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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 -
Expert Review of Clinical Pharmacology Feb 2020: Pyoderma gangrenosum (PG) is a noninfectious, reactive inflammatory neutrophilic dermatosis that is commonly associated with autoimmune and neoplastic disorders. There... (Review)
Review
: Pyoderma gangrenosum (PG) is a noninfectious, reactive inflammatory neutrophilic dermatosis that is commonly associated with autoimmune and neoplastic disorders. There are emerging diagnostic tools and treatment options for PG.: The diagnosis of PG should be seriously considered when managing ulcers to avoid unnecessary medical and surgical complications with prompt and suitable treatment. There are no standardized treatment guidelines for PG, and current therapy largely depends on the severity and progression of the disease. Systemic corticosteroids, immunosuppressant therapy, and biologic agents remain mainstay therapies. In this article, we present a literature review of recent diagnostic and novel treatment options for the management of PG. The literature research considered clinical studies or scientific reviews. Studies were identified by searching electronic databases and reference lists of respective articles till August 2019.: The true diagnosis of PG is challenging, as there is no diagnostic gold standard. PARACELSUS is a novel diagnostic tool. Biologics and small molecules are emerging systemic therapy options that are relatively new in treatment of PG.
Topics: Adrenal Cortex Hormones; Biological Factors; Dermatologic Agents; Disease Progression; Humans; Immunosuppressive Agents; Pyoderma Gangrenosum; Severity of Illness Index
PubMed: 31875484
DOI: 10.1080/17512433.2020.1709825 -
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 -
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 -
Drugs of Today (Barcelona, Spain : 1998) Sep 2021Pyoderma gangrenosum is a refractory disease characterized by chronic ulcers, presenting with central deep ulceration and undermined borders predominantly involving the...
Pyoderma gangrenosum is a refractory disease characterized by chronic ulcers, presenting with central deep ulceration and undermined borders predominantly involving the lower extremities. Oral prednisolone and/or cyclosporine has been considered to be a first line of therapy; however, there are still unmet needs for treatment. Recently, effects of adalimumab for pyoderma gangrenosum in 22 Japanese patients were examined in an open-label, multicenter study during a 26-week treatment period and a further 26-week extension period. Pyoderma gangrenosum area reduction 100 (PGAR 100, defined as complete skin re-epithelialization) response for the target ulcer was observed in 3 patients (13.6%) as early as week 6, and at week 26, 12 patients (54.5%) achieved the primary endpoint of PGAR 100. The mean percent change from baseline in target ulcer area was -63.8% at week 26. A physician's global assessment score of 0 (PGA 0) was achieved by 8 patients (36.4%), while PGA 0/1 (completely/almost clear) was achieved by 12 patients (54.5%) at week 26. Adverse events were reported by 18 patients, most commonly infections (n = 11) and serious adverse events (n = 4). These results suggest that adalimumab is effective and generally well tolerated in Japanese patients with pyoderma gangrenosum active ulcers.
Topics: Adalimumab; Humans; Pyoderma Gangrenosum
PubMed: 34586101
DOI: 10.1358/dot.2021.57.9.3293619 -
The Journal of Dermatology Feb 2024Pyoderma gangrenosum (PG), hidradenitis suppurativa (HS), and the associated autoinflammatory syndromes, including pyogenic arthritis, pyoderma gangrenosum, and acne... (Review)
Review
Pyoderma gangrenosum (PG), hidradenitis suppurativa (HS), and the associated autoinflammatory syndromes, including pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome, PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome, and pyogenic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis (PAPASH) syndrome are dermatological conditions characterized by chronic inflammation and tissue damage. Recent advances in genetic research have identified specific mutations associated with these disorders, shedding light on their underlying pathogenic mechanisms. This review aims to summarize the current knowledge of identified mutations and presumed pathophysiology in PG, HS, and the associated autoinflammatory syndromes.
Topics: Humans; Hidradenitis Suppurativa; Pyoderma Gangrenosum; Acne Vulgaris; Syndrome; Mutation; Arthritis, Infectious
PubMed: 38031879
DOI: 10.1111/1346-8138.17028 -
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 -
International Wound Journal Dec 2022
Topics: Humans; Pyoderma; Pyoderma Gangrenosum
PubMed: 35560865
DOI: 10.1111/iwj.13828 -
Clinics in Dermatology 2021Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and... (Review)
Review
Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and or/hypodermis by neutrophils; (2) their association with distinct diseases (eg, hematologic malignancy and chronic inflammatory diseases); (3) potential extracutaneous involvement; and (4) response to anti-inflammatory drugs, such as corticosteroids, dapsone, colchicine, and novel biologic therapies, such as the anti-interleukin-1 blockade. Although distinct NDs have been described, transitional forms with overlapping features are often identified. These justify a simplified classification of NDs with three major forms: superficial (epidermal or pustular) NDs, dermal (en plaques) NDs, and deep NDs. We review selected or novel variants of NDs, including subcorneal pustular dermatosis, the group of immunoglobulin A neutrophilic dermatoses, amicrobial pustular dermatosis of the folds, and neutrophilic urticarial dermatosis, as well as atypical forms of Sweet syndrome and pyoderma gangrenosum closely mimicking severe infectious diseases. Knowledge of these variants is essential for proper diagnosis, adequate management, and avoidance of a dangerous escalation of therapy, such as unnecessary immunosuppression or extensive surgery.
Topics: Anti-Inflammatory Agents; Dermatitis; Humans; Neutrophils; Pyoderma Gangrenosum; Skin Diseases, Vesiculobullous; Sweet Syndrome
PubMed: 34272020
DOI: 10.1016/j.clindermatol.2020.10.012 -
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