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American Journal of Clinical Dermatology Jul 2024Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that can be associated with primary immunodeficiency. The pathogenesis of PG has not yet been...
BACKGROUND AND OBJECTIVE
Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that can be associated with primary immunodeficiency. The pathogenesis of PG has not yet been elucidated, although contributions from dysregulation of the immune system in patients with apparent genetic predispositions have been postulated. We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-guided systematic review with the objective of identifying inborn errors of immunity in the presence of PG as well as their clinical characteristics of severity including number of PG lesions and anatomic areas affected, and treatment outcomes.
METHODS
A literature search was performed using PubMed/MEDLINE, Embase, Cochrane Library, and Web of Science through August 24, 2023, for studies published in English using the search terms: "pyoderma gangrenosum," "inborn error of immunity," "immune defect*," and a list of genetic mutations potentially associated with PG.
RESULTS
Seventy-four cases of PG associated with inborn errors of immunity were identified. The results demonstrate an association of PG with a variety of inborn errors of immunity, including genetic mutations not classically associated with the condition. Genetic mutations such as BTK, IL1RN, ITGB2, LPIN2, MEFV, NFkB1, NLRP3, NLRP12, NOD2, PSMB8, PLCG2, PSTPIP1, RAG1, TTC37, and WDR1, as well as complement component 2/complement component 4 (C2/C4) and complement component 7 (C7) deficiencies were identified in the presence of either idiopathic or syndromic PG. Of note, mutations in genes such as PSMB8, NLRP3, and IL1RN were found to be associated with a more severe and atypical course of PG, whereas mutations in RAG1 as well as those causing a C2/C4 deficiency were associated with the mildest clinical presentations of PG. Mutations in NFkB1, ITGB2, and PSTPIP1 were associated with the most heterogeneous clinical presentations.
CONCLUSIONS
Human inborn errors of immunity may be implicated in the genetic predisposition to PG and may influence the clinical presentation. Due to the rarity of these diseases, further work must be done to describe the association between inborn errors of immunity and PG. Identifying inborn errors of immunity that may contribute to the development of PG may assist in further elucidating the mechanism of PG, guiding targeted treatment, and improving clinical outcomes for these patients.
PubMed: 38951460
DOI: 10.1007/s40257-024-00875-y -
Dermatologie (Heidelberg, Germany) Jun 2024
PubMed: 38935288
DOI: 10.1007/s00105-024-05384-x -
Pharmaceuticals (Basel, Switzerland) Jun 2024is a plant of the Cannabaceae family, whose molecular composition is known for its vast pharmacological properties. Cannabinoids are the molecules responsible for... (Review)
Review
is a plant of the Cannabaceae family, whose molecular composition is known for its vast pharmacological properties. Cannabinoids are the molecules responsible for potential effects, especially tetrahydrocannabinol and cannabidiol. Scientific development has shown interest in the potential of cannabidiol in various health conditions, as it has demonstrated lower adverse events and great pharmacological potential, especially when administered topically. The present study aims to carry out a scoping review, focusing on the use of cannabidiol, in vivo models, for topical administration. Thus, the methodological approach used by the Joanna Briggs Institute was applied, and the studies were selected based on previously established inclusion criteria. Even though more information regarding the dose to achieve pharmacological potential is still needed, cannabidiol demonstrated potential in treating and preventing different conditions, such as glaucoma, atopic dermatitis, epidermolysis bullosa, and pyoderma gangrenosum.
PubMed: 38931415
DOI: 10.3390/ph17060748 -
Medicina Clinica Jun 2024
PubMed: 38926039
DOI: 10.1016/j.medcli.2024.03.031 -
The Journal of Dermatology Jun 2024
PubMed: 38923560
DOI: 10.1111/1346-8138.17357 -
JAMA Dermatology Jun 2024
PubMed: 38922593
DOI: 10.1001/jamadermatol.2024.1775 -
Cureus May 2024A patient on long-term glucocorticoid therapy for peristomal pyoderma gangrenosum (PPG) who developed mucormycosis (MM) of the wound with dissemination was presented....
A patient on long-term glucocorticoid therapy for peristomal pyoderma gangrenosum (PPG) who developed mucormycosis (MM) of the wound with dissemination was presented. The importance of skin biopsy, together with clinical evaluation in patients with PPG who are resistant to conventional therapy or who develop new symptoms related to their PPG is stressed. The risk and pathogenesis of invasive fungal infections with long-term corticosteroid therapy were explored. The epidemiology and detection of mucormycosis is discussed in this article.
PubMed: 38919219
DOI: 10.7759/cureus.61137 -
Journal of the American Academy of... Jun 2024
Review
PubMed: 38906261
DOI: 10.1016/j.jaad.2024.05.086 -
International Wound Journal Jun 2024Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains...
Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.
Topics: Humans; Pyoderma Gangrenosum; Male; Female; Skin Transplantation; Middle Aged; Aged; Wound Healing; Adult; Treatment Outcome; Aged, 80 and over; Retrospective Studies; Skin Ulcer; Vasculitis
PubMed: 38899689
DOI: 10.1111/iwj.14943 -
International Journal of Dermatology Jun 2024
PubMed: 38880986
DOI: 10.1111/ijd.17330