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Skin Health and Disease Apr 2024Myelodysplastic syndrome (MDS) may present with specific skin lesions, such as leukaemia cutis, which is a well known poor prognostic marker of leukaemia with a high...
Myelodysplastic syndrome (MDS) may present with specific skin lesions, such as leukaemia cutis, which is a well known poor prognostic marker of leukaemia with a high risk of acute leukaemic transformation. However, less is known regarding non-specific cutaneous manifestations of MDS including the prevalence, types and their prognostic and therapeutic significance, which we aimed to determine through this systematic review. We searched electronic databases (PubMed, Medline and EMBASE) from inception up to 26 January 2023 for studies reporting cutaneous manifestations of MDS. Eighty eight articles (case reports = 67, case series = 21), consisting of 134 patients were identified. We identified 6 common cutaneous manifestations: neutrophilic dermatoses ( = 64), vasculitis ( = 21), granulomatous ( = 8), connective tissue disease (CTD) ( = 7; composed of dermatomyositis ( = 5), cutaneous lupus erythematosus ( = 1), and systemic sclerosis ( = 1)), panniculitis ( = 4), immunobullous ( = 1), and other ( = 29). Cutaneous features either occurred at time of MDS diagnosis in 25.3%, preceding the diagnosis in 34.7% (range 0.5-216 months), or after diagnosis in 40.0% (range 1-132 months). Prognosis was poor (40.2% death) with 34.1% progressing to acute myeloid leukaemia (AML). 50% of those with MDS who progressed to AML had neutrophilic dermatoses ( = 0.21). Myelodysplastic syndrome was fatal in 39.2% of neutrophilic dermatoses (median time from onset of cutaneous manifestation: 12 months), 50% of vasculitis (7.5 months), 62.5% of granulomatous (15.5 months) and 14.3% of CTD (7 months). Recognition of patterns of cutaneous features in MDS will improve early diagnosis and risk stratification according to subtype and associated prognosis.
PubMed: 38577044
DOI: 10.1002/ski2.323 -
Cancers Feb 2024Cutaneous T-cell lymphomas (CTCLs) are a group of lymphoid neoplasms with high relapse rates and no curative treatment other than allogeneic stem cell transplantation... (Review)
Review
Cutaneous T-cell lymphomas (CTCLs) are a group of lymphoid neoplasms with high relapse rates and no curative treatment other than allogeneic stem cell transplantation (allo-SCT). CTCL is significantly influenced by disruption of JAK/STAT signaling. Therefore, Janus kinase (JAK) inhibitors may be promising for CTCL treatment. This study is a systematic review aiming to investigate the role of JAK inhibitors in the treatment of CTCL, including their efficacy and safety. Out of 438 initially searched articles, we present 13 eligible ones. The overall response rate (ORR) in the treatment with JAK inhibitors in clinical trials was 11-35%, although different subtypes of CTCL showed different ORRs. Mycosis fungoides showed an ORR of 14-45%, while subcutaneous-panniculitis-like T-cell lymphoma (SPTCL) displayed an ORR ranging from 75% to 100%. Five cases were reported having a relapse/incident of CTCL after using JAK inhibitors; of these, three cases were de novo CTCLs in patients under treatment with a JAK inhibitor due to refractory arthritis, and two cases were relapsed disease after graft-versus-host disease treatment following allo-SCT. In conclusion, using JAK inhibitors for CTCL treatment seems promising with acceptable side effects, especially in patients with SPTCL. Some biomarkers, like pS6, showed an association with better responses. Caution should be taken when treating patients with an underlying autoimmune disease and prior immunosuppression.
PubMed: 38473222
DOI: 10.3390/cancers16050861 -
Journal of Plastic, Reconstructive &... Apr 2024Subcutaneous fat necrosis of the newborn (SCFN) is a rare form of panniculitis manifesting as erythematous plaques or nodules at sites of brown fat in neonates. Surgical... (Review)
Review
BACKGROUND
Subcutaneous fat necrosis of the newborn (SCFN) is a rare form of panniculitis manifesting as erythematous plaques or nodules at sites of brown fat in neonates. Surgical management may be indicated in severe cases; however, there is a paucity of literature compiling presentations and outcomes of these surgical patients.
METHODS
The authors performed a systematic review, in consultation with a licensed librarian, on MEDLINE and Embase for studies including patients with SCFN who were surgically managed.
RESULTS
The search strategy generated 705 results, among which 213 (30.2%) were excluded for lack of discussion on surgical management. Twenty-two studies discussed surgical management of SCFN in 26 patients, but in 6 of these studies the patients were not surgically managed. Ultimately, 16 articles with 16 patients who were surgically managed were included in the study. Average age at diagnosis was 11.8 ± 9.8 days; average age at surgery was 39.5 ± 70.4 days. The most common etiologies were "unknown" (6, 37.5%), therapeutic hypothermia (4, 25.0%), and birth complications (4, 25.0%). Patients harbored nodules on the back (14, 87.5%), upper extremities (7, 43.8%), lower extremities (7, 43.8%), buttocks (5, 31.3%), and head or neck (3, 18.8%). Linear regression models revealed the presence of back lesions and predicted concomitant medical complications (β = 2.71, p = 0.021).
