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Magyar Sebeszet Jun 2024Kompressziós vékonybél ileus esetét ismertetjük, amelyet a bélfodor nem gyakori, inflammatorikus természetű betegsége, mesenterialis panniculitis idézett elő....
BEVEZETÉS
Kompressziós vékonybél ileus esetét ismertetjük, amelyet a bélfodor nem gyakori, inflammatorikus természetű betegsége, mesenterialis panniculitis idézett elő. A magyar szakirodalomban ilyen közléssel nem találkoztunk.
ESETISMERTETÉS
A 91 éves férfi akut hasi panaszokkal került kórházba. A vizsgálatokkal vékonybél ileus derült ki. Ennek hátterében műtétkor malignitásra gyanús, bélfodri multinodularis elváltozást fedtünk föl. A biopsziából mesenterialis panniculitist diagnosztizáltunk. A ritka, több nyitott kérdéssel terhelt entitást mutatjuk be az irodalom és a saját észleleteink tükrében.
KÖVETKEZTETÉSEK
Számos differenciáldiagnosztikai eshetőség figyelembevételével a kórkép szövettanilag igazolható. A diagnózis felállítása után a további teendőket az egyéb leletek és az adott klinikai kontextus gondos elemzése fogja meghatározni.
Topics: Humans; Male; Aged, 80 and over; Intestinal Obstruction; Diagnosis, Differential; Panniculitis, Peritoneal; Intestine, Small; Ileus
PubMed: 38941150
DOI: 10.1556/1046.2024.20004 -
International Journal of Infectious... Jun 2024Erythema nodosum (EN) is a skin manifestation of panniculitis characterized by symmetric, painful, tender nodules, and most cases are self-limiting. Few cases of EN...
Erythema nodosum (EN) is a skin manifestation of panniculitis characterized by symmetric, painful, tender nodules, and most cases are self-limiting. Few cases of EN following Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccination have been reported, and they are generally self-limiting. We reported the challenging case of a 63-year-old Asian woman with EN that persisted for more than three months after a coronavirus disease-19 (COVID-19). There was no improvement despite topical steroid and NSAIDs treatment, and the patient was successfully treated with combination of high-dose steroid and NSAIDs. There were long-lasting symptoms involving various organ symptoms persisting over three months after COVID-19, which is known as Long COVID. As part of Long COVID, there are limited cases of skin manifestations. Given that immune dysregulation due to persistent coronaviruses may contribute to refractory EN, Erythema nodosum related to COVID-19 is rare, but can occur; clinicians should be aware of the occurrence of EN following COVID-19 infection.
PubMed: 38936655
DOI: 10.1016/j.ijid.2024.107152 -
BMJ Case Reports Jun 2024A white Caucasian woman in her 30s presented with an indurated lesion on her right upper arm. Panniculitis was clinically suspected. Antinuclear antibody testing was...
A white Caucasian woman in her 30s presented with an indurated lesion on her right upper arm. Panniculitis was clinically suspected. Antinuclear antibody testing was positive but incisional biopsy showed subcutaneous panniculitis-like T-cell lymphoma (SPTCL), although with some unusual features more in keeping with lupus. Initial treatment was with oral prednisolone and radiotherapy but with only partial response. A second biopsy was taken from an area of presumed residual disease. This displayed histological features that were much more typical of lupus erythematosus profundus (LEP) but with tiny foci suggesting concomitant microscopic areas of SPTCL. Immunofluorescence for IgM was positive. This case highlights the rare occurrence of a patient with overlapping clinical and pathological features of SCPTL and LEP. It emphasises the need for close clinicopathological correlation in the workup of patients with suspected panniculitis and the importance of careful pathological examination for features of both diseases.
Topics: Humans; Female; Panniculitis; Panniculitis, Lupus Erythematosus; Lymphoma, T-Cell; Adult; Diagnosis, Differential; Biopsy; Prednisolone
PubMed: 38925673
DOI: 10.1136/bcr-2023-255592 -
Clinical Immunology (Orlando, Fla.) Jun 2024OTULIN encodes an eponymous linear deubiquitinase (DUB) essential for controlling inflammation as a negative regulator of the canonical NF-κB signaling pathway via the...
OTULIN encodes an eponymous linear deubiquitinase (DUB) essential for controlling inflammation as a negative regulator of the canonical NF-κB signaling pathway via the regulation of M1-Ub dynamics. Biallelic loss-of-function (LOF) mutations in OTULIN cause an autosomal recessive condition named Otulin-Related Autoinflammatory Syndrome (ORAS), also known as Otulipenia or AutoInflammation, Panniculitis, and Dermatosis Syndrome (AIPDS). Monoallelic OTULIN LOF, also known as OTULIN Haploinsufficiency (OHI) or Immunodeficiency 107 (IMD107), has been linked to an incompletely penetrant, dominantly inherited susceptibility to invasive Staphylococcal infections. At the same time, a recent novel ORAS-like inflammatory syndrome was described in association with a heterozygous missense mutation that appears to exert dominant negative (DN) effects. In this manuscript, we report the identification of a novel homozygous missense mutation, c.595 T > A; p.(Trp199Arg), in a Moroccan infant with an ORAS phenotype and provide experimental evidence for its pathogenicity. We go on to systematically review the literature for OTULIN-associated conditions by using the GenIA database (www.geniadb.net) to collect, extract and harmonize all clinical, laboratory and functional data for published patients and variants. Our comprehensive synthesis of genotypic, phenotypic, and mechanistic data enables a more in-depth view of the diverse mechanisms and pathways by which the OTULIN pathogenic variants may lead to human immune disease. This review may help variant classification activities and inform future variant evaluation, as well as the development of diagnostic and management guidelines. It also identifies current knowledge gaps and raises additional questions warranting future investigation.
