyphosis, arachnodactyly, and hyperextensible wrist and thumbs. The arm span was more than the height and the thumb sign and wrist sign were positive, diagnostic of Marfan syndrome. Skin biopsy from the edge of the ulcer showed infiltrates of neutrophils and lymphocytes in the dermis and subcutaneous tissue. A detailed evaluation ruled out other causes for skin ulceration and we made a diagnosis of pyoderma gangrenosum. Echocardiogram demonstrated mitral valve prolapse. Computed tomography of abdomen showed segmental mucosal thickening as skip lesions in the colon and rectum. Endoscopy showed chronic colitis with multiple punched out ulcers. Colonic biopsy showed noncaseating granulomas suggestive of Crohn’s disease. Although inflammatory bowel disease is a well-known association of pyoderma gangrenosum, we did not come across any previous reports of coexistence of pyoderma gangrenosum, Crohn’s disease, and Marfan syndrome.

Case report of pyoderma gangrenosum in association with orthopaedic trauma

<p>Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis characterized by painful skin ulcers. It is associated with various systemic diseases but is rarely associated with orthopedic conditions. We report a case of a pyogenic gangrenosum in a 40-year-old male, that occurred in association with a lower limb fracture. This patient suffered a laceration over the ankle which was the sutured initially and the fracture was managed conservatively. Later on the wound was complicated with the development of pyoderma gangrenosum. Failure to treat with antibiotics and local wound management lead to alternative diagnosis by dermatologist. Patient was successfully managed by dermatologist and fracture was managed conservatively. Such incidents of PG in association with orthopedic procedures, complicate treatment and cause delay in recovery, leading to poor patient outcome.</p>

Rituximab and Pyoderma Gangrenosum: An Investigation of Disproportionality Using a Systems Biology-Informed Approach in the FAERS Database.

Studies have found an increased risk of pyoderma gangrenosum associated with rituximab. The structural properties and pharmacological action of rituximab may affect the risk of pyoderma gangrenosum.Additionally, pyoderma gangrenosum is associated with autoimmune disorders for which rituximab is indicated. We aimed to determine whether rituximab is disproportionally associated with pyoderma gangrenosum using a systems biology-informed approach. Adverse eventreports were extracted fromthe US Food and Drug Administration Adverse Event Reporting System (FAERS,2013-20).The Bayesian Confidence Propagation Neural NetworkInformation Component was usedto test fordisproportionality. Comparators usedto determinepotentialcausal pathways included all other medicines, all medicines with a similar structure (monoclonalantibodies), allmedicines with the same pharmacological target (CD20 antagonists)and all medicines used for the sameindication(s) as rituximab. Thirty-twopyoderma gangrenosum caseswere identified, 62.5% were female, with a median age of 48 years.There was an increasedassociation of pyoderma gangrenosum with rituximabcompared with all other medicines(exponentiated Information Component 6.75,95% confidence interval(CI) 4.66-9.23). No association was observed when the comparator was either monoclonal antibodies or CD20 antagonists. Conditions for which an association of pyoderma gangrenosum with rituximab was observed weremultiple sclerosis(6.68,95% CI1.63-15.15), rheumatoid arthritis(2.67,95% CI 1.14-4.80) and non-Hodgkin's lymphoma (2.94, 95% CI 1.80-3.73). Pyoderma gangrenosumwas reported more frequently with rituximab compared with all other medicines. The varying results when restricting medicines for the same condition suggest the potential for confounding by indication. Post-market surveillance ofbiologic medicinesin FAERS should consider a multi-faceted approach, particularly when the outcome of interest isassociated with the underlying immune condition being treated by themedicine of interest.

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.

Pyoderma gangrenosum in association with hematologic malignancies: Systematic review

Pyoderma gangrenosum in association with hematologic malignancies: Systematic review

Pyoderma gangrenosum in childhood acute lymphoblastic leukemia

Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which occurs either idiopathically or associated with various systemic diseases and malignancies. Although the association of PG with myeloid malignancies is well known, its association with lymphoid malignancy especially in children is extremely rare. We describe PG in association with acute lymphoblastic leukemia in a 4-year-old child, an occurrence which has been reported just once previously to the best of our knowledge.

