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Clinical Lymphoma, Myeloma & Leukemia Sep 2023Mature T- and NK-cell neoplasms (MTNKN) collectively represent a rare disorder, representing less than 15% of all non-Hodgkin lymphoma (NHL) cases and qualifying for... (Review)
Review
Mature T- and NK-cell neoplasms (MTNKN) collectively represent a rare disorder, representing less than 15% of all non-Hodgkin lymphoma (NHL) cases and qualifying for orphan disease designation by the U.S. Food and Drug Administration (FDA). These consist of 9 families in the fifth revised WHO classification of lymphoid neoplasms, which are made up of over 30 disease subtypes, underscoring the heterogeneity of clinical features, molecular biology, and genetics across this disease group. Moreover, the 5 most common subtypes (peripheral T-cell lymphoma, not otherwise specified; nodal TFH cell lymphoma, angioimmunoblastic type; extranodal NK-cell/T-cell lymphoma; adult T-cell leukemia/lymphoma; and ALK-positive or -negative anaplastic large cell lymphoma) comprise over 75% of MTNKN cases, so other subtypes are exceedingly rare in the context of all NHL diagnoses and consequently often lack consensus on best practices in diagnosis and management. In this review, we discuss the following entities-enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and primary cutaneous ɣδ T-cell lymphoma (PCGD-TCL) - with an emphasis on clinical and diagnostic features and options for management.
Topics: Adult; Humans; Lymphoma, T-Cell, Peripheral; Lymphoma, Extranodal NK-T-Cell; Lymphoma, Large-Cell, Anaplastic; Panniculitis
PubMed: 37302955
DOI: 10.1016/j.clml.2023.05.012 -
Cureus May 2024Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome presents a unique challenge in diagnosis and management because of its rarity and heterogeneous initial...
Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome presents a unique challenge in diagnosis and management because of its rarity and heterogeneous initial presentation. This manuscript presents a case series of two patients with PPP syndrome, shedding light on the diagnostic process and care for this uncommon condition. PPP syndrome is characterized by the simultaneous occurrence of pancreatitis or pseudocysts alongside polyarthritis and panniculitis. While its exact pathophysiology remains obscure, pancreatic inflammation is assumed to trigger the hematogenous dissemination of pancreatic enzymes, leading to fat necrosis and subsequent panniculitis, as well as chondronecrosis and/or osteonecrosis causing polyarthritis. Despite its recognition in medical literature since the late 1980s, PPP syndrome remains poorly understood, with only a limited number of cases reported globally. Its rarity and varied initial manifestations often result in misdiagnosis, causing delays in appropriate treatment. The presented case series highlights key clinical features and diagnostic clues of PPP syndrome. Both patients exhibited initial symptoms of inflammatory polyarthritis, accompanied by characteristic findings of "ghost cells" on skin biopsy. Additionally, radiographic and laboratory evidence revealed pancreatic changes consistent with this syndrome. This case series underscores the importance of multidisciplinary collaboration in managing PPP syndrome. Early recognition and accurate diagnosis are pivotal in initiating prompt and effective therapeutic interventions, thereby improving patient outcomes and minimizing long-term sequelae.
PubMed: 38826929
DOI: 10.7759/cureus.59471 -
BMJ Case Reports Sep 2023A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a...
A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a history of alcohol dependence with multiple episodes of acute pancreatitis. With polyarthritis progressing relentlessly, unresponsive to non-steroidal anti-inflammatory drugs and steroids, a provisional diagnosis of sarcoidosis was considered. Indeed, he was treated with azathioprine and rituximab with no effect. Biopsy of the skin nodule revealed subcutaneous fat necrosis, foam cells, deposition of eosinophilic amorphous material and calcification. Synovial fluid aspiration from the arthritic knee obtained purulent but surprisingly culture-negative material, rich in triglycerides. Abdominal CT confirmed chronic pancreatitis. Final diagnosis of pancreatitis, panniculitis and polyarthritis (PPP) syndrome was made. He underwent surgical pancreatic ductal drainage leading to complete remission of symptoms. PPP syndrome triad occurs due to leakage of pancreatic enzymes into systemic circulation and subsequent fat necrosis. Surgical drainage of pancreatic duct is often curative.
Topics: Humans; Male; Pancreatitis; Acute Disease; Panniculitis; Arthritis; Subcutaneous Fat; Fat Necrosis
PubMed: 37751979
DOI: 10.1136/bcr-2023-254732 -
Clinical Journal of Gastroenterology May 2024Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin...
Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin lesions (panniculitis), and pancreatic inflammation (pancreatitis), known as PPP. The pathogenesis of this phenomenon is not fully understood but is believed to involve lipolysis by pancreatic enzymes at lipid-rich skin and joint sites. PPP primarily affects middle-aged males with a history of alcohol use disorder. Diagnosis can be challenging due to the absence of typical abdominal symptoms. Delayed diagnosis may significantly worsen outcomes. Supportive therapy is the mainstay, but resolution requires addressing the underlying pancreatic abnormality. We present a case of a patient with a history of alcohol use disorder and recurrent acute pancreatitis who developed joint pain and skin rash. Extensive work-up ruled out other causes, and imaging and biopsy confirmed the diagnosis of PPP. Symptomatic management and treatment of the underlying pancreatic abnormality led to complete resolution of symptoms. Our case serves to raise awareness of this rare but potentially fatal syndrome.
PubMed: 38775908
DOI: 10.1007/s12328-024-01977-0 -
Journal of Investigative Medicine High... 2024Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is... (Review)
Review
Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.
Topics: Humans; Male; Biopsy; Diagnosis, Differential; Fatal Outcome; Lymphohistiocytosis, Hemophagocytic; Lymphoma, T-Cell; Lymphoma, T-Cell, Cutaneous; Panniculitis; Skin; Skin Neoplasms; Young Adult
PubMed: 38742532
DOI: 10.1177/23247096241253337 -
European Journal of Radiology Oct 2023To analyze the computed tomography (CT) findings of idiopathic mesenteric panniculitis and the factors related to its characteristics and to improve the understanding of...
OBJECTIVES
To analyze the computed tomography (CT) findings of idiopathic mesenteric panniculitis and the factors related to its characteristics and to improve the understanding of the disease.
METHODS
The imaging findings of 121 patients with mesenteric panniculitis were retrospectively analyzed, along with related factors such as age, sex, and abdominal visceral fat area.
RESULTS
Among the 121 patients, two had midgut malrotation, and the lesions were located outside the mesentery on the right side of the abdominal cavity, while the lesions in the other patients were located around the mesentery on the left side of the abdominal cavity, presenting as patchy or patchy fuzzy high-density masses. Annulus fibrosus and/or fatty ring signs were also observed in some patients. Scattered soft tissue nodules were observed around the mesentery in 119 patients. Eight patients had intestinal tract traction and retraction, and one patient had secondary intestinal obstruction. Nearly half of the patients had mesenteric vascular changes, and three had mesenteric vascular thrombosis. Among the 121 patients, there were 89 males and 32 females, aged 22-83, with an average age of 52.14 ± 13.53 years. The distribution range of abdominal visceral fat area (VFA) in 121 patients was 79.85-331.65 cm.
CONCLUSION
Mesenteric panniculitis has certain characteristic imaging findings that can be accompanied by often ignored changes in the mesenteric blood vessels and intestinal tubes. Intestinal obstruction and mesenteric vascular thrombosis are rare complications, and their primary causes are often overlooked. Mesenteric panniculitis was correlated with sex, age, and VFA.
Topics: Female; Male; Humans; Adult; Middle Aged; Aged; Panniculitis, Peritoneal; Retrospective Studies; Intestinal Obstruction; Tomography, X-Ray Computed; Thrombosis
PubMed: 37666075
DOI: 10.1016/j.ejrad.2023.111071 -
Clinical Immunology (Orlando, Fla.) Oct 2023Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous non-Hodgkin lymphoma involving CD8 T cells, the genetic underpinnings of which remain...
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous non-Hodgkin lymphoma involving CD8 T cells, the genetic underpinnings of which remain incompletely understood. Here we report two unrelated patients with B cell Expansion with NF-κB and T cell Anergy (BENTA) disease and a novel presentation of SPTCL. Patient 1 presented early in life with recurrent infections and B cell lymphocytosis, linked to a novel gain-of-function (GOF) CARD11 mutation (p.Lys238del). He developed SPTCL-like lesions and membranoproliferative glomerulonephritis by age 2, treated successfully with cyclosporine. Patient 2 presented at 13 months with splenomegaly, lymphadenopathy, and SPTCL with evidence of hemophagocytic lymphohistiocytosis. Genetic analysis revealed two in cis germline GOF CARD11 variants (p.Glu121Asp/p.Gly126Ser). Autologous bone marrow transplant resulted in SPTCL remission despite persistent B cell lymphocytosis. These cases illuminate an unusual pathological manifestation for BENTA disease, suggesting that CARD11 GOF mutations can manifest in cutaneous CD4and CD8 T cell malignancies.
