Materiały źródłowe

• #1 Diagnosis and treatment of pyoderma gangrenosum

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1513476/

  Pyoderma gangrenosum is a rare but serious ulcerating skin disease, the treatment of which is mostly empirical. […] This can delay the diagnosis and have serious clinical consequences. The diagnosis of pyoderma gangrenosum is based mainly on clinical findings because biopsies show no specific diagnostic features. […] In many cases, however, a biopsy can help exclude other conditions such as malignancy, infections, or cutaneous vasculitis. […] The differential diagnosis of pyoderma gangrenosum is wide. […] No single, specific treatment exists and few controlled trials of treatment have been done. […] Most clinicians use a stepwise approach and both topical and systemic treatments. […] Immunosuppression is the mainstay of treatment, and the most commonly used drugs are corticosteroids and ciclosporin.

• #2 Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

  https://www.mdpi.com/2571-8800/4/3/28

  Pyoderma gangrenosum (PG) is a rare entity that is characterized by infiltration of neutrophils into the dermis, causing the formation of rapidly enlarging, painful and necrotic skin ulcers. […] However, the diagnosis of PG proves difficult, firstly because there are multiple variants of the disease and secondly because it is a clinical diagnosis and can appear similar to that of other diseases such as vasculitis, skin/soft tissue infections and malignancy. […] Early diagnosis of pyoderma gangrenosum is of essential importance, as it is key to initiating treatment and minimizing the occurrence of large, necrotic and extremely painful ulcers that can become secondarily infected and lead to the formation of disfiguring scars. […] The diagnosis of PG, however, can prove extremely difficult due to a lack of standardized clinical diagnostic criteria to aid in timely recognition and due to the fact that PG is a clinical diagnosis and the differential diagnosis for ulcerative lesions is quite vast.

• #3 Pyoderma gangrenosum – a guide to diagnosis and management 

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6542232/

  Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. […] The differential diagnosis includes all other causes of cutaneous ulceration as there are no definitive laboratory or histopathological criteria for PG. […] Despite being a well-recognised condition, there is often a failure to make an early diagnosis of PG. This diagnosis should be actively considered when assessing ulcers, as prompt treatment may avoid the complications of prolonged systemic therapy, delayed wound healing and scarring. […] Thus far there are no validated, established diagnostic clinical or pathological criteria to diagnose PG. Su et al have proposed a diagnostic tool requiring two major and two minor criteria, maintaining PG as a diagnosis of exclusion.

• #4 Diagnosis and treatment of pyoderma gangrenosum | The BMJ

  https://www.bmj.com/content/333/7560/181

  Pyoderma gangrenosum is a rare but serious ulcerating skin disease, the treatment of which is mostly empirical. […] This can delay the diagnosis and have serious clinical consequences. […] Early diagnosis and prompt treatment reduce the risk of scars, and disfigurement may occur if the diagnosis is missed.

• #5 Pyoderma Gangrenosum – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/pyoderma-gangrenosum

  Diagnosis of pyoderma gangrenosum is clinical and is a diagnosis of exclusion after other causes of ulceration have been ruled out. […] Expansion of ulceration after surgical debridement strongly suggests pyoderma gangrenosum. […] Biopsies of lesions are not often diagnostic but may be supportive; many biopsies from a leading edge show vasculitis with neutrophils and fibrin in superficial vessels. […] Patients who have bullous (atypical) pyoderma gangrenosum should be monitored with periodic clinical assessment and complete blood count for development of a hematologic disorder. […] Diagnose pyoderma gangrenosum clinically.

• #6 Pyoderma Gangrenosum: Symptoms, Causes, and Treatment — DermNet

  https://dermnetnz.org/topics/pyoderma-gangrenosum

  Pyoderma gangrenosum is diagnosed by its characteristic appearance and severe pain. The pathergy test is usually positive (a skin prick test causing a papule, pustule, or ulcer). […] A biopsy may be necessary to rule out other causes of ulceration. Pyoderma gangrenosum causes a neutrophilic inflammatory infiltrate, but this may be absent when on treatment. […] Criteria published after a Delphi consensus exercise in 2018 supersede the Su criteria with a single major criterion required to diagnose pyoderma gangrenosum (a neutrophilic infiltrate). The addition of four or more of the eight minor criteria yields a sensitivity of 86% and a specificity of 90% for pyoderma gangrenosum. […] Histopathology of ulcer edge must show a neutrophilic infiltrate. […] Exclusion of infection […] Pathergy

• #7 Pyoderma gangrenosum – a guide to diagnosis and management – International Labour Organization

  https://labordoc.ilo.org/discovery/fulldisplay?docid=cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6542232&context=PC&vid=41ILO_INST:41ILO_V1&lang=en&adaptor=Primo%20Central

  Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. There are several subtypes, with ‘classical PG’ as the most common form in approximately 85% cases. This presents as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/erythematous border. The lower legs are most frequently affected although PG can present at any body site. Other subtypes include bullous, vegetative, pustular, peristomal and superficial granulomatous variants. The differential diagnosis includes all other causes of cutaneous ulceration as there are no definitive laboratory or histopathological criteria for PG. […] Despite being a well-recognised condition, there is often a failure to make an early diagnosis of PG. This diagnosis should be actively considered when assessing ulcers, as prompt treatment may avoid the complications of prolonged systemic therapy, delayed wound healing and scarring.