PubMed: 38914362
DOI: 10.1016/j.clim.2024.110292 -
Plastic and Reconstructive Surgery.... Jun 2024Endometriosis is a common gynecological disorder described as the presence of functional endometrial tissue outside the uterus, which can also be found in extrapelvic...
BACKGROUND
Endometriosis is a common gynecological disorder described as the presence of functional endometrial tissue outside the uterus, which can also be found in extrapelvic locations. Although patients seeking treatment for endometriosis usually present to gynecologists, there are rare cases of endometriosis encountered by plastic surgeons in routine practice, either incidentally or as a concomitant finding.
METHODS
We present a rare case of a 36-year-old woman with symptoms of panniculitis desiring panniculectomy. During surgery, an abdominal mass was excised and confirmed by pathological analysis to be endometriosis. A comprehensive literature review was conducted using the PubMed search engine of the National Institutes of Health to identify cases of endometriosis in plastic surgery. Following screening of the results, 14 articles were included in this analysis that fit the criteria of our search.
RESULTS
Of the 14 articles reviewed, cutaneous endometriosis was the most common subtype found in plastic surgery. None of the studies described findings of endometriosis in routine panniculectomies. Several identified endometriosis discovered during cosmetic abdominoplasties.
CONCLUSIONS
Endometriosis encountered in plastic surgery is a rare but clinically important occurrence, with the cutaneous subtype representing the majority of cases. Endometriosis should always be on the differential diagnosis when an abdominal mass is found in a patient with a history of abdominal surgery. Abdominal masses found during routine aesthetic or reconstructive surgery should be submitted for tissue analysis to guide possible secondary treatments.
PubMed: 38911574
DOI: 10.1097/GOX.0000000000005904 -
Pediatric Dermatology Jun 2024Acquired generalized lipodystrophy (AGL) is a rare disease characterized by variable loss of adipose tissue and concurrent metabolic derangements, typically with...
Acquired generalized lipodystrophy (AGL) is a rare disease characterized by variable loss of adipose tissue and concurrent metabolic derangements, typically with childhood or adolescent onset. AGL has three subclassifications: panniculitis (type 1), autoimmune disease (type 2), and idiopathic (type 3). This report highlights a rare case of AGL type 1 in a previously healthy 3-year-old female who presented with diffuse erythematous subcutaneous nodules, progressive lipoatrophy, and histopathological findings of a lobular panniculitis.
PubMed: 38887123
DOI: 10.1111/pde.15668 -
Zeitschrift Fur Rheumatologie Jun 2024Erythema nodosum (EN) is the most frequently occurring form of acute panniculitis. It is characterized by painful red to livid raised nodules or bumps that typically...
Erythema nodosum (EN) is the most frequently occurring form of acute panniculitis. It is characterized by painful red to livid raised nodules or bumps that typically occur symmetrically in the shin area. The cause of EN is often a reaction of the immune system to various triggers including infections, inflammatory diseases or medications. In approximately half of the cases no trigger can be identified. After treatment of the underlying pathology EN is typically self-limiting.
PubMed: 38884811
DOI: 10.1007/s00393-024-01529-4 -
Pediatric Dermatology Jun 2024Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) or proteasome-associated autoinflammatory syndrome is a rare...
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) or proteasome-associated autoinflammatory syndrome is a rare autoinflammatory disorder that typically presents in infancy with characteristic symptoms, including recurrent fever, panniculitis, and progressive lipodystrophy, among other findings. We present a case of mother and child with CANDLE syndrome. The child was eventually started on baricitinib with normalization of rash and systemic findings.
PubMed: 38881047
DOI: 10.1111/pde.15667 -
Haematologica Jun 2024Not available.
Not available.
PubMed: 38867583
DOI: 10.3324/haematol.2023.284738 -
The Journal of Dermatological Treatment Dec 2024The incidence of cutaneous paradoxical reactions associated with IL-17 inhibitors has gained attention in recent literature. Our report aims to investigate the... (Review)
Review
The incidence of cutaneous paradoxical reactions associated with IL-17 inhibitors has gained attention in recent literature. Our report aims to investigate the characteristics of one rare paradoxical reaction, presenting as Behcet's disease. We reported one case of Behcet's-like disease induced by secukinumab in a patient with psoriasis. This patient, a young woman with a long history of psoriasis, showed significant improvement in her psoriatic condition after receiving four doses of secukinumab. Unexpectedly, she developed symptoms such as high fever, painful oral and genital ulcers, facial maculopapules, and erythema nodosum-like lesions on her lower limbs. Despite neutrophilia, there was no evidence of infection found in her laboratory tests. Histological analysis of a skin biopsy highlighted subcutaneous panniculitis and a mixed inflammatory cell infiltrate in the dermis. The patient was consequently diagnosed with secukinumab-induced Behcet's-like disease. Additionally, we have reviewed nine other documented cases of Behcet's-like disease triggered by IL-17 inhibitors. This group showed no significant gender preference, suffering from conditions such as psoriasis, ankylosing spondylitis, and hidradenitis suppurativa. Oral and genital ulcers were prevalent among the paradoxical reactions noted. Marked improvement was observed in all patients upon discontinuation of the IL-17 inhibitors. Our report serves to alert physicians to this uncommon but significant paradoxical effect that may arise with anti-IL-17 treatment.
Topics: Humans; Female; Antibodies, Monoclonal, Humanized; Behcet Syndrome; Psoriasis; Adult; Interleukin-17; Skin
PubMed: 38857894
DOI: 10.1080/09546634.2024.2347440