Pyoderma gangrenosum avec anticorps anti-cytoplasme des neutrophiles de type anti-protéinase 3 (PR3-ANCA) induits par le propylthiouracile

Synthetic antithyroid drugs are often used in the treatment of hyperthyroidism, regardless of aetiology. They may cause various side effects, including the development of anti-neutrophil cytoplasmic antibodies (ANCA), ANCA-associated vasculitis, and neutrophilic dermatoses. Propylthiouracil (PTU) is the antithyroid drug most frequently implicated in ANCA-associated diseases specifically involving anti-myeloperoxidase ANCA (MPO-ANCA). To our knowledge, there are no clinical reports describing the association of pyoderma gangrenosum (PG) and anti-proteinase3-ANCA (PR3-ANCA) induced by PTU, with ANCA levels decreasing after antithyroid drug withdrawal. A 68-year-old woman was treated with propylthiouracil (PTU) for toxic multinodular goitre. She presented necrotic ulceration of the lower abdomen. The patient's history, physical examination, and bacteriological and histological samples led to a diagnosis of pyoderma gangrenosum. This pyoderma involved ANCA with antigenic specificity for proteinase 3. Withdrawal of PTU and a short course of corticosteroids and cyclosporine resulted in rapid and complete resolution of the pyoderma gangrenosum as well as a decrease in ANCA. No relapse was observed one year after cessation of treatment. We report a case of PG associated with PR3-ANCA induced by PTU, without any demonstrable vasculitis.

Bullous Pyoderma Gangrenosum Associated with Hairy Cell Leukemia and Its Complete Response to Cladribine Therapy.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis seen in association with systemic disorders including hematologic malignancies. Hairy cell leukemia (HCL) however, is an unusual association of PG. We describe a 49-year old lady who presented to our hematology clinic with easy fatiguability and ulcerative skin lesions of 6months duration. Examination revealed pallor and massive splenomegaly. Indurated, ulcerated lesion with undermined edges and necrotic base was observed on left thigh. Investigations revealed pancytopenia and bone marrow examination identified typical hairy cells. Flow cytometry of marrow aspirate was suggestive of classical HCL. BRAF V600E mutation was detected in peripheral blood by reverse transcriptase polymerase chain reaction. Skin biopsy revealed neutrophilic dermatosis and findings classical of bullous PG. Cladribine therapy (0.09mg/kg/day by continuous intravenous infusion for 7days) led to remission of both HCL and PG after a duration of 4weeks. Cladribine monotherapy in a case of PG with HCL may avoid the additional immunosuppresion risk imposed by treating PG separately with corticosteroids. Immunosuppressive role of cladribine might be helpful in treating PG concurrent with HCL.

Pyoderma gangrenosum révélant une diverticulite colique : deux cas

The association of pyoderma gangrenosum (PG) with colonic diverticulitis infection (DI) is relatively unknown. Herein, we describe two cases of PG with full recovery after colonic surgery. Case 1: an 83-year-old man presented with lesions on his legs that had been present for several weeks, and a diagnosis of PG was confirmed histologically. Abdominopelvic computed tomography (CT) performed on account of biological inflammatory syndrome revealed DI complicated by abscesses. Following the failure of two different antibiotic regimens, sigmoidectomy was performed. Postoperatively, the skin lesions healed without local or systemic corticosteroids. Case 2: a 63-year-old woman presented PG resistant to local and systemic corticosteroids and dapsone for several months. A particularly severe flare was accompanied by abdominal pain and inflammatory syndrome. CT revealed perforated sigmoid DI. Sigmoidectomy was performed after failure of drug therapy. The patient's PG subsequently improved and had disappeared without recurrence at 24months. Both of these cases of PG revealed DI. The hypothesis is that DI constituted a source of colonic inflammation, sending out bacterial antigenic stimuli that resulted in PG through deposition of circulating immune complexes. Removal of this inflammatory source appears to have enabled healing of PG. DI must be added to the list of systemic diseases associated with PG. In the case of isolated PG, CT may be used to detect asymptomatic DI. Early diagnosis could prevent serious gastrointestinal complications.

Association of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PASH) Shares Genetic and Cytokine Profiles With Other Autoinflammatory Diseases

Association of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PASH) Shares Genetic and Cytokine Profiles With Other Autoinflammatory Diseases

Pyoderma gangrenosum associated with solid organ malignancies.