• #8 Pyoderma gangrenosum: a diagnostic and therapeutic challenge

  https://elenaconde.com/en/pyoderma-gangrenosum-a-diagnostic-and-therapeutic-challenge/

  Pyoderma gangrenosum is an inflammatory disease that leads to skin ulcers and its diagnosis and treatment can represent a real challenge for healthcare professionals. […] Essentially because we do not know many clues to understand the behaviour of this disease, it remains a diagnosis of exclusion and may be resistant to treatment. […] Pyoderma gangrenosum typically occurs with the onset of single or multiple erythemato-violaceous pustules that rapidly progress to necrotic ulcers with an infiltrated purpuric rim. […] Worsening of lesions following skin biopsy or surgical debridement of necrotic tissue is also typical. […] Although pyoderma gangrenosum has typical histological findings (neutrophilic inflammatory infiltrate), they are not pathognomonic and may vary depending on the evolution of the lesion over time. It is therefore a diagnosis of exclusion. […] Although wound progression may occur after biopsy, this procedure is needed to rule out other aetiologies that require management other than pyoderma gangrenosum.

• #9 Pyoderma Gangrenosum – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/pyoderma-gangrenosum

  The diagnosis is usually based on the appearance of the sores. […] Doctors diagnose pyoderma gangrenosum by the appearance of the sores and by ruling out other diseases that cause sores to form on the skin. The diagnosis of pyoderma gangrenosum is strongly suggested if the sores worsen after minor trauma or a surgical procedure (such as a skin biopsy). […] A sample of skin may need to be removed and examined under a microscope (called a skin biopsy), even though this procedure might worsen the problem temporarily.

• #10 Diagnosis and treatment of pyoderma gangrenosum

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1513476/

  When corticosteroids fail, the most widely used alternative is ciclosporin. […] Pyoderma gangrenosum can occur on any skin surface and should be considered in any ulcer or surgical wound that does not heal. […] If pyoderma is suspected, the patient should be referred urgently for a specialist opinion.

• #11 Pyoderma gangrenosum: challenges and solutions | CCID

  https://www.dovepress.com/pyoderma-gangrenosum-challenges-and-solutions-peer-reviewed-fulltext-article-CCID

  Pyoderma gangrenosum (PG) is a rare disease, but commonly related to important morbidity. […] PG presents as painful, single or multiple lesions, with several clinical variants, in different locations, with a non specific histology, which makes the diagnosis challenging and often delayed. […] In the classic ulcerative variant, characterized by ulcers with inflammatory undermined borders, a broad differential diagnosis of malignancy, infection, and vasculitis needs to be considered, making PG a diagnosis of exclusion. […] Moreover, there are no definitively accepted diagnostic criteria. […] The diagnosis is commonly challenging and delayed, and misdiagnosis is also frequent, mostly by non-dermatologists who are less aware of this entity. […] The multiple clinical variants (classic ulcerative, pustular, bullous, and superficial granulomatous), and the overlapping forms with other neutrophilic diseases, frequently observed in clinical practice, make the diagnosis even more difficult.

• #12 Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

  https://www.mdpi.com/2571-8800/4/3/28

  Over the years, three diagnostic criteria have been proposed to aid in the recognition of pyoderma gangrenosum. […] In 2004, Su et al. proposed guidelines consisting of two major and two minor criteria, also known as the Mayo diagnostic criteria, named after Dr. Su’s work at the Mayo Clinic. […] According to this tool, diagnosis of PG is made by fulfilling two major and two minor criteria. […] 14 years after Su et al. proposed their diagnostic criteria, Maverakis et al. presented additional diagnostic criteria based on an international Delphi consensus of experts that was found to yield a sensitivity of 86% and a specificity of 90% in diagnosing pyoderma gangrenosum if one major criterion and/or four minor criteria are met. […] In 2018, the PARACELSUS score was released by Dissemond et al. alongside Maverakis et al.’s international Delphi criteria as an additional set of proposed diagnostic criteria for pyoderma gangrenosum.