Topics: Male; Humans; Child, Preschool; CD8-Positive T-Lymphocytes; Lymphocytosis; Panniculitis; Lymphoma, T-Cell; Immunologic Deficiency Syndromes
PubMed: 37562721
DOI: 10.1016/j.clim.2023.109732 -
BMJ Case Reports Oct 2023Pancreatic panniculitis is a rare cutaneous manifestation of pancreatic disease with only scant case reports available to guide management. In this report, a woman in...
Pancreatic panniculitis is a rare cutaneous manifestation of pancreatic disease with only scant case reports available to guide management. In this report, a woman in her 60s developed a painful, erythematous and indurated, nodular rash after an episode of acute pancreatitis postendoscopic retrograde cholangiopancreatography (ERCP). While clinically and radiologically the pancreatitis improved with standard conservative management, the panniculitis remained severely debilitating. Repeat testing of serum pancreatic enzymes revealed persistent and marked elevation. Octreotide was started to inhibit pancreatic enzyme release, and the lesions improved, with resolution of the panniculitis correlating with falling serum pancreatic enzyme levels. Hence, serial pancreatic enzyme testing may have utility in monitoring and management of pancreatic panniculitis.
Topics: Female; Humans; Acute Disease; Lipase; Pancreas; Pancreatitis; Panniculitis; Aged
PubMed: 37899080
DOI: 10.1136/bcr-2022-254370 -
The Journal of Dermatology Feb 2024There are various types of cutaneous lupus erythematosus (CLE), either with or without the association of systemic lupus erythematosus (SLE). In some of the subtypes of... (Review)
Review
There are various types of cutaneous lupus erythematosus (CLE), either with or without the association of systemic lupus erythematosus (SLE). In some of the subtypes of cutaneous lupus, histopathology reveals neutrophil infiltration in the lesional skin; however, the significance of neutrophils in CLE is not yet fully elucidated. Recent studies have shown that neutrophil extracellular traps (NETs) formation by activated neutrophils is observed in several types of CLE, including lupus panniculitis, subacute lupus erythematosus, and acute lupus erythematosus, although the number of reports is small. Excessive NETosis, due to either increased NETs formation or defective clearance of NETs, may play a role in the induction of autoimmunity and autoantibody production in SLE, as well as endothelial damage, thrombus formation, and vascular damage in the lesional skin. CLE is an excessive interferon-driven autoimmune disease. Plasmacytoid dendritic cells are located in lupus erythematosus skin and contribute to the etiology of skin lesions as a main producing cell of type I interferon. Neutrophils, monocytes, and keratinocytes also produce type I interferon via several triggers. Neutrophils play an important role in the innate immune response in SLE. In this review, several types of CLE with neutrophil infiltration, as well as the role of neutrophils are discussed.
Topics: Humans; Neutrophils; Lupus Erythematosus, Cutaneous; Skin; Lupus Erythematosus, Systemic; Interferon Type I
PubMed: 38009863
DOI: 10.1111/1346-8138.17036 -
International Journal of Dermatology Jul 2023VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations.
BACKGROUND
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations.
METHODS
We conducted a retrospective study of all patients with genetically confirmed VEXAS syndrome seen at our institution. Available clinical photographs and skin biopsy slides were reviewed.
RESULTS
Cutaneous manifestations developed in 22/25 (88%) patients with VEXAS syndrome. From this group, 10/22 (45%) developed skin involvement before or at the time of other clinical features of VEXAS. Twenty distinct dermatologic presentations of VEXAS from 14 patients were reviewed, and histopathologic patterns were classified as follows: neutrophilic urticarial dermatosis (n = 5, 25%), leukocytoclastic/urticarial vasculitis (n = 4, 20%), urticarial tissue reaction (n = 4, 20%), neutrophilic dermatosis (n = 3, 15%), neutrophilic panniculitis (n = 2, 10%), and nonspecific chronic septal panniculitis (n = 2, 10%). Common systemic findings included macrocytic anemia (96%), fever (88%), thrombocytopenia (76%), weight loss (76%), ocular inflammation (64%), pulmonary infiltrates (56%), deep venous thrombosis or pulmonary embolism (52%), and inflammatory arthritis (52%).
CONCLUSIONS
Cutaneous involvement is a common feature of VEXAS syndrome, and histopathologic findings exist on a spectrum of neutrophilic inflammatory dermatoses.
Topics: Humans; Retrospective Studies; Skin; Urticaria; Dermatitis; Panniculitis; Mutation
PubMed: 36890121
DOI: 10.1111/ijd.16635