Pyoderma gangrenosum (PG) can be associated with systemic diseases, including inflammatory bowel disease, inflammatory arthritis, and hematologic malignancy. Previous literature exploring the rare association between PG and solid organ malignancy is predominantly limited to single case reports. We retrospectively reviewed the cases of five patients with PG and solid organ malignancies at our institution between 1996 and 2013. For the five patients identified, PG and solid organ malignancy occurred within three months of each other. Mean age at onset of PG was 55.6 years, and four patients were women. Three patients had breast carcinoma (one was recurrent), and two had gastrointestinal carcinoma. PG occurred before (n = 2), after (n = 2), or synchronously with (n = 1) the malignancy. In one patient with a history of PG and quiescent inflammatory bowel disease, recurrent PG developed synchronously with a new diagnosis of metastatic rectosigmoid carcinoma. Three patients had partial or complete response of PG with treatment of PG alone. To our knowledge, our study is the first to report that the onset of PG can herald a recurrence of a solid organ malignancy and that recurrent PG can be associated with solid organ malignancy. A possible association of solid organ malignancy and PG should be considered in patients with PG of unknown etiology or with a history of malignancy.

Association of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PASH) Shares Genetic and Cytokine Profiles With Other Autoinflammatory Diseases

Association of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PASH) Shares Genetic and Cytokine Profiles With Other Autoinflammatory Diseases

Pyoderma Gangrenosum: A Paraneoplastic Syndrome Associated with Occult Colorectal Malignancy? Report of A Case and Review of Literature

Introduction: Pyoderma gangrenosum (PG) is an uncommon sterile inflammatory neutrophilic dermatosis. Approximately 50% of PG patients have associated inflammatory bowel disease, rheumatic disorders or malignancies. Literature search reported only 2 cases of PG in association with colorectal cancer (CRC). Case Report: A 74-year-old patient presented to the emergency department with central abdominal pain and vomiting for 24 hours. Recently, the patient was worked up for iron deficiency anemia and was due to have colonoscopy. She was also discovered to have multiple skin lesions on her left hand, which were ulcerated, erythematous, indurated, and thick with scaly plaque. Initially the differential diagnosis included: neutrophilic dermatosis, atypical mycoblastosis or mycosis. Skin biopsy revealed PG. The skin lesion did not respond to high-dose systemic steroids. Two months later during presentation to the emergency department with abdominal pain, abdominal and pelvic CT showed proximal sigmoid colon mass with nodular extension probably involving adjacent small bowel loops but no liver metastasis. Colonoscopy showed stenosing sigmoid mass, which was confirmed by biopsy to be adenocarcinoma. Laparotomy with resection of the sigmoid, upper part of the rectum, and a short segment of the involved small bowel was performed. Histology confirmed adenocarcinoma of the sigmoid (T4N0Mx). The skin lesions healed spontaneously following surgery. Conclusion: Early treatment of CRC can enhance the outcome of patients with PG, which may point toward a related pathological process. Furthermore, PG may be an important predictor of CRC. We advise on detailed gastrointestinal tract GIT investigation for unresponsive PG.

Association of pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) shares genetic and cytokine profiles with other autoinflammatory diseases.

The association of pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) has recently been described and suggested to be a new entity within the spectrum of autoinflammatory syndromes, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive T-cells. We conducted an observational study on 5 patients with PASH syndrome, analyzing their clinical features, genetic profile of 10 genes already known to be involved in autoinflammatory diseases (AIDs), and cytokine expression pattern both in lesional skin and serum. In tissue skin samples, the expressions of interleukin (IL)-1β and its receptors I and II were significantly higher in PASH (P = 0.028, 0.047, and 0.050, respectively) than in controls. In PASH patients, chemokines such as IL-8 (P = 0.004), C-X-C motif ligand (CXCL) 1/2/3 (P = 0.028), CXCL 16 (P = 0.008), and regulated on activation, normal T cell expressed and secreted (RANTES) (P = 0.005) were overexpressed. Fas/Fas ligand and cluster of differentiation (CD)40/CD40 ligand systems were also overexpressed (P = 0.016 for Fas, P = 0.006 for Fas ligand, P = 0.005 for CD40, and P = 0.004 for CD40 ligand), contributing to tissue damage and inflammation. In peripheral blood, serum levels of the main proinflammatory cytokines, that is, IL-1β, tumor necrosis factor-α, and IL-17, were within the normal range, suggesting that in PASH syndrome, the inflammatory process is mainly localized into the skin. Four out of our 5 PASH patients presented genetic alterations typical of well-known AIDs, including inflammatory bowel diseases, and the only patient lacking genetic changes had clinically evident Crohn disease. In conclusion, overexpression of cytokines/chemokines and molecules amplifying the inflammatory network, along with the genetic changes, supports the view that PASH syndrome is autoinflammatory in origin.