• #12 Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

  https://www.mdpi.com/2571-8800/4/3/28

  Pyoderma gangrenosum (PG) is a rare entity that is characterized by infiltration of neutrophils into the dermis, causing the formation of rapidly enlarging, painful and necrotic skin ulcers. […] However, the diagnosis of PG proves difficult, firstly because there are multiple variants of the disease and secondly because it is a clinical diagnosis and can appear similar to that of other diseases such as vasculitis, skin/soft tissue infections and malignancy. […] Early diagnosis of pyoderma gangrenosum is of essential importance, as it is key to initiating treatment and minimizing the occurrence of large, necrotic and extremely painful ulcers that can become secondarily infected and lead to the formation of disfiguring scars. […] The diagnosis of PG, however, can prove extremely difficult due to a lack of standardized clinical diagnostic criteria to aid in timely recognition and due to the fact that PG is a clinical diagnosis and the differential diagnosis for ulcerative lesions is quite vast.

• #13 The challenges of managing patients with pyoderma gangrenosum: three case reports :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-24-no-1/challenges-managing-patients-pyoderma-gangrenosum-three-case-reports

  Su et al. proposed a diagnostic criterion for classic ulcerative PG, to consider the essential features for diagnosing PG. […] The proposed diagnostic criteria by Su et al. aids the clinician with the diagnosis of PG; this should not be used in isolation but as a guide. […] The accurate diagnosis of PG is challenging.

• #13 The challenges of managing patients with pyoderma gangrenosum: three case reports :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-24-no-1/challenges-managing-patients-pyoderma-gangrenosum-three-case-reports

  Pyoderma gangrenosum (PG), although first described nearly 100 years ago, remains challenging for clinicians. […] There are no specific guidelines for the diagnosis and treatment of PG. […] The aim of this paper is to provide an overview of the challenges faced by the clinician in diagnosing and managing patients with PG. […] Histopathology and laboratory findings in PG are non-specific; therefore, the diagnosis is based on clinical history, physical examination and confirmed through a process of elimination. […] There should be a high index of suspicion in patients with non-healing ulcers, especially in the presence of systemic diseases. […] The important factor to take into consideration is that PG mimics many other conditions, leading to misdiagnosis. […] Early referral to a physician or dermatologist is recommended if PG is suspected.

• #14 Pathology Outlines – Pyoderma gangrenosum

  https://www.pathologyoutlines.com/topic/skinnontumorpyodermagangrenosum.html

  Pyoderma gangrenosum is a diagnosis of exclusion based on clinical and histological findings […] Nonspecific clinical and histologic findings; a diagnosis of exclusion […] Proposed diagnostic criteria by Delphi Consensus of International Experts (JAMA Dermatol 2018;154:461): Need major criteria + ≥ 4 minor criteria […] Major criteria: neutrophilic infiltrate on biopsy of ulcer edge […] Minor criteria: Exclude infection […] Pathergy […] History of inflammatory bowel disease or inflammatory arthritis […] History of papule, pustule, vesicle within 4 days of ulceration […] Peripheral erythema, undermining border and tenderness […] Multiple ulceration, at least 1 on anterior lower leg […] Healed ulcer site with wrinkled paper scar […] Decreased ulcer size upon initiating immunosuppressant.

• #15 Pyoderma gangrenosum — the long road from diagnosis to treatment | Kleemann | Rheumatology Forum

  https://journals.viamedica.pl/rheumatology_forum/article/view/95871

  According to the new Delphi criteria, neutrophilic inflammatory infiltration on histopathological examination of a specimen from the ulcer margin is necessary to diagnose the disease. […] The PARACELSUS score is a novel, easy-to-implement, effective and sensitive diagnostic tool for PG. […] The three major diagnostic criteria are rapidly progressive disease, lack of a corresponding differential diagnosis and a reddish-violaceous wound margin (found in 98.3% of PG patients). […] A total score of 10 or more indicates a high probability of PG and differentiates it from venous leg ulcers. […] In the patient described, the diagnosis has presented many difficulties over the years due to the inconclusive histopathological findings and doubts about the vascular aetiology of the lesions.

• #15 Pyoderma gangrenosum — the long road from diagnosis to treatment | Kleemann | Rheumatology Forum

  https://journals.viamedica.pl/rheumatology_forum/article/view/95871

  Pyoderma gangrenosum is a rare inflammatory skin disease. It is a type of neutrophilic dermatosis characterised by painful, rapidly enlarging ulcerations. […] Using the patient’s case and the available literature, we show that the diagnosis and treatment of pyoderma gangrenosum are complex and require a multi-specialist approach due to its rarity and systemic comorbidities. New diagnostic criteria and developed treatment algorithms may facilitate diagnosis and shorten the time to introduce effective treatment, including biological therapy, and current research on PG appears promising. […] The diagnostic criteria for classic ulcerative PG were confirmed by a consensus of international experts at Delphi in 2018 and aimed to standardise the diagnostic process. […] Making a diagnosis of PG is difficult and requires a multi-specialist approach. It is often diagnosed by exclusion.

