Subcutaneous panniculitis-like T-cell lymphoma in two unrelated individuals with BENTA disease.

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 CD4+and CD8+ T cell malignancies.

Diagnosis and Treatment of Subcutaneous Panniculitis-like T-cell Lymphoma: A Systematic Literature Review.

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.

Clinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.

Importance: There is a knowledge gap about subcutaneous panniculitis-like T-cell lymphoma (SPTCL) owing to its rarity and diagnostic difficulty, resulting in an absence of well-documented large case series published to date. Objective: To generate consensus knowledge by a joint multi-institutional review of SPTCL and related conditions. Design, Setting, and Participants: This retrospective clinical and pathological review included cases initially diagnosed as SPTCL at 6 large US academic centers. All cases were reviewed by a group of pathologists, dermatologists, and oncologists with expertise in cutaneous lymphomas. Through a process of group consensus applying defined clinical and pathological diagnostic criteria, the cohort was classified as (1) SPTCL or (2) adipotropic lymphoproliferative disorder (ALPD) for similar cases with incomplete histopathological criteria for SPTCL designation. Exposures: Cases of SPTCL diagnosed between 1998 and 2018. Main Outcomes and Measures: The main outcome was disease presentation and evolution, including response to therapy, disease progression, and development of hemophagocytic lymphohistiocytosis. Results: The cohort of 95 patients (median [range] age, 38 [2-81] years; female-to-male ratio, 2.7) included 75 cases of SPTCL and 20 cases of ALPD. The clinical presentation was similar for both groups with multiple (61 of 72 [85%]) or single (11 of 72 [15%]) tender nodules mostly involving extremities, occasionally resulting in lipoatrophy. Hemophagocytic lymphohistiocytosis (HLH) was only observed in SPTCL cases. With a mean follow-up of 56 months, 60 of 90 patients (67%) achieved complete remission with a median (range) of 3 (1-7) cumulative therapies. Relapse was common. None of the patients died of disease progression or HLH. Two patients with ALPD eventually progressed to SPTCL without associated systemic symptoms or HLH. Conclusions and Relevance: In this case series of patients initially diagnosed as having SPTCL, results showed no evidence of systemic tumoral progression beyond the adipose tissue. The SPTCL experience in this study confirmed an indolent course and favorable response to a variety of treatments ranging from immune modulation to chemotherapy followed by hematopoietic stem cell transplantation. Morbidity was primarily associated with HLH.

Subcutaneous panniculitis-like T-cell lymphoma in a 14-year-old female homozygous for HAVCR2 mutation.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma preferentially involving subcutis. A link between patients with SPTCL and HAVCR2 mutations has recently been discovered. We present a 14-year-old girl of Chinese heritage who was diagnosed with SPTCL in the context of homozygous HAVCR2 status for c.245A>G p. (Tyr82Cys) and achieved complete remission after treatment with cyclosporin and steroids. Dermatologists should be aware of the diagnostic, management and familial genetic counselling utility of HAVCR2 for investigating and managing patients with SPTCL.

A Rare Case of Subcutaneous Panniculitis-Like T Cell Lymphoma with Hemophagocytic Lymphohistiocytosis Mimicking Cellulitis.

BACKGROUND Subcutaneous panniculitis-like T cell lymphoma and primary cutaneous γdelta T cell lymphoma are rare forms of non-Hodgkin lymphoma presenting as skin nodules or plaques. CASE REPORT Here, we present a case of a 48-year-old man with multiple subcutaneous, tender, erythematous nodules on his right thigh and left arm. Multiple courses of antibiotics were administered with no significant improvement in the patient's lesions. The skin biopsy report showed CD3/CD8 lymphocytic rimming of the adipocytes and the patient was diagnosed with subcutaneous panniculitis-like T cell lymphoma. A subsequent bone marrow biopsy showed hemophagocytic lymphohistiocytosis. The patient underwent treatment with the cyclophosphamide, hydroxydaunorubicin, Oncovin, prednisone, and etoposide chemotherapy regimen and received an autologous peripheral blood stem cell transplant. CONCLUSIONS Nodular skin lesions can result from a variety of noninfectious causes in addition to bacterial and fungal infections. This case highlights the importance of early biopsy of skin lesions that do not respond to standard therapy to establish an accurate diagnosis and start timely treatment to prevent poor outcomes.

Viral panniculitis in a patient with disseminated opportunistic Enterovirus infection.