CONCLUSIONS
Patients undergoing surgical management for SCFN most commonly harbor lesions on the back and extremities that are secondary to therapeutic hypothermia or of unknown origin. Reporting of additional cases is needed to further elucidate surgical management and outcomes.
Topics: Infant, Newborn; Humans; Infant; Subcutaneous Fat; Fat Necrosis; Panniculitis; Hypothermia, Induced; Buttocks
PubMed: 38442509
DOI: 10.1016/j.bjps.2024.02.027 -
Journal of the American Academy of... Feb 2024
Topics: Humans; Proto-Oncogene Proteins B-raf; Melanoma; Protein Kinase Inhibitors; Panniculitis; Antineoplastic Combined Chemotherapy Protocols; Mutation; Skin Neoplasms; Pyridones; Oximes
PubMed: 37943239
DOI: 10.1016/j.jaad.2023.10.008 -
Autoimmunity Reviews Apr 2023Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known. (Review)
Review
INTRODUCTION
Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known.
METHODS
We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected.
RESULTS
Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control.
CONCLUSION
Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
Topics: Adult; Humans; Dermatomyositis; Subcutaneous Tissue; Autoantibodies; Myositis; Panniculitis; Lipodystrophy
PubMed: 36736986
DOI: 10.1016/j.autrev.2023.103284 -
Journal of Cosmetic Dermatology Oct 2022Cryolipolysis is characterized by localized and controlled cooling of the subcutaneous adipose tissue, in a non-invasive way, causing a localized panniculitis, followed... (Review)
Review
BACKGROUND
Cryolipolysis is characterized by localized and controlled cooling of the subcutaneous adipose tissue, in a non-invasive way, causing a localized panniculitis, followed by adipocyte death by apoptosis and, consequently, a decrease in adipose tissue in the treated area.
AIM(S)
To evaluate the scientific evidence and methodological qualities about effects, adverse reactions, and level of satisfaction of cryolipolysis for the reduction of subcutaneous adipose tissue.
METHODS
A systematic review was carried out according to the PRISMA recommendation. Searches were conducted in different databases. We included studies that used a randomized control and self-control design and were carried out in humans. Articles published in English and Portuguese were screened, with no time limit regarding the year of publication. The methodological quality of the studies was assessed using the Cochrane Rob2 scale.
RESULTS
Of 381 articles, seven were considered eligible for inclusion. After applying the Cochrane Rob2 scale, five studies were included in the final sample. Most studies showed significant results for cryolipolysis in reducing localized fat. The incorporation of a dietary program into the treatment was shown to contribute to a significant improvement in the lipid profile and liver enzymes, which does not happen when cryolipolysis is applied in isolation. Rare adverse effects have been identified, but never persisting beyond a month.
CONCLUSIONS
Cryolipolysis is an effective technique for reducing localized fat, safe, and well tolerated, with most participants satisfied at the end of the treatment. However, more randomized controlled studies should be carried out, since there is a limited number of articles with good methodological quality.
Topics: Humans; Cryotherapy; Subcutaneous Fat; Adipose Tissue; Adipocytes; Obesity; Hyperplasia; Lipectomy; Treatment Outcome
PubMed: 35869825
DOI: 10.1111/jocd.15265 -
Medicine Apr 2022Mesenteric panniculitis (MP) is a non-specific, localized inflammation at the mesentery of small intestines which often gets detected on computed tomography. An... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Mesenteric panniculitis (MP) is a non-specific, localized inflammation at the mesentery of small intestines which often gets detected on computed tomography. An association with malignant neoplasms remains unclear. We performed a systematic review and meta-analysis to examine the association of malignancy with MP.
METHODS
MEDLINE, EMBASE, Web of Science, and Cochrane databases were searched for articles published from inception to 2020 that evaluated the association of malignant neoplasms with MP in comparison with control groups. Using random-effects method, a summary odds ratio (OR) estimate with 95% confidence intervals for malignant neoplasms in MP was estimated.
RESULTS
Four case-control studies reporting data on 415 MP patients against 1132 matched-controls met inclusion criteria and were analyzed. The pooled OR for finding a malignant neoplasm in patients with MP was 0.907 (95% CI: 0.688-1.196; P = .489). The heterogeneity was mild and non-significant. Also, there was no heightened risk of any specific type of malignancy with MP. Three more case-series with unmatched-control groups (MP: 282, unmatched-controls: 17,691) were included in a separate analysis where the pooled OR of finding a malignant neoplasm was 2.963 (95% CI: 1.434-6.121; P = .003). There was substantial heterogeneity in this group.
CONCLUSION
This meta-analysis of matched controlled studies proves absence of any significant association of malignant neoplasms with MP. Our study also demonstrates that the putative association of malignancy with MP is mainly driven by uncontrolled studies or case-series.