Familial pyoderma gangrenosum in association with common variable immunodeficiency

Funding sources: none. Conflicts of interest: none declared. Dear Editor, Pyoderma gangrenosum (PG) is a rare skin disease characterized by noninfective, painful, necrotizing ulcerations. PG is extremely rare in the paediatric population; 4% of patients are infants and children.1 The occurrence of PG in a familial pattern is extremely rare. As far as we could ascertain, only three reports of familial predisposition in PG have been published previously in the English literature.2 3 4 The aetiopathogenesis of PG is still unclear. Its association with a variety of immunological disorders suggests an underlying defective immune system.5 6 However, to date, no cases of PG have been described in the context of common variable immunodeficiency (CVID). We report familial PG in two siblings with CVID, which we believe to be the first documentation of this kind. Patient 1, a 9‐year‐old boy born to nonconsanguineous parents, presented with painful ulcers that had appeared 2 months before. He had a history of recurrent episodes of lower respiratory tract infection since the age of 3 years leading to bronchiectasis. On clinical examination, the child was febrile, underweight and had multiple ulcers with undermined, violaceous margins and indurated bases on his neck, scalp, upper and lower extremities, groin and buttocks. The oral mucosa was particularly affected, presenting pustular exophytic lesions with an erythematous halo and friable membrane that easily disintegrated leading to exulcerated and vegetating lesions of the labial mucosa (Fig. 1). The rest of the clinical examination was unremarkable. Laboratory investigations revealed an elevated level of inflammatory markers. Cultures for bacteria and fungi from skin lesions and blood were repeatedly negative. A skin biopsy specimen revealed an ulcerated epidermis with a massive neutrophilic infiltrate in the upper and lower dermis. Based on the clinical and histological features, the diagnosis of PG was made and the patient was treated with oral prednisone 1 mg kg−1 daily, which induced a great improvement in his lesions and complete resolution in 4 weeks. The steroids were slowly tapered with no recurrence 1 year after the diagnosis. CVID was diagnosed when screening investigations revealed low IgG and IgM levels (IgG 5 g L−1, normal range 6·13–15·12; IgM 0·4 g L−1, normal range 0·7–2·84), absent response to polysaccharide and protein antigens, a reverse CD4:CD8 ratio, with normal complement levels and nitroblue tetrazolium test functional assays. The percentage of total B cells was within the lower normal range (5% of circulating lymphocytes) but with a reduced number of memory B cells identified by the surface marker CD27 (2% of B cells, normal range 6·5–29·2%).

Postoperative pyoderma gangrenosum in association with renal cell carcinoma and chronic lymphocytic leukemia.

IntroductionPyoderma gangrenosum (PG) is a rare sterile neutrophilic dermatosis characterized by painful recurrent ulcerations. It is frequently associated with inflammatory bowel disease, rheumatoid arthritis, or malignancies. PG is a diagnosis of exclusion, and it is based on clinical presentation, histology, history of an underlying disease, and exclusion of other causes of ulceration.Case ReportThe authors report a 62-year-old male who developed a nonhealing ulcer at the site of incision following nephrectomy for renal cell carcinoma. Past medical history included chronic lymphocytic leukemia treated with rituximab. Histology of the skin lesion showed a phlegmonous nonspecific inflammation without being able to differentiate between a necrotizing wound infection and PG. The patient’s condition was initially diagnosed as an infectious process and treated accordingly. After unsuccessful results with systemic antibiotics, high-dose corticosteroids induced prompt healing of the wound. On these bases, the diagnosis of postoperative PG within chronic lymphocytic leukemia and renal cell carcinoma was made.ConclusionFaced with postoperative necrotizing ulceration resistant to correctly administered antibiotics, PG must be considered. In such condition, the diagnosis must not be guided primarily by histology and early advice of a dermatologist is recommended.