Infection-induced panniculitis has been described in association with a broad range of microorganisms. Among those, viral panniculitis represents a minor category, with only a few anecdotal reports in the literature documenting viral infection in the subcutaneous fat. Herein, we report a woman in her 30s with seropositive rheumatoid arthritis on rituximab and prednisone, who presented with a 6-month history of progressive multisystem manifestations, including unintentional weight loss, fever, fatigue, myopathy, pancreatitis, and sensorineural hearing loss. She had indurated plaques on her thighs characterized by predominantly lobular panniculitis with chronic lymphohistiocytic inflammation. Molecular studies performed at the Centers for Disease Control and Prevention identified evidence of Enterovirus group with the highest identity of Coxsackievirus A9. Enterovirus RNA was also detected in the cerebrospinal fluid and muscle. Based on the findings, a diagnosis of disseminated enteroviral infection in the setting of B-cell depletion was rendered. To the best of our knowledge, this represents the first reported case of viral panniculitis with documentation of Coxsackievirus A9 in the skin. Since rituximab may be used for the treatment of autoimmune dermatological diseases, familiarity of the potential occurrence of severe enteroviral infections in the setting of immunosuppressive treatment is important for dermatopathologists.

Panniculitis caused by progesterone injection can be treated by physical therapy.

A method for the treatment of panniculitis caused by progesterone injection is introduced. Sixteen patients achieved good results. This is a 9-year single center retrospective study. Of all the 5633 patients who received progesterone injection, 16 developed panniculitis at the injection site. Pathological examination confirmed the occurrence of panniculitis. The patient received physical therapy. These treatments are determined by the course of the patient. Compared with patients without panniculitis, patients with panniculitis received more than one injection of progesterone. In 16 patients, symptoms and local signs disappeared completely in 15 patients. One patient did not take physical therapy according to the doctor's advice after the treatment improved. However, 1 month later, the patient went to see the doctor again and received the relevant physical therapy, and still achieved good results. Progesterone injection may lead to panniculitis, which is rare but may cause serious consequences. Physical therapy can be effective.

Matcha Improves Metabolic Imbalance-Induced Cognitive Dysfunction.

This study was conducted to assess the protective effect of extract of match (EM) on high-fat diet- (HFD-) induced cognitive deficits in male C57BL/6 mice. It was found that EM improved glucose tolerance status by measuring OGTT and IPGTT with HFD-induced mice. EM protected behavioral and memory dysfunction in Y-maze, passive avoidance, and Morris water maze tests. Consumption of EM reduced fat mass, dyslipidemia, and inflammation in adipose tissue. Also, EM ameliorated hepatic and cerebral antioxidant systems. EM improved the cerebral cholinergic system by regulating ACh contents and expression of AChE and ChAT. Also, EM restored mitochondrial function in liver and brain tissue. EM attenuated hepatic inflammatory effect, lipid synthesis, and cholesterol metabolism by regulating the protein expression of TNF-α, TNFR1, p-IRS-1, p-JNK, IL-1ß, iNOS, COX-2, HMGCR, PPARγ, and FAS. Finally, EM regulated cognitive function and neuroinflammation in the whole brain, hippocampus, and cerebral cortex by regulating the protein expression of p-JNK, p-Akt, p-tau, Aß, BDNF, IDE, COX-2, and IL-1ß. These findings suggest that EM might be a potential source of functional food to improve metabolic disorder-associated cognitive dysfunction.

[Panniculitis in association with target therapy in melanoma patient, what the dermatologist should know: A case report]. / Paniculitis secundaria a terapia target en paciente con melanoma, lo que el dermatólogo debe saber: reporte de un caso.

Target therapies are currently a therapeutic option increasingly used for the management of patients with metastatic melanoma. However, there are multiple adverse pharmacological effects associated with their use that have been described. Cutaneous adverse reactions are the most frequent. We report the case of a 55-year-old man with a diagnosis of stage IV BRAFV600E-mutated metastatic cutaneous melanoma undergoing treatment with dabrafenib/trametinib, who consulted due to the development of erythematous nodular lesions in the upper and lower limbs associated with febrile sensation during the course of treatment. Infection was ruled out and a biopsy of the skin lesions was done, which provided the histopathological confirmation of a predominantly septal, granulomatous with leukocytoclastic vasculitis, mixed panniculitis. Panniculitis associated with this therapy has been described in the literature and has been considered an immune-mediated pharmacological adverse effect. It is considered to be related to a better prognosis in the treatment of metastatic melanoma. Consequently, as shown in this case report, target therapy should not be discontinued and symptomatic medication should be given to alleviate patient discomfort. The dermatologist should know and properly interpret this adverse effect and prescribe the most appropriate management for the patient.

