History Of Pyoderma Gangrenosum Research Articles

Development of Postsurgical Pyoderma Gangrenosum with New Keloid after Keloid Resection.

Keloids are a dermal fibroproliferative disorder and can arise from trauma, acne, vaccination, and herpes zoster. Pyoderma gangrenosum (PG) is a painful ulcerative skin disorder that is associated with neutrophilic dysfunction. However, the pathophysiologies of keloids and PG are not fully understood. The authors encountered an unusual case of a 24-year-old woman who presented with an anterior chest keloid that bore an ulcer. The keloid was resected along with the ulcer, and histology revealed the ulcer to be a neutrophilic PG ulcer. A year after surgery, another ulcer developed in the scar. The ulcer met the PARACELSUS criteria of a postsurgical PG ulcer. After treatment with systemic prednisone and adalimumab for 250 days, the ulcer re-epithelialized. However, relapsed keloids were then observed at the PG site. Corticosteroid taping may be the safest therapy for patients with a history of PG. Conversely, if there is suspicion that a patient is prone to keloid development, diagnostic biopsies and surgical management of PG ulcers should be avoided or conducted with care.

S2736 Insulin-Like Growth Factor I Receptor Inhibitor-Induced Inflammatory Bowel Disease

Introduction: Graves’ orbitopathy is an autoimmune disease that develops in approximately 40% of patients with Graves’ disease. Insulin-like growth factor I receptor (IGF-IR) is overexpressed in this disease and plays a central role in this ophthalmopathy. Teprotumumab is a monoclonal antibody against IGF-IR used for active Graves’ orbitopathy. We report a patient who presented with rectal bleeding and was found to have a reoccurrence of inflammatory bowel disease (IBD) thought to be secondary to recent teprotumumab therapy. Case Description/Methods: A 46-year-old female with a diagnosis of Crohn’s disease, off of therapy for 4 years with endoscopic and histological remission, history of pyoderma gangrenosum, psoriasis, vitiligo, and Graves’ orbitopathy presented to the emergency department for rectal bleeding. On review, the patient had finished teprotumumab infusions 17 days prior to presentation. Following each infusion, she would experience 2 days of self-resolving rectal bleeding. After her last infusion, the bleeding persisted for more than a week leading her to seek medical care. Labs were significant for hemoglobin 11.4, CRP 0.4, and ESR 28. Colonoscopy revealed continuous severe inflammation extending from the dentate line up to the sigmoid colon as well as the presence of a cecal patch. Biopsies showed active proctocolitis with cryptitis, crypt abscesses and lymphoplasmacytosis. Patient was started on solumedrol 20 mg IV every 8 hours. Her symptoms resolved with the IV solumedrol and she was discharged home on a course of oral corticosteroids. Discussion: IGF-1 induces proliferation of regulatory T cells and pauses progression of autoimmune disease in the bowel. Animal models have suggested that IGF-1 is involved in mucosal repair and has an anti-apoptotic function in gut mucosa. Patients with active IBD have reduced levels of IGF-1. Therefore, it could be inferred that the use of teprotumumab, a medication whose method of action is to inhibit this growth factor receptor, may exacerbate or illicit IBD. The drug prescribing information does caution that teprotumumab can trigger exacerbation of the underlying IBD and recommends close monitoring of IBD patients. This case highlights a potentially detrimental side effect and appropriate discussions with patients should be held prior to this medications usage. Ideally the gastroenterologist should be kept abreast of the decision to initiate such therapy to allow for careful monitoring.

Squamous cell carcinoma of verrucous type in the setting of prior pyoderma gangrenosum: a case report

IntroductionPyoderma gangrenosum and cutaneous squamous cell carcinoma are two conditions well reported in the literature and may exist concurrently in the same patient. In fact, there have been reports of misdiagnosis of one for the other. The two conditions occurring in the same location, however, has not yet been reported.Case descriptionWe report the case of a 57-year-old Caucasian male with history of pyoderma gangrenosum of the right lateral tibial area who developed squamous cell carcinoma of verrucous type at the same site. Excisional surgery was considered to treat this patient, but he was ultimately managed with radiation therapy to the affected area due to the size of the lesion and the risk of triggering proliferation of the pyoderma gangrenosum.ConclusionsWe hope that this case report will add to the literature of both conditions, show a unique presentation of both conditions, and emphasize the inclusion of both pyoderma gangrenosum and squamous cell carcinoma when developing a differential diagnosis of a chronic, nonhealing wound.