• #16 Computer-Assisted Differential Diagnosis of Pyoderma Gangrenosum and Venous Ulcers with Deep Neural Networks

  https://www.mdpi.com/2077-0383/11/23/7103

  Pyoderma gangrenosum (PG) is often situated on the lower legs, and the differentiation from conventional leg ulcers (LU) is a challenging task due to the lack of clear clinical diagnostic criteria. […] The CNN achieved a sensitivity of 97% (95% confidence interval (CI) 84.2–99.9%) and outperformed dermatologists, with a sensitivity of 72.7% (CI 54.4–86.7%) significantly (p < 0.03). [...] For the first time, a deep neural network was demonstrated to be capable of diagnosing PG, solely on the basis of photographs, and with a greater sensitivity compared to that of dermatologists. [...] The PARACELSUS-Score consists of differently valued criteria, such as rapidly progressing disease, assessment of relevant differential diagnoses and a reddish-violaceous wound border. A total score value of 10 points or more indicates a high likelihood of PG and often correctly differentiates PG from venous leg ulcers.

• #17 Pyoderma Gangrenosum of the Breast: A Rare Location of Neutrophilic Dermatoses

  https://clinmedjournals.org/articles/cmrcr/clinical-medical-reviews-and-case-reports-cmrcr-9-403.php/1000

  The PARACELSUS score contains major criteria which are: Progressing disease and Assessment of relevant differential diagnoses, Reddish-violaceous wound border and Minor criteria: Amelioration by immunosuppressant drugs, characteristically irregular ulcer shape, extreme Pain visual analog scale > 4/10, localization of lesion at the site of trauma, suppurative inflammation in histopathology, undermined wound border, a systemic disease associated. […] The diagnostic criteria are scored from 3 to 1 point. The diagnosis is established when the score is greater than or equal to 10 out of 20. […] It has been well documented in the literature that pyoderma gangrenosum is usually associated to many different diseases such as inflammatory bowel disease, autoimmune diseases, and hematologic malignancies but in our case we did not find any other diseases associated to PG. […] Understanding and early diagnosis of this dermatoses is crucial in order to provide the best health support for our patients.

• #17 Pyoderma Gangrenosum of the Breast: A Rare Location of Neutrophilic Dermatoses

  https://clinmedjournals.org/articles/cmrcr/clinical-medical-reviews-and-case-reports-cmrcr-9-403.php/1000

  Pyoderma gangrenosum (PG) is a rare entity belonging to the neutrophilic dermatoses. […] The location to the breast represents is unusual, poorly documented in the literature and whose diagnosis remains difficult to establish. […] The positive diagnosis is clinical; however it remains difficult to establish since there are no precise diagnostic criteria and many other diseases can have the same clinical aspect. […] The diagnosis can be challenging, since there are no defined criteria for PG, it is often considered as a skin infection which results in a delay in the diagnosis and treatment. […] Different scores and guidelines were proposed for the diagnosis: The mayo diagnostic criteria in 2004, the international Delphi Criteria and PARACELSUS score in 2018, all had major and minor criteria but in 2021 a cross-sectional retrospective cohort study found that the PARACELSUS score was the most accurate and precise tool to use in the diagnosis of PG.

• #18 Pyoderma gangrenosum: Understanding the difficult diagnosis | MDedge

  https://www.mdedge.com/content/pyoderma-gangrenosum-understanding-difficult-diagnosis

  Several diagnostic frameworks exist: the Su Criteria, the PARACELCUS Score, and the Delphi Consensus Criteria. The Delphi criteria identified the highest percentage of cases (89%) in a study comparing the three, published in 2020. The frameworks “are helpful in the clinic, but they are not to be used as criteria for diagnosis. They’re really for classification,” Dr. Callen said.

• #19 Pyoderma gangrenosum – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pyoderma-gangrenosum/diagnosis-treatment/drc-20350392

  Your healthcare professional will talk with you about your symptoms and medical history and conduct a physical exam. No test can confirm a diagnosis of pyoderma gangrenosum. But you may need tests to rule out other conditions that have similar symptoms. These may include blood tests, a chest X-ray, a colon exam or a skin biopsy. A biopsy is a procedure to remove a sample of tissue for testing in a lab. […] Correct and early diagnosis is key to effective treatment. You may be referred to a specialist in skin conditions. This type of doctor is called a dermatologist. […] You’re likely to first see your regular healthcare professional. You may then be referred to a doctor who specializes in skin conditions. This type of doctor is called a dermatologist. […] Did you have a skin biopsy?

