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International Wound Journal Apr 2019Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to... (Review)
Review
Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to successfully control PG. Our aim was to systematically evaluate and compare the clinical effectiveness of TNFα inhibitors in adults with PG. A literature search including databases such as PubMed, Embase, Scopus, and Web of Science was conducted, using search terms related to PG and TNFα inhibitors. Studies and case reports were included if patients were diagnosed with PG, over the age of 18 and administered TNFα inhibitor. A total of 3212 unique citations were identified resulting in 222 articles describing 356 patients being included in our study. The study we report found an 87% (95% CI: 83%-90%) response rate and a 67% (95% CI: 62%-72%) complete response rate to TNFα inhibitors. No statistically significant differences in the response rates (P = 0.6159) or complete response rates (P = 0.0773) to infliximab, adalimumab, and etanercept were found. In our study TNFα inhibitors demonstrated significant effectiveness with response and complete response rates supporting the use of TNFα inhibitors to treat PG in adults. Our study suggests that there is no significant difference in effectiveness among infliximab, adalimumab, and etanercept.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Antirheumatic Agents; Etanercept; Female; Humans; Immunologic Factors; Infliximab; Male; Middle Aged; Pyoderma Gangrenosum; Tumor Necrosis Factor-alpha
PubMed: 30604927
DOI: 10.1111/iwj.13067 -
International Journal of Molecular... Feb 2024Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG... (Review)
Review
Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG is not fully understood; however, it is most commonly considered a disease in the spectrum of neutrophilic dermatoses. The treatment of PG remains challenging due to the lack of generally accepted therapeutic guidelines. Existing therapeutic methods focus on limiting inflammation through the use of immunosuppressive and immunomodulatory therapies. Recently, several reports have indicated the successful use of biologic drugs and small molecules administered for coexisting diseases, resulting in ulcer healing. In this review, we summarize the discoveries regarding the pathophysiology of PG and present treatment options to raise awareness and improve the management of this rare entity.
Topics: Humans; Pyoderma Gangrenosum; Immunosuppressive Agents; Inflammation; Biological Products; Immunomodulation
PubMed: 38397117
DOI: 10.3390/ijms25042440 -
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 -
Orphanet Journal of Rare Diseases Apr 2007Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis. Clinically it starts with sterile pustules that rapidly progress and turn into painful ulcers... (Review)
Review
Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis. Clinically it starts with sterile pustules that rapidly progress and turn into painful ulcers of variable depth and size with undermined violaceous borders. The legs are most commonly affected but other parts of the skin and mucous membranes may also be involved. Course can be mild or malignant, chronic or relapsing with remarkable morbidity. In many cases PG is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatic or haematological disease and malignancy. Diagnosis of PG is based on history of an underlying disease, typical clinical presentation, histopathology, and exclusion of other diseases that would lead to a similar appearance. The peak of incidence occurs between the ages of 20 to 50 years with women being more often affected than men. Aetiology has not been clearly determined yet. The treatment of PG is a challenge. Randomized, double-blinded prospective multicenter trials for PG are not available. The best documented treatments are systemic corticosteroids and cyclosporin A. Combinations of steroids with cytotoxic drugs are used in resistant cases. The combination of steroids with sulfa drugs or immunosuppressants has been used as steroid-sparing modalities. Anti-tumor necrosis alpha therapy in Crohn's disease showed a rapid response of PG. Skin transplants and the application of bioengineered skin is useful in selected cases as a complement to the immunosuppressive treatment. Topical therapy with modern wound dressings is useful to minimize pain and the risk of secondary infections. Despite recent advances in therapy, the prognosis of PG remains unpredictable.
Topics: Administration, Topical; Adolescent; Adrenal Cortex Hormones; Adult; Aged; Anti-Infective Agents; Child; Child, Preschool; Cytotoxins; Diagnosis, Differential; Female; Humans; Immunosuppressive Agents; Infant; Male; Middle Aged; Prognosis; Pyoderma Gangrenosum
PubMed: 17433111
DOI: 10.1186/1750-1172-2-19 -
Indian Journal of Dermatology,... 2017
Review
Topics: Administration, Topical; Dermatologic Agents; Diagnosis, Differential; Humans; Pyoderma Gangrenosum; Skin Diseases
PubMed: 27917844
DOI: 10.4103/0378-6323.195077 -
International Wound Journal Oct 2019Peristomal pyoderma gangrenosum (PPG) is an under-recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that... (Review)
Review
Peristomal pyoderma gangrenosum (PPG) is an under-recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that promotes wound healing and allows for adhesion of the stoma appliance. A crushed oral prednisolone tablet mixed with Stomahesive Protective Powder (ConvaTec) was applied topically to seven patients with PPG and resulted in pain relief and wound healing in six of seven patients. Only one patient experienced recurrence. The novel topical therapy we describe is cost-effective, readily available, and easily applied in any inpatient or outpatient setting.
Topics: Administration, Topical; Aged; Female; Follow-Up Studies; Humans; Male; Middle Aged; Prednisolone; Pyoderma Gangrenosum; Retrospective Studies; Risk Assessment; Sampling Studies; Surgical Stomas; Talc; Wound Healing
PubMed: 31298491
DOI: 10.1111/iwj.13164 -
Head and Neck Pathology Dec 2017Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The... (Review)
Review
Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. The condition can affect any anatomical site, however the head and neck are rarely involved. Although the oral cavity is subject to recurrent minor trauma through everyday activities such as mastication and oral hygiene, as well as during dental treatment, oral lesions appear to be extremely rare. In an effort to provide a detailed explanation of the oral manifestations of PG, a systematic search was conducted using medical databases. A total of 20 cases of PG with oral involvement were reported in the English and French literature. The objectives of this article are to present the pertinent diagnostic criteria and to discuss the differential diagnosis and therapeutic modalities.