Risk of developing pyoderma gangrenosum after procedures in patients with a known history of pyoderma gangrenosum—A retrospective analysis

The risk of postoperative pyoderma gangrenosum (PG) in patients with a known history of PG is unknown. To quantify risk and identify patient- and/or procedure-related risk factors for postsurgical recurrence or exacerbation of PG in patients with a known history of PG. We retrospectively evaluated the likelihood of postsurgical recurrence or exacerbation of PG for all patients with a confirmed diagnosis of PG at Brigham and Women's Hospital and Massachusetts General Hospital from 2000 to 2015. In all, 5.5% of procedures (n=33) led to recurrence of PG in 15.1% of patients (n=25). Compared with skin biopsy, small open surgical procedures had an adjusted odds ratio (aOR) of 8.65 (95% confidence interval [CI], 1.55-48.33) for PG recurrence or exacerbation; large open surgical procedures had an aOR of 5.97 (95% CI, 1.70-21.00); and Mohs micrographic surgery/skin excision had an aOR of 6.47 (95% CI, 1.77-23.61). PG chronically present at the time of the procedure had an aOR of 4.58 (95% CI, 1.72-12.22). Immunosuppression, time elapsed since the original PG diagnosis, and procedure location did not significantly influence risk. Our study is limited by its retrospective nature and relatively small sample size. There is a small but clinically meaningful risk for postsurgical recurrence or exacerbation of PG in patients with a known history of PG; higher risks occur with more invasive procedures and chronically present PG.

Ziarniniak grzybiasty o dramatycznym przebiegu u pacjenta z piodermią zgorzelinową w wywiadzie – opis przypadku

Ziarniniak grzybiasty o dramatycznym przebiegu u pacjenta z piodermią zgorzelinową w wywiadzie – opis przypadku

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.

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Introduction: Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a rare inflammatory disorder in children that presents with cutaneous lesions with dermal neutrophilic infiltrate, fever, and leukocytosis. Extracutaneous manifestations usually include ocular, musculoskeletal, oral, and central nervous system. We present a case of Sweet syndrome in a child presenting with respiratory failure secondary to severe airway involvement. A 9-year-old male with a history of pyoderma gangrenosum, seizure disorder, and Factor V Leiden deficiency presented to a local hospital with several weeks of developing cutaneous rash, ocular discharge, oral ulcers, and dysphagia. He rapidly progressed to respiratory failure requiring nasal intubation under fiberoptic guidance. During intubation, he was noted to have an edematous, friable trachea with mucosal hypertrophy and sloughing. No other airway abnormalities were noted. He had mild leukocytosis with WBC 13.5 and ANC 11. On Hospital Day #4, a repeat bronchoscopy was performed to assess the progression of disease. This revealed diffuse lower airway edema, papillary lesions, epithelial sloughing and copious secretions. Evaluation of lavage fluid obtained from the bronchoscopy showed neutrophilic predominance (89%). Given his history of pyoderma gangrenosum and presenting cutaneous rash, dermatology was also consulted and a skin biopsy that was performed revealed dense neutrophilic dermal infiltrate. Methylprednisolone 2 mg/kg/day was initiated, resulting in a demonstrable improvement in his cutaneous and mucosal lesions, pulmonary compliance and quantity of tracheal secretions. Follow up bronchoscopy prior to extubation revealed mild improvement in airway edema and sloughing and, incidentally, erosion of approximately 60% of his nasal septum with clean mucosal edges and no active signs of necrosis was noted. After several days of intravenous steroid therapy the patient was successfully extubated. Dapsone therapy was initiated upon extubation. The remaining extensive work-up for underlying etiology of Sweet syndrome remains negative. Discussion: Sweet syndrome has been described infrequently, with only several hundred case reports in the literature, and fewer than 50 reports in children and adolescents. This patient met all of the major and minor criteria for Sweet syndrome as described by von den Driesch. While there have been descriptions of mucosal and airway involvement in Sweet syndrome, the extent of airway involvement in this presentation of Sweet syndrome-including nasal septum destruction-is abnormal, especially in a pediatric patient. This case highlights the importance of utilizing a thorough history and physical in uncovering the unusual underlying etiology of acute respiratory failure of our pediatric patient. The prior history of pyoderma gangrenosum combined with the findings during the current admission led to a diagnosis of Sweet syndrome and appropriate therapy.

What Do Autoinflammatory Syndromes Teach About Common Cutaneous Diseases Such as Pyoderma Gangrenosum? A Commentary

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerating skin lesions with rapid onset and recalcitrant treatment course. PG treatment targets an array of inflammatory pathways with variable success. One of the hallmark features of PG is its association with a broad spectrum of systemic disorders. The authors hypothesize that there are common inflammatory pathways linking these systemic disorders to neutrophilic dermatoses. Rare autoinflammatory diseases offer insights into the understanding of inflammatory skin conditions. This article explores observations of the natural history of PG that illuminate aspects of PG pathogenesis, highlighting the role of autoinflammatory mediators.

