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Advances in Wound Care Dec 2020Pyoderma gangrenosum (PG) is a rare neutrophilic ulcerative dermatosis that poses a high burden of morbidity due to underdiagnosis, resistance to therapy, and limited... (Review)
Review
Pyoderma gangrenosum (PG) is a rare neutrophilic ulcerative dermatosis that poses a high burden of morbidity due to underdiagnosis, resistance to therapy, and limited therapeutic options. Optimization of wound care strategies and multimodal anti-inflammatory approaches are necessary to mitigate multiple converging pathways of inflammation leading to delayed healing, which is further complicated by additional factors such as pathergy. PG treatment typically involves reducing inflammation, controlling pain, promoting wound healing, and treating the underlying etiology. Recent advances have been made with regard to targeted therapies for PG with topical, intralesional, and systemic medications. Wound management includes gentle cleansing without sharp debridement, limited topical antibacterial use, and maintenance of a moist environment to promote epithelial migration. Wound dressings and compression therapy, in particular, introduce a wide variety of therapeutic options. Dressings should aim to target the specific PG wound type, depending on the depth and exudative nature of the wound, as well as local secondary factors. Superficial wounds, eschar, exudative wounds, granulating wounds, and colonized wounds are managed with variable approaches to the same underlying principles of pathergy avoidance, moisture balance, and reduction of immunogenic inflammatory stimuli. The importance of compression therapy to decrease edema and overgranulation fits within this treatment paradigm. As each of these treatment modalities offers a complex mixture of advantages and limitations, development of a systematic treatment algorithm in the future can help direct a more tailored path toward wound healing.
Topics: Administration, Topical; Anti-Inflammatory Agents; Compression Bandages; Critical Care; Debridement; Humans; Pyoderma Gangrenosum; Wound Healing
PubMed: 32320358
DOI: 10.1089/wound.2020.1168 -
Wounds : a Compendium of Clinical... Jun 2021Pyoderma gangrenosum (PG) is an uncommon inflammatory neutrophilic disorder with a spectrum of clinical presentations with variable courses. Most cases are associated... (Review)
Review
Pyoderma gangrenosum (PG) is an uncommon inflammatory neutrophilic disorder with a spectrum of clinical presentations with variable courses. Most cases are associated with an autoimmune disorder and manifest in middle-aged adults as a painful lesion that progresses to painful necrotizing ulcers of the lower extremity. Owing to its variability, clinical diagnosis remains difficult and many patients are often misdiagnosed, with resulting delay in treatment. While early immunosuppressant therapy is key to preventing progression of PG, surgical treatment has been met with criticism because of the risk of potentiating pathergy, an exaggerated skin reaction due to trauma. This article presents a case series in which 3 patients with PG lesions underwent different treatment methods, including surgical debridement and use of fetal bovine dermis (FBD). The use of FBD in conjunction with medical treatment provided pain relief and wound coverage as well as encouraged growth of granulation tissue and long-term stability. Commercial cellular and tissue-based products used to aid in accelerating PG wound closure are also reviewed.
Topics: Adult; Animals; Cattle; Humans; Immunosuppressive Agents; Lower Extremity; Middle Aged; Pyoderma Gangrenosum
PubMed: 34356034
DOI: No ID Found -
Iranian Journal of Allergy, Asthma, and... Dec 2016Auto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin,... (Review)
Review
Auto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto-antibodies or auto-reactive T cells, and an inborn error of innate immunity. A narrative literature review was carried out of studies related to auto-inflammatory syndromes to discuss the pathogenesis and clinical manifestation of these syndromes. This review showed that the main monogenic auto-inflammatory syndromes are familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), Blau syndrome, TNF receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA). The data suggest that correct diagnosis and treatment of monogenic auto-inflammatory diseases relies on the physicians' awareness. Therefore, understanding of the underlying pathogenic mechanisms of auto-inflammatory syndromes, and especially the fact that these disorders are mediated by IL-1 secretion stimulated by monocytes and macrophages, facilitated significant progress in patient management.
