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Journal of the American Academy of... Jun 2024
Review
PubMed: 38906261
DOI: 10.1016/j.jaad.2024.05.086 -
Journal of Cutaneous Medicine and... 2024
Topics: Humans; Pyoderma Gangrenosum; Sulfasalazine; Anti-Inflammatory Agents, Non-Steroidal
PubMed: 38462891
DOI: 10.1177/12034754241238713 -
Frontiers in Immunology 2023This scoping review explores the effectiveness of IL-1 pathway inhibitors in managing PSTPIP1-associated inflammatory diseases (PAID). These diseases are marked by...
INTRODUCTION
This scoping review explores the effectiveness of IL-1 pathway inhibitors in managing PSTPIP1-associated inflammatory diseases (PAID). These diseases are marked by abnormal IL-1 pathway activation due to genetic mutations.
METHODS
Our methodology adhered to a pre-published protocol and involved a thorough search of MEDLINE and EMBASE databases up to February 2022, following the Joanna Briggs Institute Reviewer's Manual and the PRISMA Extension for Scoping Reviews. The review included studies reporting on IL-1 pathway inhibitor use in PAID patients.
RESULTS
From an initial pool of 5,225 articles, 36 studies involving 43 patients were selected. The studies predominantly used observational designs and exhibited diversity in patient demographics, treatment approaches, and outcomes. Anakinra and canakinumab demonstrated promise in treating sterile pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) and PSTPIP1-associated myeloid-related-proteinemia inflammatory (PAMI) syndromes, with scant data on other syndromes. Notably, there was a paucity of information on the adverse effects of these treatments, necessitating cautious interpretation of their safety profile.
CONCLUSION
Current evidence on IL-1 pathway inhibitors for PAID is primarily from observational studies and remains limited. Rigorous research with larger patient cohorts is imperative for more definitive conclusions. Collaborative efforts among specialized research centers and international health initiatives are key to advancing this field.
Topics: Humans; Acne Vulgaris; Adaptor Proteins, Signal Transducing; Antibodies, Monoclonal, Humanized; Arthritis, Infectious; Cytoskeletal Proteins; Interleukin 1 Receptor Antagonist Protein; Interleukin-1
PubMed: 38259483
DOI: 10.3389/fimmu.2023.1339337 -
Metabolites Jun 2023Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for... (Review)
Review
Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for obesity. Over the years, the number of reported cases associated with medical gastroenterological conditions, particularly inflammatory bowel disease (IBD), has progressively increased. To date, there are no systematic reviews in the literature on BADAS. The design of an a priori protocol was based on PRISMA guidelines, and a search of PubMed and Scopus databases was conducted for articles published between 1971 and 2023 related to the topic. Fifty-one articles including 113 patients with BADAS were analyzed in this systematic review. Bariatric surgery and IBD were the most frequently reported causes of BADAS, accounting for 63.7% and 24.7% of all cases, respectively. A total of 85% of cases displayed the typical dermatological presentation, including urticarial maculopapular lesions centered by a vesicopustule, with the majority of lesions located on the upper limbs (73.5%). Polyarthralgia or localized arthritis were always present. Atypical presentations included cellulitis-like, erythema-nodosum-like, Sweet-syndrome-like and pyoderma-gangrenosum-like manifestations. Gastrointestinal symptoms were frequently observed in IBD-related cases (67.9%). The histopathology showed a neutrophilic infiltrate (96.6%). The most commonly used treatment regimens consisted of systemic corticosteroids, metronidazole and tetracyclines, either alone or in combination. A relapsing-remitting course was observed in 52.1% of patients. In conclusion, BADAS is a neutrophilic dermatosis that presents with a wide variety of cutaneous manifestations, both typical and atypical. Gastrointestinal symptoms are frequently observed, particularly in cases related to IBD. The histopathology is clear but not specific compared with other neutrophilic dermatoses. The diagnosis can be challenging, but the relapsing-remitting course and the strong association with polyarthralgia and gastrointestinal disease can aid in the diagnosis.