• #20 Pyoderma Gangrenosum: Causes, Symptoms & Treatments

  https://my.clevelandclinic.org/health/diseases/17825-pyoderma-gangrenosum-pg

  Pyoderma gangrenosum can be challenging to diagnose. No single test can confirm a diagnosis, so healthcare providers often order tests to rule out other possible causes of skin problems. These diseases include: […] These tests may include: […] Pyoderma gangrenosum is difficult to diagnose, so its important to have realistic expectations. You may need to see several specialists and undergo many tests to rule out other conditions. This may delay proper treatment. […] If you have pyoderma gangrenosum, its important to discuss your diagnosis with any healthcare provider whos considering a procedure that may damage your skin, such as surgery. They must consider if the procedures benefits outweigh the risks of triggering pyoderma gangrenosum. If you must have the procedure, theyll need to closely supervise you to prevent any new ulcers from developing.

• #21 Pyoderma gangrenosum – a guide to diagnosis and management 

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6542232/

  More recently, Maverakis et al have proposed new criteria based on a consensus of international experts, requiring one major and four minor criteria. […] The biopsy should include the active border of the ulcer and penetrate deep to subcutaneous tissues. […] The histological findings can be variable and depend on the site of the biopsy and age of the lesion. […] PG remains a clinical and sometimes challenging diagnosis and although histology of skin biopsies can be supportive, the main value of the skin biopsy is to exclude other causes of cutaneous ulceration and to allow specimens to be sent for bacterial, mycobacterial and fungal cultures.

• #22 Pyoderma Gangrenosum Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/1123821-workup

  The histopathologic findings in pyoderma gangrenosum are not specific. Nevertheless, a biopsy is suggested in almost all instances because it is useful for excluding other diseases (eg, infections and malignancy). Microscopic features of pyoderma gangrenosum include massive neutrophilic infiltration, hemorrhage, and necrosis of the overlying epidermis. Histologically, this finding may simulate an abscess or cellulitis, though no organisms are seen and cultures generally are negative or demonstrate only typical skin flora. Neutrophils are often around and within the vessel walls, but the full picture of vasculitis is usually absent.

• #22 Pyoderma Gangrenosum Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/1123821-workup

  Pyoderma gangrenosum is a diagnosis of exclusion, in that no specific criteria have been established for confirming the diagnosis. All other potential causes of similar lesions must be excluded before the diagnosis of pyoderma gangrenosum is made. […] Routine blood work to evaluate for an underlying systemic illness in persons with pyoderma gangrenosum includes a complete blood count (CBC), a comprehensive chemistry profile (including a liver function test), and a urinalysis. In addition, a hepatitis profile should be performed. […] Tissue cultures of the ulcer or erosion for bacteria, fungi, atypical mycobacteria, and viruses are needed. The exact cultures to be performed depend on the individual situation. The cultures should be held for 6 weeks because some of the potential agents may take that long to grow in culture.

• #23 Pyoderma Gangrenosum: A Diagnostic Challenge for the Surgical Consultant | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n1/harrington-pyoderma-gangrenosum/

  Pyoderma gangrenosum is an inflammatory neutrophilic dermatitis characterized by the formation of small pustules which rapidly progress to ulcerating wounds. […] Diagnosis is made based on clinical signs, including erythema, undermined borders, multiple ulcers, and history of inflammatory bowel disease (IBD), along with biopsy characteristics, as defined in the Delphi criteria. […] Historically, diagnosis of PG was one of exclusion; however, in 2018, a panel of experts released a Delphi consensus of diagnostic criteria consisting of one major and eight minor criteria. […] This panel determined the single major criteria for diagnosis to be the presence of neutrophilic infiltrate on a biopsy taken from an ulcer edge. […] In testing criteria against a series of known cases and mimickers, these criteria demonstrated an 86% sensitivity and a 90% specificity.

• #24 Pyoderma Gangrenosum: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pyoderma-gangrenosum-pro

  Investigations are needed to exclude other conditions and look for associated disease. Infection, vascular disorders and malignancies in particular need to be excluded. […] The diagnosis is clinical, and a diagnosis of exclusion. […] Blood tests for any underlying systemic condition include: FBC, inflammatory markers, LFTs, urine protein and rheumatological investigations may be appropriate. […] Autoantibodies – patients with PG are often p-ANCA (perinuclear) positive, particularly if inflammatory bowel disease is present. The presence of c-ANCA (cytoplasmic) may indicate GPA. […] Endoscopy may be required to confirm or exclude inflammatory bowel disease. […] Swabs and cultures of the ulcer. […] Biopsy of the lesion – often indicated to exclude other causes, although there are no specific diagnostic features of PG. Biopsy may sometimes cause extension of the ulcer.

• #25 Pyoderma Gangrenosum: What to Include in the Differential Diagnosis

  https://www.patientcareonline.com/view/pyoderma-gangrenosum-what-include-differential-diagnosis

  Pyoderma gangrenosum (PG) is a chronic, recurrent condition characterized by cutaneous ulceration. […] Although these lesions have a characteristic presentation, the differential diagnosis of PG is wide-ranging. […] It is prudent to send skin biopsy specimens for both histologic assessment and tissue culture. […] Because PG is frequently associated with underlying systemic disorders, a thorough work-up is warranted in patients presenting with this condition. The work-up should include studies of the GI tract, serologic studies, radiographic examination, complete blood cell count, bone marrow aspirate and biopsy, and serum and urine protein electrophoresis and immunoelectrophoresis.