Las terapias target constituyen hoy en día una alternativa terapéutica cada vez más utilizada para el manejo de pacientes con melanoma metastásico. Sin embargo, se han descrito múltiples efectos farmacológicos adversos asociados a su uso, siendo los cutáneos los de mayor prevalencia. Se presenta el caso de un hombre de 55 años con diagnóstico de melanoma cutáneo metastásico etapa IV, BRAFV600E mutado, en tratamiento con dabrafenib/trametinib que consultó por desarrollo de lesiones nodulares eritematosas sensibles en extremidades superiores e inferiores, asociadas a sensación febril durante el curso del tratamiento. Se descartó alguna infección sobreagregada. Se realizó una biopsia de las lesiones cutáneas, con confirmación diagnóstica histopatológica de una paniculitis mixta de predominio septal, granulomatosa y con vasculitis leucocitoclástica. La paniculitis asociada a esta terapia ha sido descrita en la literatura y se ha considerado un efecto farmacológico inmunomediado adverso, relacionándose a un mejor pronóstico para el melanoma metastásico en tratamiento. Por lo tanto, así como en el caso presentado, se evita la suspensión del fármaco y se asocia terapia sintomática en caso de mayores molestias del paciente. Es de alta relevancia para el dermatólogo conocer e interpretar adecuadamente este efecto adverso farmacológico, y así indicar el manejo más adecuado para el paciente.

B Lymphocytes and Adipose Tissue Inflammation.

The immune system plays an important role in obesity-induced adipose tissue inflammation and the resultant metabolic dysfunction, which can lead to hypertension, dyslipidemia, and insulin resistance and their downstream sequelae of type 2 diabetes mellitus and cardiovascular disease. While macrophages are the most abundant immune cell type in adipose tissue, other immune cells are also present, such as B cells, which play important roles in regulating adipose tissue inflammation. This brief review will overview B-cell subsets, describe their localization in various adipose depots and summarize our knowledge about the function of these B-cell subsets in regulating adipose tissue inflammation, obesity-induced metabolic dysfunction and atherosclerosis.

Paniculitis secundaria a terapia target en paciente con melanoma, lo que el dermatólogo debe saber: reporte de un caso / Panniculitis in association with target therapy in melanoma patient, what the dermatologist should know: a case report

Las terapias target constituyen hoy en día una alternativa terapéutica cada vez más utilizada para el manejo de pacientes con melanoma metastásico. Sin embargo, se han descrito múltiples efectos farmacológicos adversos asociados a su uso, siendo los cutáneos los de mayor prevalencia. Se presenta el caso de un hombre de 55 años con diagnóstico de melanoma cutáneo metastásico etapa IV, BRAFV600E mutado, en tratamiento con dabrafenib/trametinib que consultó por desarrollo de lesiones nodulares eritematosas sensibles en extremidades superiores e inferiores, asociadas a sensación febril durante el curso del tratamiento. Se descartó alguna infección sobreagregada. Se realizó una biopsia de las lesiones cutáneas, con confirmación diagnóstica histopatológica de una paniculitis mixta de predominio septal, granulomatosa y con vasculitis leucocitoclástica. La paniculitis asociada a esta terapia ha sido descrita en la literatura y se ha considerado un efecto farmacológico inmunomediado adverso, relacionándose a un mejor pronóstico para el melanoma metastásico en tratamiento. Por lo tanto, así como en el caso presentado, se evita la suspensión del fármaco y se asocia terapia sintomática en caso de mayores molestias del paciente. Es de alta relevancia para el dermatólogo conocer e interpretar adecuadamente este efecto adverso farmacológico, y así indicar el manejo más adecuado para el paciente.