PubMed: 37512497
DOI: 10.3390/metabo13070790 -
Frontiers in Immunology 2022Hidradenitis suppurativa were associated with comorbidities in various organ systems. Inflammatory dermatological diseases such as pyoderma gangrenosum were reported to... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Hidradenitis suppurativa were associated with comorbidities in various organ systems. Inflammatory dermatological diseases such as pyoderma gangrenosum were reported to be associated with hidradenitis suppurativa. Nevertheless, as for the association between hidradenitis suppurativa and psoriasis, evidences were insufficient. In many studies, the association between psoriasis and hidradenitis suppurativa has been reported. However, some evidence seems to be controversial. The purpose of the systematic review and meta-analysis was to assess whether there was significant association between HS and psoriasis.
METHODS
On June 01, 2022, we appraised 2,795 articles from databases including PubMed, Web of Science and Embase. Search syntaxes were based on 'hidradenitis suppurativa' or 'acne inversa' with "psoriasis", "comorbidities" or 'epidemiology'. Synonyms were determined based on MeSH terms and Emtree. Observational results that evaluated the odds ratio for people with hidradenitis suppurativa who had psoriasis were extracted for qualitative synthesis.
RESULTS
After the selection process of the initial 2,795 studies, ten observational studies, including 3 cohort studies, 1 case-control study, and 6 cross-sectional studies, were extracted for critical appraisal. Based on the integration of 7 studies (with more than 560,000 participants included), people with hidradenitis suppurativa had a higher risk of having psoriasis, with a 2.67-fold risk (95% CI, 1.84, 3.87). The association remained in the sensitivity analyses utilizing strict adjustment models. In the analysis that only included studies with a similar study design and adjustments in obesity-related factors, the risk of people with hidradenitis suppurativa having psoriasis was 3.24 (95% CI, 2.27, 4.62). In male patients with HS, the risk of having psoriasis was 4.30-fold higher than male patients without HS (95% CI, 2.37, 7.78). Likewise, in an analysis including 3 cross-sectional studies, the risk of female HS patients having psoriasis was 3.94-fold higher than female HS-free patients (95% CI, 2.34, 6.63).
CONCLUSIONS
The co-occurrence of hidradenitis suppurativa and psoriasis can greatly increase the burden of the disease. Psoriasis could be one of the critical comorbidities of hidradenitis suppurativa and should be recommended for future screening and follow up. The association between the two diseases should be kept in mind in managing hidradenitis suppurativa patients. More prospective studies are needed to establish the true magnitude of the association between psoriasis and hidradenitis suppurativa.
Topics: Humans; Male; Female; Cross-Sectional Studies; Case-Control Studies; Hidradenitis Suppurativa; Psoriasis; Comorbidity
PubMed: 36532043
DOI: 10.3389/fimmu.2022.1033844 -
Pharmaceutics Jan 2022(1) Background: Colchicine is a natural alkaloid with anti-inflammatory properties used to treat various disorders, including some skin diseases. This paper aims to... (Review)
Review
(1) Background: Colchicine is a natural alkaloid with anti-inflammatory properties used to treat various disorders, including some skin diseases. This paper aims to incorporate all the available studies proposing colchicine as a treatment alternative in the management of cutaneous conditions. (2) Methods: In this systematic review, the available articles present in various databases (PubMed, Scopus-Embase, and Web of Science), proposing colchicine as a treatment for cutaneous pathological conditions, have been selected. Exclusion criteria included a non-English language and non-human studies. (3) Results: Ninety-six studies were included. Most of them were case reports and case series studies describing colchicine as single therapy, or in combination with other drugs. Hidradenitis suppurativa, pyoderma gangrenosum, erythema nodosum, erythema induratum, storage diseases, perforating dermatosis, bullous diseases, psoriasis, vasculitis, acne, urticaria, stomatitis, actinic keratosis, and pustular dermatosis were the main diseases discussed in literature. Although the therapeutic outcomes were variable, most of the studies reported, on average, good clinical results (4) Conclusions: Colchicine could be, as a single therapy or in combination with other drugs, a possible treatment to manage several skin diseases.
PubMed: 35214027
DOI: 10.3390/pharmaceutics14020294 -
Frontiers in Immunology 2021This study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China...
OBJECTIVES
This study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.
METHODS
The clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.
RESULTS
A total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 . 83%), whereas arthritis was less common (50 . 98.1%). Steroid and/or biological agents were effective in most patients.