• #26 Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

  https://www.mdpi.com/2571-8800/4/3/28

  Diagnostic criteria have been proposed to aid in the earlier recognition of PG, which will be discussed later in this review. […] The diagnosis of PG is difficult for several reasons. These lesions are similar in appearance to other neutrophilic dermatoses such as Sweet Syndrome and subcorneal pustular dermatosis and can also mimic necrotizing skin infections, vasculitis, arterial and venous insufficiency ulcers, cryoglobulinemia, hematologic issues such as Sickle cell disease, antiphospholipid antibody syndrome, calciphylaxis and even cutaneous Crohn’s disease, making the differential diagnosis for PG quite large and resulting in significant delays in recognition and treatment. […] A lack of standardized diagnostic and histological criteria adds an additional difficulty to recognizing PG lesions, thus making it a clinical diagnosis.

• #27 Pyoderma Gangrenosum — Pediatric EM Morsels

  https://pedemmorsels.com/pyoderma-gangrenosum/

  Diagnosis is often delayed. […] It is a diagnosis of exclusion. […] There is no specific serologic or radiographic markers for it. […] Biopsy often performed to rule out other causes (ex, malignancy, infection, vasculitis). […] Neutrophilic dermal infiltration demonstrated. […] Other conditions to consider (abridged): […] Infection […] Cutaneous anthrax […] Atypical myobacterium […] Traumatic ulceration […] Cutaneous malignancy […] Vascular insufficiency […] Behcets disease […] Vasculitis.

• #28 Pyoderma Gangrenosum – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/pyoderma-gangrenosum/

  A general, simple workup is performed, but as there is no biological nor immunological marker for PG, it is a diagnosis of exclusion. PG may be erroneously underdiagnosed, which leads to potentially harmful surgical interventions, or overdiagnosed, which may lead to unnecessary anti-inflammatory / immunosuppressive therapy with its attendant risks. The differential includes vascular conditions, infections, malignancies, and artefactual dermatoses.

• #29 Gap of knowledge in diagnosis of pyoderma gangrenosum in clinical specialties education

  https://www.degruyter.com/document/doi/10.1515/dx-2020-0153/html?lang=en

  Pyoderma gangrenosum (PG) is a chronic ulcerative neutrophilic dermatosis. It presents a diagnostic challenge due to the absence of disease-specific markers or histopathology, lack of universally accepted diagnostic criteria, and many mimicking diseases including necrotizing soft tissue infections (NSTI). PG cases often present first to specialties other than dermatology. […] We found an underrepresentation of PG among major textbooks and journals in clinical specialties, especially in differentiating PG from its mimickers. Misdiagnosis and resultant mismanagement of PG can lead to disastrous outcomes. […] We recommend that PG be added to the differential diagnoses of chronic ulcers in educational resources. We also suggest the addition of identification and differentiation of PG to learning materials and lectures for providers in specialties who may encounter PG, NSTI, or similarly presenting diseases to address this gap.

• #30 A challenging diagnosis: case report of extensive pyoderma gangrenosum | CCID

  https://www.dovepress.com/a-challenging-diagnosis-case-report-of-extensive-pyoderma-gangrenosum–peer-reviewed-fulltext-article-CCID

  Pyoderma gangrenosum (PG) is a rare dermatological condition characterized by the rapid progression of a painful, necrolytic ulcer with an irregular, undermined border and commonly affects the lower extremities, mainly in the pretibial area. The diagnosis of PG is not easy. Due to lack of diagnostic laboratory test and histopathological findings indicative of PG, it is often misdiagnosed as an infection. This results in delayed or inappropriate treatment of the condition, which leads to devastating consequences such as limb amputation and death. […] PG is a diagnosis of exclusion and can only be made after common causes of ulcers such as infection and malignancy have been ruled out. However, the management of suspected infected or malignant ulcers may require debridement or excision, which can exacerbate PG through a process called pathergy (a term used to describe an exaggerated skin injury occurring after trauma).