Topics: Acne Vulgaris; Antirheumatic Agents; Arthritis; Arthritis, Infectious; Cryopyrin-Associated Periodic Syndromes; Familial Mediterranean Fever; Hereditary Autoinflammatory Diseases; Humans; Immunity, Innate; Infliximab; Interleukin 1 Receptor Antagonist Protein; Interleukin-1; Macrophages; Mevalonate Kinase Deficiency; Monocytes; Pyoderma Gangrenosum; Sarcoidosis; Synovitis; Uveitis
PubMed: 28129677
DOI: No ID Found -
JPMA. the Journal of the Pakistan... Jan 2022Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that... (Review)
Review
Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that PG is not bacterial in origin, as previously believed. A significant number of cases of PG report a phenomenon called pathergy, which is characterized by the appearance of new lesions after the application of trauma to the skin. This represents a unique challenge for surgeons in cases that are refractory to medical therapy. The objective of this study is to review past literature and report a case of PG in a 19-year old woman, who presented with recurrence, after undergoing skin grafting one year back. The patient was referred for split thickness skin grafting (STSG) to reduce the psychological and physical morbidity as a result of this disease.
Topics: Adult; Female; Humans; Pyoderma Gangrenosum; Skin; Skin Transplantation; Young Adult
PubMed: 35099456
DOI: 10.47391/JPMA.11-1109 -
Anais Brasileiros de Dermatologia 2021Pyoderma gangrenosum associated to the use of cocaine/levamisole is a rare condition associated to their consumption. Cocaine use is frequent in Colombia, and the... (Review)
Review
Pyoderma gangrenosum associated to the use of cocaine/levamisole is a rare condition associated to their consumption. Cocaine use is frequent in Colombia, and the substance is contaminated with levamisole, an anthelmintic that increases the psychotropic effects and enhances its side effects. We present three clinical cases of patients with ulcerated lesions, in which the diagnosis was pyoderma gangrenosum secondary to the use of cocaine contaminated with levamisole. This called the attention of the health staff to investigate the abuse of substances in gangrenous pyoderma and also evidence that the interruption of consumption was the basis of management.
Topics: Cocaine; Cocaine-Related Disorders; Colombia; Humans; Levamisole; Pyoderma Gangrenosum
PubMed: 33640188
DOI: 10.1016/j.abd.2020.06.014 -
BMJ Case Reports Jun 2021A 75-year-old woman was admitted with sepsis and treated with broad-spectrum antibiotics until examination of her lower limbs noted necrotising wounds. Surgical...
A 75-year-old woman was admitted with sepsis and treated with broad-spectrum antibiotics until examination of her lower limbs noted necrotising wounds. Surgical intervention was advised by the plastic surgeons; however, she was deemed unsuitable for intensive care. She underwent incision and drainage of the necrotic area and biopsies were taken. She deteriorated clinically and the decision was made for best supportive care and was therefore transferred to the inpatient palliative care unit for end-of-life care. However, she stabilised, and based on culture sensitivities, antibiotics were restarted. It was also noted that the patient had a 3-month history of loose stools, which had not been addressed previously. The biopsies were suggestive of pyoderma gangrenosum, prompting a dermatology review, and prednisolone and doxycycline were started. The wounds and her loose stools improved, and with ongoing rehabilitation, she made a full recovery. Referral to gastroenterology was made.
Topics: Aged; Biopsy; Diagnosis, Differential; Drainage; Female; Humans; Prednisolone; Pyoderma Gangrenosum
PubMed: 34099445
DOI: 10.1136/bcr-2020-240133 -
The New England Journal of Medicine Oct 2002Pyoderma gangrenosum is a diagnosis of exclusion, and the misdiagnosis of pyoderma gangrenosum can result in substantial complications in patients who have other causes... (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a diagnosis of exclusion, and the misdiagnosis of pyoderma gangrenosum can result in substantial complications in patients who have other causes of severe cutaneous ulceration.
METHODS
We reviewed the charts of 240 patients with a diagnosis of pyoderma gangrenosum who were evaluated at our institution from 1975 through 2000, including 157 consecutive patients treated for presumed pyoderma gangrenosum from 1984 through 1992. We also reviewed the English-language literature.
RESULTS
Ninety-five patients (49 from our institution and 46 described in the literature) had skin ulcers with a clinical resemblance to pyoderma gangrenosum. The final diagnoses were vascular occlusive or venous disease, vasculitis, cancer, primary infection, drug-induced or exogenous tissue injury, and other inflammatory disorders. Of the 95 patients studied, 64 had been treated for pyoderma gangrenosum for a median of 10 months (range, 3 to 180). These 64 included 15 of the 157 consecutive patients treated for pyoderma gangrenosum at our institution (10 percent). Of the ulcers in the 64 patients treated for pyoderma gangrenosum, it was clear that those in 23 patients (36 percent) did not respond to treatment directed at pyoderma gangrenosum, those in 8 (12 percent) were exacerbated by such treatment, and those in 15 (23 percent) improved with such treatment.