Target therapies are currently a therapeutic option increasingly used for the management of patients with metastatic melanoma. However, there are multiple adverse pharmacological effects associated with their use that have been described. Cutaneous adverse reactions are the most frequent. We report the case of a 55-year-old man with a diagnosis of stage IV BRAFV600E-mutated metastatic cutaneous melanoma undergoing treatment with dabrafenib/trametinib, who consulted due to the development of erythematous nodular lesions in the upper and lower limbs associated with febrile sensation during the course of treatment. Infection was ruled out and a biopsy of the skin lesions was done, which provided the histopathological confirmation of a predominantly septal, granulomatous with leukocytoclastic vasculitis, mixed panniculitis. Panniculitis associated with this therapy has been described in the literature and has been considered an immune-mediated pharmacological adverse effect. It is considered to be related to a better prognosis in the treatment of metastatic melanoma. Consequently, as shown in this case report, target therapy should not be discontinued and symptomatic medication should be given to alleviate patient discomfort. The dermatologist should know and properly interpret this adverse effect and prescribe the most appropriate management for the patient.

Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare form of cytotoxic T-cell lymphoma. It is believed that SPTL in patients without hemophagocytic syndrome (HPS) follows an indolent course; in contrast, SPTL in patients with HPS has been associated with unfavorable survival. To provide more clinical data on SPTL in Asian populations and to identify optimal therapeutic strategies for SPTL, we assessed the clinicopathological features and long-term follow-up data of 10 Taiwanese SPTL patients diagnosed at a single center. Our study demonstrates a group of patients with high incidence of HPS (50%), rather aggressive courses, and early progression. A total of eight patients underwent hematopoietic stem cell transplant (HSCT), including one autologous HSCT and seven allogeneic HSCT. Seven of eight patients receiving HSCT achieved durable remission and maintained in remission for over 30 months (range 30-132 months). There was no difference in 3-year survival of patients with HPS (80%) compared with patients without HPS (80%). Of long-term survivors in the HPS group, three of four received HSCT (autologous HSCT, n = 1; allogeneic HSCT, n = 2). Our study indicated that HSCT is a curative option for eligible SPTL patients with HPS.

Necrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma.

A 51-year-old white woman with a past medical history significant for steroid-dependent ulcerative colitis, rheumatoid arthritis, and diabetes mellitus presented to the hospital with fever and painful, erythematous subcutaneous nodules on the medical aspects of both thighs. Histopathologic examination showed features suggestive of an abscess, but her condition failed to improve with intravenous broad-spectrum antibiotics. Molecular studies detected T cell receptor-ß gene rearrangements. The lesions later exhibited signs of necrosis, requiring multiple debridements as well as therapy with hyperbaric oxygen. She was later referred to the MD Anderson Cancer Center for evaluation for possible subcutaneous panniculitis-like T cell lymphoma.

Paniculitis por micobacterias atípicas tras mesoterapia / Panniculitis Due to Atypical Mycobacteria After Mesothera

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[Idiopathic lipoatrophic panniculitis in an adult treated with hydroxychloroquine and adipocyte grafting]. / Panniculite lipoatrophiante idiopathique de l'adulte traitée par hydroxychloroquine et autogreffe de cellules graisseuses.

BACKGROUND: Idiopathic lipoatrophic panniculitis is a rare disease. Various terms are used in the literature to describe similar entities: annular lipoatrophy of the ankles, lipophagic panniculitis and lipoatrophic panniculitis. In any event, it consists of an inflammatory process progressing to necrosis of the fatty tissue. PATIENTS AND METHODS: Herein we report the case of a 37-year-old woman presenting characteristic signs: erythematous, annular, symmetrical, painful, inflammatory lesions of the lower extremities, progressing to areas of atrophy with an indurated inflammatory periphery. Histopathology showed lobular panniculitis composed primarily of lymphocytes and macrophages. The etiological investigations ruled out all other causes of lobular panniculitis. The patient was successfully treated with hydroxychloroquine 400mg/day for 1 year and she subsequently underwent fat grafting on the secondary lesions. DISCUSSION: Our literature review identified 23 reported cases of idiopathic lipoatrophic panniculitis since 2006, including 10 cases of annular ankle lipoatrophy. The most commonly used treatments are corticosteroids, hydroxychloroquine and methotrexate, with good efficacy being seen on the inflammatory phase. This disease seems a good indication for fat grafting to limit aesthetic sequelae. CONCLUSION: Lipoatrophic panniculitis, a rare disease, results in significant aesthetic sequelae which may be limited by early treatment initiation. The benefit-risk ratio of hydroxychloroquine suggests its value as first-line therapy in this pathology. Our case also demonstrates the value of filling of the secondary atrophy by fat grafting after the inflammatory period has ended.