Topics: Diagnosis, Differential; Humans; Oral Ulcer; Pyoderma Gangrenosum
PubMed: 28275955
DOI: 10.1007/s12105-017-0804-3 -
International Wound Journal Oct 2020Pyoderma gangrenosum (PG) is a rare disease of unknown aetiology, first described over a century ago. Initially thought to have an infectious cause, and now primarily... (Review)
Review
Pyoderma gangrenosum (PG) is a rare disease of unknown aetiology, first described over a century ago. Initially thought to have an infectious cause, and now primarily considered an autoinflammatory condition, PG continues to be poorly understood, commonly misdiagnosed, and difficult to treat. In this review, we discuss the journey of our understanding of PG to date, including first descriptions, challenges with diagnosis, presumed pathogenesis, and treatments used. We highlight major historical landmarks and their importance, explain the rationale behind current investigations, note outstanding gaps in knowledge, and explore the future directions of PG research. We summarise what we have known, what we are working on knowing, and what we have yet to explore about PG, illustrating overall trends to invigorate future research.
Topics: Humans; Pyoderma Gangrenosum
PubMed: 32378319
DOI: 10.1111/iwj.13389 -
PLoS Neglected Tropical Diseases Oct 2019Scabies is a WHO neglected tropical disease common in children in low- and middle-income countries. Excoriation of scabies lesions can lead to secondary pyoderma...
BACKGROUND
Scabies is a WHO neglected tropical disease common in children in low- and middle-income countries. Excoriation of scabies lesions can lead to secondary pyoderma infection, most commonly by Staphyloccocus aureus and Streptococcus pyogenes (group A streptococcus, GAS), with the latter linked to acute post-streptococcal glomerulonephritis (APSGN) and potentially rheumatic heart disease (RHD). There is a paucity of data on the prevalence of these skin infections and their bacterial aetiology from Africa.
METHODOLOGY/PRINCIPAL FINDINGS
A cross-sectional study, conducted over a four-month period that included the dry and rainy season, was conducted to determine the prevalence of common skin infections in Sukuta, a peri-urban settlement in western Gambia, in children <5 years. Swabs from pyoderma lesions were cultured for S. aureus and GAS. Of 1441 children examined, 15.9% had scabies (95% CI 12.2-20.4), 17.4% had pyoderma (95% CI 10.4-27.7) and 9.7% had fungal infections (95% CI 6.6-14.0). Scabies was significantly associated with pyoderma (aOR 2.74, 95% CI 1.61-4.67). Of 250 pyoderma swabs, 80.8% were culture-positive for S. aureus, and 50.8% for GAS. Participants examined after the first rains were significantly more likely to have pyoderma than those examined before (aRR 2.42, 95% CI 1.38-4.23), whereas no difference in scabies prevalence was seen (aRR 1.08, 95% CI 0.70-1.67). Swab positivity was not affected by the season.
CONCLUSIONS/SIGNIFICANCE
High prevalence of scabies and pyoderma were observed. Pyoderma increased significantly during the rainy season. Given the high prevalence of GAS pyoderma among children, further research on the association with RHD in West Africa is warranted.
Topics: Algorithms; Child, Preschool; Climate; Coinfection; Cross-Sectional Studies; Female; Gambia; Glomerulonephritis; Humans; Infant; Male; Mycoses; Odds Ratio; Prevalence; Pyoderma; Rheumatic Heart Disease; Risk Factors; Scabies; Seasons; Staphylococcal Skin Infections; Staphylococcus aureus; Streptococcal Infections; Streptococcus pyogenes
PubMed: 31609963
DOI: 10.1371/journal.pntd.0007801 -
The Pan African Medical Journal 2020Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary... (Observational Study)
Observational Study
Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary study's aim was to examine all cases of PG observed in our department over a 6-year period in order to describe the relevant characteristics and outcome under therapy. Fourteen patients were included (5 women, 9 men). The average age of our patients was 40,15 years. The classical, ulcerative form was found in 10 cases (71.42%), the pustular form in 4 cases (27.57%) and PG was multifocal in 4 cases. The PG was located preferentially to the lower limbs. Histological examination was realized in all patients and objectified inflammatory infiltrate composed of polymorphonuclear neutrophils in all cases with vasculitis in 4 cases. Six patients (42.85%) had associated disease at diagnosis of PG, including inflammatory bowel disease in two cases (14.28%), a blood disease in 2 cases (14.28%), lymph node tuberculosis and inflammatory arthritis in 1 case (7%). The most frequent first-line treatments were oral corticosteroids (7 cases) and other treatments used were colchicine in 2 cases, topical corticosteroids in 3 cases with good clinical evolution. Our study confirms that PG is a rare disease, associated in almost half of cases with systemic disease already present at diagnosis; in our Moroccan background, it is most often inflammatory bowel disease, hematological or solid cancer and tuberculosis.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Child; Colchicine; Female; Humans; Male; Middle Aged; Prospective Studies; Pyoderma Gangrenosum; Treatment Outcome; Young Adult
PubMed: 32733629
DOI: 10.11604/pamj.2020.36.59.12329