A report of two patients with a history of pyoderma gangrenosum where total knee arthroplasty was performed under prophylactic immunosuppression

IntroductionPyoderma gangrenosum (PG) is a rare relapsing autoimmuneinflammatory disorder resulting in skin necrosis and ulcera-tion. It can be idiopathic or associated with other conditions.Sterile pustular lesions can occur 24 –48 h following cutane-ous trauma in up to 30 % of patients with PG. Blobnet firstdescribed this process in 1937 that he termed pathergy [1].Lack of surgical awareness and a striking clinical similaritybetween pathergy and wound sepsis make both delayed diag-nosisandfailuretoinitiatecorrecttreatmentlikelytooccur.PGcan be extremely difficult to treat, often requiring systemicimmunosuppression. Recurrent disease is common, but itsrelative rarity means that there is a lack of published evidenceregarding its treatment. None of the commonly used agentshave been formally assessed through a randomized controlledtrial. Many of the commonly used agents are associated withunpleasant and potentially serious side effects. Descriptions ofsurgical site pathergy following arthroplasty surgery are limit-ed. Only six cases following knee arthroplasty [ 2–7]andtwofollowinghiparthroplasty[8,9]haveappearedintheliterature.Only one describes a case with a clear past history of PG [ 8].No reports have described the indication for prophylactic im-munosuppressionagainstpathergy,norwhichpharmacologicalregimen might optimally prevent this while minimizingdelayed wound healing. We describe the first reports of pro-phylacticpreoperativeimmunosuppressionintwopatientswitha known history of PG undergoing elective total knee replace-ment (TKR) surgery.Case reportsPatient 1A 76-year-old man with tricompartmental osteoarthritis waslisted for an elective right TKR. Preoperative assessmentrevealed a past history of contralateral lower limb PG thatoccurred following trauma (Fig. 1). This was initially resis-tant to high dose prednisolone and ciclosporin therapy buthealed 6 months following the administration of intravenousinfliximab. In the absence of formal guidance or evidence,the multidisciplinary team decided on a preoperative treat-ment protocol of prophylactic immunosuppression withprednisolone (60 mg daily for 14 days, commencing 5 daysprior to surgery and then reducing by 5 mg every 5 dayspostoperatively). This was on the basis that prednisolonewas felt to have the lowest risk side effect profile. A primarycemented right TKR was performed without complication.Standard intravenous antimicrobial prophylaxis was admin-istered. On day nine postsurgery, the surgical site appearedto have healed well with no evidence of pathergy. Thepatient was independently mobile and discharged.The patient returned on day 21 postsurgery with a swol-len right knee and a small amount of serous wound dis-charge. Clinically, the soft tissues were healthy, but a mildsystemic inflammatory response was present with a whitecell count (WCC) of 13.9×10

Leukocyte adhesion defect type 1 presenting with recurrent pyoderma gangrenosum

Leukocyte adhesion deficiency 1 (LAD-1) is a rare autosomal recessive disorder of leukocyte function. LAD-1 affects about 1 per 10 million individuals and is characterized by recurrent bacterial and fungal infections and depressed inflammatory responses despite striking blood neutrophilia. Patients with the severe clinical form of LAD-1 express <0.3% of the normal amount of the β2-integrin molecules, whereas patients with the moderate phenotype may express 2-7%. Skin infection may progress to large chronic ulcers with polymicrobial infection, including anaerobic organisms. The ulcers heal slowly, require months of antibiotic treatment, and often require plastic surgical grafting. The diagnosis of LAD-1 is established most readily by flow cytometric measurements of surface CD11b in stimulated and unstimulated neutrophils using monoclonal antibodies directed against CD11b. Pyoderma gangrenosum (PG) is an uncommon condition characterized by recurrent sterile, inflammatory skin ulcers. Commonly, PG occurs in the context of inflammatory bowel disease or rheumatic, hematologic, or immunologic disorders. Here, we present a 5-year-old female with a long history of PG, which healed with atrophic scarring, who was ultimately diagnosed with leukocyte adhesion deficiency type 1 (LAD1). She had a good response to high-dose prednisone therapy (2 mg/kg) and was discharged after 3 weeks of admission but only to be re-admitted 3 weeks later with severe pneumonia. During hospital stay, she developed pneumothorax and pneumomediastinum and later succumbed to her illness.