• #31 Pyoderma gangrenosum – Wikipedia

  https://en.wikipedia.org/wiki/Pyoderma_gangrenosum

  Diagnosis of PG is challenging owing to its variable presentation, clinical overlap with other conditions, association with several systemic diseases, and absence of defining histopathologic or laboratory findings. Misdiagnosis and delayed diagnosis are common. It has been shown that up to 39% of patients who initially received a diagnosis of PG have an alternative diagnosis. […] In addition to a biopsy demonstrating a neutrophilic infiltrate, patients must have at least 4 minor criteria to meet diagnostic criteria. These criteria are based on histology, history, clinical examination and treatment. […] Histology: Exclusion of infection (including histologically indicated stains and tissue cultures) […] Pathergy (ulcer occurring at sites of trauma, with ulcer extending past area of trauma)

• #32 Pyoderma Gangrenosum Differential Diagnoses

  https://emedicine.medscape.com/article/1123821-differential

  Pyoderma gangrenosum is often misdiagnosed, and multiple attempts at grafting often occur before the diagnosis is made. The breakdown at the harvest site is a clue to the diagnosis and is an indicator of the pathergy often seen in active pyoderma gangrenosum patients. […] In addition to the conditions listed in the differential diagnosis of pyoderma gangrenosum, the following should be considered: […] Skin ulcers misdiagnosed as pyoderma gangrenosum.

• #33 Pyoderma Gangrenosum

  https://practicingclinicians.com/the-exchange/pyoderma-gangrenosum-

  A patient presents to your clinic with a painful, rapidly expanding ulcer on a lower extremity. If pyoderma gangrenosum (PG) isn’t on your differential, it should be. Pyoderma gangrenosum is a rare, reactive neutrophilic dermatosis that, if undiagnosed, can be life-altering and potentially limb-threatening. […] Often initially mistaken for infection, PG is considered a diagnosis of exclusion. Clinically, the patient may present with a history of a pustule or pustular plaque rapidly expanding into a painful ulceration, sometimes preceded by trauma. […] A biopsy of the active border of the ulcer should be performed and sent for hematoxylin and eosin (HE) staining, as well as tissue culture sent for bacterial, fungal, and atypical mycobacterial pathogens. […] Although rare, it is important for every clinician to consider PG in the differential when working up a patient with a rapidly expanding ulceration, as a delay in diagnosis can be devastating. Understanding of the associated conditions seen with PG is critical in providing the patient with the thorough evaluation they require.

• #34 Pyoderma Gangrenosum on the Lower Extremity | Consultant360

  https://www.consultant360.com/photoclinic/pyoderma-gangrenosum-lower-extremity

  A 53-year-old woman presented for a dermatology consult for a spreading ulcer on the lateral left calf. […] The clinical presentation is important because pyoderma gangrenosum is a diagnosis of exclusion and no definitive diagnostic serology or histology exists. […] Early diagnosis of pyoderma gangrenosum is crucial because it can significantly reduce patient morbidity potentially caused by unnecessary treatment and interventions as well as disfiguring scarring, psychological consequences, and multiple care visits. […] A diagnosis of pyoderma gangrenosum is often delayed for multiple reasons, including variable presentation, more common acute or severe cutaneous disorders that mimic pyoderma gangrenosum, and relative rarity of these lesions in primary and acute care settings.

• #35 How to Save a Limb: Identification of Pyoderma Gangrenosum | MDedge

  https://mdedge.com/cutis/article/241235/infectious-diseases/how-save-limb-identification-pyoderma-gangrenosum

  A diagnosis of PG can be made if the patient meets 1 major criterion and 4 minor criteria. […] Although our patient’s biopsy demonstrated a mixed infiltrate, PG was not excluded due to spontaneous resolution at the time of biopsy, emphasizing the need to biopsy subsequent new lesions if neutrophils are not initially seen. […] The diagnosis of PG is challenging because it relies heavily on clinical signs and often mimics SSTI. Gathering a detailed medical history is critical to make the diagnosis of PG.

• #36 Pyoderma Gangrenosum – PACES

  https://www.medicaleducationleeds.com/paces/pyoderma-gangrenosum/

  Pyoderma gangrenosum should be considered in any non-healing ulcer or wound. […] It is a diagnosis of exclusion: histology is variable and often nonspecific: neutrophil infiltration, epidermis necrosis. […] Refer urgently to a dermatologist for diagnosis and treatment.

• #37 Usefulness of Ultrasound in the Diagnosis and Follow-up of Pyoderma Gangrenosum | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-usefulness-ultrasound-in-diagnosis-follow-up-articulo-S1578219017303360

  A 78-year-old woman, with a past history of systemic hypertension and a chronic myeloproliferative syndrome, came to the dermatology emergency room for the presence of 2 painful, erythematous-purpuric lesions that had arisen on her left lower limb a week earlier. […] Additional tests included skin ultrasound with a linear 18MHz probe (Esaote MylabGold 25), which revealed a well-defined, hypoechoic subepidermal structure measuring 3.291.14cm. […] Based on these findings, we made a diagnosis of chronic myeloproliferative syndrome-associated pyoderma gangrenosum (PG) and started treatment with oral prednisone at a dose of 1mg/kg/d. […] The correlation between the ultrasound and pathology findings helps to confirm the clinical diagnosis. […] Different diagnostic criteria have been proposed for PG, but the exclusion of other similar diseases is always an important consideration, with ultrasound study being particularly important for this purpose.