Topics: Databases, Factual; Humans; Mesentery; Neoplasms; Panniculitis, Peritoneal; Tomography, X-Ray Computed
PubMed: 35512070
DOI: 10.1097/MD.0000000000029143 -
Clinical Rheumatology Mar 2022Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) which preferentially infiltrates into subcutaneous adipose tissue is rare, however may mimic autoimmune diseases... (Review)
Review
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) which preferentially infiltrates into subcutaneous adipose tissue is rare, however may mimic autoimmune diseases from the aspect of clinical manifestations. Here, we describe a 16-year-old young man, who initially presented with eyelid erythema and swelling, accompanied by fever and muscle and bone marrow involvement. He was preliminarily considered as a patient with classical dermatomyositis (DM), but finally diagnosed as SPTCL concomitant with paraneoplastic inflammatory myositis, confirmed by in total 8 times repeated biopsies at different sites. After systematically reviewing the literatures, we summarized the main features of SPTCL mimicking DM with eyelid edema as the presenting manifestation. The cautionary tale reminds rheumatologists of considering mimickers in patients with atypical autoimmune-like manifestations. Suitable biopsy is critical for diagnosis and improving prognosis.
Topics: Adolescent; Dermatomyositis; Diagnosis, Differential; Erythema; Eyelids; Humans; Lymphoma, T-Cell; Male; Panniculitis
PubMed: 34786628
DOI: 10.1007/s10067-021-05992-1 -
Scandinavian Journal of Rheumatology Jan 2022: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease. It is associated with mutations. Its main features are recurrent episodes...
: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease. It is associated with mutations. Its main features are recurrent episodes of fever and serositis. Patients can display dermatological manifestations such as erysipelas-like erythema, generally considered as a neutrophilic dermatosis (ND). It has been suggested that FMF can be associated with other types of ND. Our aim was to perform a systematic review of the literature to assess the link between ND and FMF.: A systematic review of the literature was performed using MEDLINE from 1946 to 2018. Three independent investigators identified reports of non-erysipelas-like erythema neutrophilic dermatosis (NEND) associated with FMF, selected the criteria to establish the diagnosis of FMF and ND, and evaluated the link between the two conditions. FMF-associated NEND was supported by confirmation of both diagnoses and exclusion of other causes of ND.: Eighteen articles were selected. Nine articles reported FMF patients with the following NEND: neutrophilic panniculitis (n = 4), Sweet syndrome (n = 6), and pyoderma gangrenosum (n = 1). None of these cases was supported by histological confirmation, fulfilled diagnostic criteria for definitive or probable FMF, or confirmed the exclusion of all the most frequent diseases associated with NEND. As a result, there is insufficient evidence to support a potential relationship between NEND and FMF.: The association between FMF and NEND remains unclear. In FMF patients with NEND, every differential diagnosis and alternative cause of NEND should be excluded before drawing any conclusions about a potential causal relationship.
Topics: Diagnosis, Differential; Familial Mediterranean Fever; Humans; Mutation; Pyrin; Skin Diseases
PubMed: 34159892
DOI: 10.1080/03009742.2021.1904588 -
Hematology/oncology and Stem Cell... Jan 2023The aim of this systematic review is to investigate different diagnostic methods and the available treatment options for subcutaneous panniculitis-like T-cell lymphoma...
OBJECTIVES
The aim of this systematic review is to investigate different diagnostic methods and the available treatment options for subcutaneous panniculitis-like T-cell lymphoma (SPTCL).
METHODS
We searched PubMed, Web of Science, SCOPUS, EBSCO, and CINAHL Plus for published case reports of SPTCL. From each record, we extracted data of the diagnostic methods, immunohistochemical profile, clinical characteristics, and the treatment approaches provided. Data were summarized and narratively synthesized to highlight the various diagnostic methods and treatment options of SPTCL.
RESULTS
Our literature search yielded 1293 unique citations. Following screening, nine articles reporting a total of 15 cases were included in this systematic review. All patients presented with subcutaneous nodules. Three of the 15 cases were initially misdiagnosed. The atypical lymphoid cells were positive for CD2, CD3, granzyme B, and TIA-1 and negative for CD1a, EBER, and CD20 in all the reported cases. The atypical lymphoid cells were positive for CD45RO in four out of seven cases, positive for CD56 in three out of 12 cases tested, while positive for CD5 and CD8 in the majority of cases. Therapy ranged from topical agents to immunosuppressive agents all the way to multiagent chemotherapy.
CONCLUSION
SPTCL is a rare lymphoma. Diagnosis is highly dependent on the immunohistochemical stains added to histopathologic and radiologic findings. Therapy is dependent on the pace of the disease, with encouraging results obtained with single-agent cyclosporine.
Topics: Humans; Lymphoma, T-Cell, Cutaneous; Lymphoma, T-Cell; Panniculitis; Skin Neoplasms
PubMed: 34015273
DOI: 10.1016/j.hemonc.2021.04.001