A Rare Case of Pyoderma Gangrenosum in a HIV Positive Patient

HIV infection can lead to varied spectrum of associated disease conditions. Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We report a rare case of pyoderma gangrenosum in association with Non hodgkins lymphoma(NHL) of diffuse large B cell type. In this case the lesion which showed NHL features occurred in the perianal region, coexisting with pyoderma gangrenosum lesions in the perianal, lower limb and abdominal region. Another interesting feature is the occurrence of both these conditions in a HIV-positive patient with severe immunologic failure to first line antiretroviral therapy contributing to the refractoriness to treatment. Key Words: HIV; pyoderma gangrenosum; Non Hodgkins Lymphoma DOI: http://dx.doi.org/10.3126/ajms.v2i2.4029 Asian Journal of Medical Sciences 2 (2011) 128-130

Pyoderma gangrenosum and ulcerative colitis in the tropics

Pyoderma gangrenosum is a rare inflammatory skin condition, characterized by progressive and recurrent skin ulceration. There may be rapidly enlarging, painful ulcers with undermined edges and a necrotic, hemorrhagic base. Disorders classically associated with pyoderma gangrenosum include rheumatoid arthritis, inflammatory bowel disease, paraproteinemia and myeloproliferative disorders. There have been some reports of the occurrence of pyoderma gangrenosum in Africa, and in Nigeria, but only one specifically reported pyoderma gangrenosum in association with ulcerative colitis. We report on a 45-year-old man who presented with pyoderma gangrenosum associated with ulcerative colitis; the second report in Nigeria. The skin lesions were managed with daily honey wound dressings. Oral dapsone and prednisolone were started. The frequency of the bloody diarrhea decreased, and was completely resolved by the second week after admission. The ulcers also showed accelerated healing. The goal of therapy is directed towards the associated systemic disorder, if present.

Pyoderma Gangrenosum in Association with Autoimmune Neutropenia of Infancy

We report the case of a 10-month-old girl who presented with a spontaneous ulcer on the left buttock which failed to heal despite antibiotic therapy. Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karyotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported.

Oral 8, A case of pyoderma gangrenosum in association with autoimmune neutropenia of infancy

A 10-month-old girl presented in January 2006 with a 10-day history of a rapidly enlarging 2-cm ulcer on the left buttock which had not responded to antibiotic therapy. The ulcer had an indurated red border with mild surrounding erythema. Systemic examination was unremarkable. Skin swabs showed skin flora only and subsequent treatment with Trimovate® (GlaxoSmithKline, Uxbridge, U.K.) cream, mupirocin ointment and oral azithromycin had no effect. A skin biopsy taken 8 days after presentation showed active polymorph infiltration, granulation tissue formation and a heavy mixed inflammatory cell infiltrate within the ulcer. Tissue culture showed Enterococcus faecalis which was felt to represent colonization in the napkin area. A full blood count showed a neutropenia of 0·36 × 109 L–1 (normal 1·5–8·5 × 109) with a raised monocyte count of 1·36 × 109 L–1 (0·2–0·8 × 109). Renal and liver function tests, autoimmune screen, immunoglobulins, vitamin B12, folate and C-reactive protein were normal. A clinical diagnosis of pyoderma gangrenosum was made and the ulcer was treated with topical clobetasol propionate ointment under occlusion, resulting in dramatic improvement within 3 days followed by complete healing over in 2 weeks. The patient subsequently developed mild recurrent ear and upper respiratory tract infections and repeat full blood counts showed a persistent neutropenia < 0·3 × 109 L–1 associated with an intermittent monocytosis and thrombocythaemia. In view of the known association between pyoderma gangrenosum and myelodysplasia a bone marrow aspirate was performed which showed normocellular marrow with no cytogenetic abnormalities. Further blood tests showed an IgG granulocyte-specific antibody in the serum consistent with a diagnosis of autoimmune neutropenia of infancy. She is currently on prophylactic trimethoprim 2 mg kg–1 daily and nystatin suspension, and remains fit and healthy. Autoimmune neutropenia runs a benign clinical course associated with minor infections, and is normally self-limiting by 24 months of age.1 Pyoderma gangrenosum is rare in childhood but frequently involves the perianal and genital area in this age group.2 Recognized associations include inflammatory bowel disease and haematological malignancy, although an association with autoimmune neutropenia of infancy has to our knowledge not previously been described. References 1 Taniuchi S, Masuda M, Hasui M et al. Differential diagnosis and clinical course of autoimmune neutropenia in infancy; comparison with congenital neutropenia. Acta Paediatr 2002; 91:1179–82. 2 Graham JA, Hansen KK, Rabinowitz LG, Esterly NB. Pyoderma gangrenosum in infants and children. Pediatr Dermatol 1994; 11:10–17.