• #38 Computer-Assisted Differential Diagnosis of Pyoderma Gangrenosum and Venous Ulcers with Deep Neural Networks

  https://www.mdpi.com/2077-0383/11/23/7103

  Nonetheless, failed or delayed diagnosis is common and represents a substantial risk for a worse clinical outcome. […] Therefore, a prompt diagnosis and treatment are very important in PG and health professionals should be aware of this disease. Supporting an early diagnosis of PG via the use of artificial intelligence might, therefore, be of substantial benefit for the patients.

• #39 A challenging diagnosis: case report of extensive pyoderma gangrenosum | CCID

  https://www.dovepress.com/a-challenging-diagnosis-case-report-of-extensive-pyoderma-gangrenosum–peer-reviewed-fulltext-article-CCID

  Diagnosis of PG in our patient was extremely difficult, made more complicated by the fact that her initial tissue cultures grew Staphylococcus and Enterobacter. As a result, she was placed on antibiotics and underwent repeated surgical debridement for a presumed diagnosis of a wound infection. Diagnosis of PG was considered only after repeated biopsies revealed dermal neutrophilia, failure of treatment with broad-spectrum antibiotics, and most importantly, the wound exhibited pathergy following debridement. However, multiple debridements of her ulcers and the delay in instituting appropriate treatment led to extensive ulcerations and prolonged recovery for the patient. […] Delayed diagnosis or misdiagnosis of PG will certainly lead to exacerbation and progression of the disease. In our case, misdiagnosis of the patients condition resulted in multiple debridements of her ulcers and a delay in instituting appropriate treatment. The outcome was extensive ulcerations and prolonged hospital recovery.

• #40 Pyoderma Gangrenosum (PG) | OHSU

  https://www.ohsu.edu/dermatology/pyoderma-gangrenosum-pg

  Pyoderma gangrenosum (PG) is a rare skin condition characterized by open sores or wounds that develop on the skin (skin ulcers). […] Diagnosing pyoderma gangrenosum (PG) can be challenging in clinical practice, which often leads to misdiagnosis. PG is rare, there are no specific laboratory or histopathologic markers to test for the disease, and currently there are no diagnostic frameworks that are widely accepted as gold standard. […] At OHSU, diagnosis is made by a thorough testing and exclusion process to rule out other similar conditions such as chronic venous ulcers (CVU) the most common cause of lower extremity ulcers. […] Dr. Ortega is leading efforts to identify specific markers that could be used to minimize the challenge of diagnosing PG worldwide, thus improve patient outcomes. Currently three clinical diagnostic frameworks have been proposed to diagnose PG.

• #41 Pyoderma gangrenosum – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/pyoderma-gangrenosum/

  Pyoderma gangrenosum is a rare treatable cause of skin ulceration. […] No specific test exists to confirm a diagnosis of pyoderma gangrenosum. Diagnosis is based on a combination of clinical assessment, exclusion of other causes of skin ulceration and certain investigations. […] Your doctor will need to exclude other conditions that can look like pyoderma gangrenosum such as venous ulcers, inflammation of blood vessels or skin, infection, injury and cancer. […] Your doctor may consider taking a small sample of skin (biopsy) to be examined under the microscope in a laboratory. This test is helpful in ruling out other causes of skin ulceration. Pyoderma gangrenosum does not have a specific appearance under the microscope however there is usually an abundance of white blood cells (neutrophils).

• #42 Pyoderma gangrenosum: a review of the clinical, mechanistic and therapeutic landscape :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-30-number-1/pyoderma-gangrenosum-review-clinical-mechanistic-and-therapeutic-landscape

  Pyoderma gangrenosum (PG) remains a difficult condition to diagnose, mainly because it was previously seen as a diagnosis of exclusion, although newer diagnostic criteria have been proposed in order to overcome this. […] The diagnosis of PG can often be challenging, with many cases being initially misdiagnosed. This is because PG shares some overlapping features with other diseases, and there are no diagnostic histological or laboratory investigations. PG has traditionally been a diagnosis of exclusion although, recently, there have been two proposed criteria for PG diagnosis known as the Delphi consensus and the PARACELSUS score. […] The Delphi consensus has a sensitivity of 86% and specificity of 90%. It requires identification of neutrophilic infiltrate within biopsy of the ulcer edge as its sole major criteria.

• #43 Challenges in Early Detection and Management of Pyoderma Gangren

  https://www.iomcworld.com/open-access/challenges-in-early-detection-and-management-of-pyoderma-gangrenosum-case-study-123552.html

  Early detection and accurate diagnosis are vital for appropriate management, but it is often hindered by the wide spectrum of differential diagnoses and the lack of specific diagnostic tests. […] The diagnostic criteria proposed by the international experts Delphi consensus play a crucial role in establishing the diagnosis of PG.

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