CONCLUSIONS
The misdiagnosis of pyoderma gangrenosum is not uncommon and exposes patients to risks associated with its treatment. A thorough evaluation is required in all patients suspected of having pyoderma gangrenosum in order to rule out alternative diagnoses.
Topics: Arterial Occlusive Diseases; Diagnostic Errors; Humans; Pyoderma Gangrenosum; Retrospective Studies; Skin; Skin Diseases, Infectious; Skin Neoplasms; Skin Ulcer; Vasculitis
PubMed: 12409543
DOI: 10.1056/NEJMoa013383 -
International Wound Journal Feb 2017Pyoderma gangrenosum is a neutrophilic dermatosis clinically characterised by the presence of painful skin ulcerations with erythematous and undetermined borders and...
Pyoderma gangrenosum is a neutrophilic dermatosis clinically characterised by the presence of painful skin ulcerations with erythematous and undetermined borders and histologically by the presence of neutrophilic infiltrates in the dermis. Granulocyte and monocyte adsorption apheresis, also called granulocytapheresis, is a therapeutic strategy for extracorporeal immunomodulation that selectively removes activated granulocytes and monocytes/macrophages from the peripheral blood. Here, we report a case of a 73-year-old patient affected by a severe form of pyoderma gangrenosum presenting with multiple painful ulcers and pustules on his trunk and extremities. The disease was resistant to high doses of methylprednisolone and methotrexate and successfully treated by granulocyte and monocyte adsorption apheresis. To the best of our knowledge, this is the first report on the efficacy of granulocyte and monocyte adsorption apheresis in pyoderma gangrenosum in Europe.
Topics: Adsorption; Aged; Blood Component Removal; Europe; Granulocytes; Humans; Male; Monocytes; Pyoderma Gangrenosum; Treatment Outcome
PubMed: 27790848
DOI: 10.1111/iwj.12684 -
Respiratory Medicine Apr 2015Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatologic disease that occasionally is accompanied by extracutaneous manifestations, amongst these is... (Review)
Review
Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatologic disease that occasionally is accompanied by extracutaneous manifestations, amongst these is pulmonary involvement. The etiology is unknown. More than 50% of PG cases are associated with an underlying systemic disease such as inflammatory bowel disease, rheumatoid arthritis, hematological disorder or malignancy. Extracutaneous manifestations are rare and only 29 cases of pulmonary involvement have been reported previously in the literature. Pyoderma gangrenosum is usually diagnosed in the third to sixth decade, but early debut in childhood is also described. Skin manifestations are usually evident before pulmonary involvement, although primary lung affection is seen. Pulmonary involvement is diagnosed simultaneously or from a few weeks up to several years after the diagnosis of cutaneous PG. The most important differential diagnoses are lung cancer, lung abscess and Wegener's granulomatosis. Histological specimens will exclude these diagnoses. The treatment of PG is immune modulation, but due to the rarity of the disease, only one randomized treatment trials exists [1] and the long term course of PG with pulmonary involvement is unknown. We present two cases of pulmonary manifestations of pyoderma gangrenosum and a review of the literature.
Topics: Anti-Infective Agents; Biopsy; Dapsone; Diagnosis, Differential; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Infliximab; Lung; Male; Middle Aged; Multiple Pulmonary Nodules; Prednisolone; Pyoderma Gangrenosum; Respiratory Function Tests; Skin; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 25622759
DOI: 10.1016/j.rmed.2014.12.016 -
BMJ Case Reports Aug 2022The a priori risk of infection is high when a patient presents with an ulcerative skin condition and elevated inflammatory parameters. If the ulceration is progressive...
The a priori risk of infection is high when a patient presents with an ulcerative skin condition and elevated inflammatory parameters. If the ulceration is progressive despite adequate antibiotic therapy and tissue cultures are negative, pyoderma gangrenosum should be considered as the diagnosis. This rare infection mimicking skin condition can develop and worsen due to surgery. In this paper, we report two cases that illustrate the importance of making this clinical diagnosis in a timely manner in order to avoid unnecessary surgical interventions and worsening of the clinical picture.
Topics: Humans; Pyoderma Gangrenosum; Skin; Skin Ulcer; Anti-Bacterial Agents
PubMed: 37198754
DOI: 10.1136/bcr-2021-247770