CONCLUSIONS
The rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.
Topics: Acne Vulgaris; Adaptor Proteins, Signal Transducing; Adult; Arthritis, Infectious; Biological Products; Cytoskeletal Proteins; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Humans; Male; Middle Aged; Mutation; Phenotype; Predictive Value of Tests; Pyoderma Gangrenosum; Steroids; Treatment Outcome
PubMed: 34745107
DOI: 10.3389/fimmu.2021.735851 -
Journal of Translational Autoimmunity 2020In the last decade, new scientific findings significantly improved our understanding of the molecular pathogenesis of autoinflammation and have resulted in the... (Review)
Review
In the last decade, new scientific findings significantly improved our understanding of the molecular pathogenesis of autoinflammation and have resulted in the identification and definition of several pyoderma gangrenosum-associated autoinflammatory syndromes (PGAAIS) as new and distinct clinical entities. These different clinical entities include PAPA (pyogenic arthritis, pyoderma gangrenosum and acne conglobata), PASH (pyoderma gangrenosum, acne and suppurative hidradenitis), PAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and pyogenic arthritis), PsAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and psoriatic arthritis), PASS (pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and axial spondyloarthritis) and PAC (pyoderma gangrenosum, acne and ulcerative colitis), which can be distinguished by their clinical presentation and the presence or absence of mutations in several genes, such as the genes encoding proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1), nicastrin (NCSTN), Mediterranean fever (MEFV) and nucleotide-binding oligomerization domain-containing protein (NOD). In this systematic review, we summarize the present knowledge of this rapidly developing hot topic and provide a guide to enable the easy diagnosis of these syndromes in everyday clinical practice. Moreover, we report a rare case of PASS syndrome demonstrating successful treatment with adalimumab and another case of a previously unreported combination of symptoms, including psoriatic arthritis, pyoderma gangrenosum, suppurative hidradenitis and Crohn's disease (newly coined PsAPSC), as examples. Because of the identification of similar genetic and pathogenic mechanisms of PGAAIS, we think the wide variety of seemingly different syndromes may represent distinct phenotypes of one disease.
PubMed: 33305249
DOI: 10.1016/j.jtauto.2020.100071 -
Dermatology (Basel, Switzerland) 2021There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently... (Meta-Analysis)
Meta-Analysis
BACKGROUND
There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients.
METHODS
A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission.
CONCLUSION
The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.
Topics: Hidradenitis Suppurativa; Humans
PubMed: 32942279
DOI: 10.1159/000509873 -
Medicina (Kaunas, Lithuania) Aug 2020Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated...
UNLABELLED
Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime.
MATERIALS AND METHODS
A systemic literature review was performed, investigating articles and case reports on dermatological manifestations in children with IBD starting from 2005. A total of 159 potentially suitable articles were identified and after the exclusion process, 75 articles were selected.
RESULTS
The most common dermatological manifestations reported in pediatric IBD are erythema nodosum and pyoderma gangrenosum. More rare cases of metastatic Crohn's disease, epidermolysis bullosa acquisita, small-vessel vasculitis, necrotizing vasculitis, leukocytoclastic vasculitis, cutaneous polyarteritis nodosa, and Sweet's syndrome have been reported. Oral manifestations of IBD are divided into specific (tag-like lesions, mucogingivitis, lip swelling with vertical fissures, aphthous stomatitis, and pyostomatitis vegetans) and non-specific. IBD treatment may present with side effects involving the skin and mucosa. Anti-tumor necrosis factor agents have been linked to opportunistic skin infections, psoriasiform lesions, and a potentially increased risk for skin cancer. Cutaneous manifestations such as acrodermatitis enteropathica, purpuric lesions, and angular cheilitis may appear secondary to malnutrition and/or malabsorption.
CONCLUSIONS
The correct diagnosis of dermatological manifestations in pediatric IBD is of paramount importance because of their impact on disease activity, treatment options, and a patient's psychological status.
Topics: Biological Factors; Child; Colitis, Ulcerative; Crohn Disease; Humans; Immunosuppressive Agents; Malabsorption Syndromes; Receptors, Tumor Necrosis Factor; Skin Diseases
PubMed: 32842528
DOI: 10.3390/medicina56090425