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1.
Korean J Gastroenterol ; 74(3): 175-182, 2019 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-31554034

RESUMO

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.


Assuntos
Artrite/diagnóstico , Pancreatite/diagnóstico , Paniculite/diagnóstico , Artrite/tratamento farmacológico , Artrite/patologia , Artrite Gotosa/diagnóstico , Osso e Ossos/diagnóstico por imagem , Celulite (Flegmão)/diagnóstico , Diagnóstico Diferencial , Eritema/diagnóstico , Eritema/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Octreotida/uso terapêutico , Pancreatite/tratamento farmacológico , Pancreatite/patologia , Paniculite/tratamento farmacológico , Paniculite/patologia , Tomografia Computadorizada por Raios X
2.
Arkh Patol ; 81(3): 37-44, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31317929

RESUMO

Panniculitis is a heterogeneous group of diseases that are characterized by a subcutaneous adipose tissue (SAT) lesion and frequently occur with involvement of the musculoskeletal system and viscera in the process. This is a chronic multiple organ disease. The gold standard for its diagnosis is noted to be a morphological study. The paper presents the current classification of panniculitis. OBJECTIVE: To study the morphological features of idiopathic lobular panniculitis (ILP) in accordance with the nature of the course and clinical forms of the disease. SUBJECT AND METHODS: Biopsy specimens were studied in 62 patients with various clinical forms of ILP. Biopsy and surgical materials were investigated by light optical morphological methods. The results were statistically processed using a statistical analysis software Statistica Version 10 package for Windows ('StatSoft Inc.', USA). The differences were considered statistically significant at an error level of p<0.05. Methods, such as Pearson's c2 test (analysis of contingency tables), Student's t-test, Z-test for comparison of proportions, nonparametric tests, such as Mann-Whitney U-test, Kruskal-Wallis test, were used to assess the results. RESULTS: The nature of the morphological parameters of the disease corresponded to the course of ILP. The acute course of the disease was characterized by the predominance of liponecrosis and inflammation concurrent with productive-destructive vasculitis. In the chronic course, there was a preponderance of lymphohistiocytic infiltrate with gigantic macrophages (lipophages) and granuloma-like structures. The above morphological changes were characteristic of the phagocytic morphological stage of node formation. The fibroplastic stage of panniculitis was absent in this study. The morphological features of the disease were shown depending on the form of idiopathic lobular panniculitis, which may be of diagnostic value in the differential diagnosis of panniculitis. CONCLUSION: The pathologist must first of all pay attention to the preferential localization of pathological changes (in the SAT septa or slices), the presence or absence of vasculitis and the nature of the infiltrate. The skin in panniculitis is typically intact or has minimal changes following the pattern seen in reactive ones. Panniculitis shows an undulatory course, and therefore all signs of this disease are present in biopsy specimens; however, morphological signs of the acute inflammatory, lipophagic or fibroplastic stage of the disease predominate depending on the stage and activity of the process.


Assuntos
Paniculite , Vasculite , Biópsia , Granuloma , Humanos , Paniculite/diagnóstico , Paniculite/patologia , Pele/patologia , Vasculite/diagnóstico , Vasculite/patologia
3.
Diagn Pathol ; 14(1): 80, 2019 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-31311562

RESUMO

BACKGROUND: Panniculitis-like T-cell lymphoma is an uncommon type of non-Hodgkin lymphoma, occurring usually in the form of nodules within the subcutaneous fat tissue of the extremities or trunk. In the literature, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is described as a distinct type of T-cell lymphoma with a variable clinical behavior, depending on molecular phenotype of T-cell receptor (TCR) and on the presence or absence of hemophagocytic syndrome. CASE PRESENTATION: We present a bioptic and autoptic case of a 65-years old Caucasian man with panniculitic T-cell lymphoma with morphological and immunohistochemical features of SPTCL, limited to the retroperitoneal and mesenteric mass, i.e. without any cutaneous involvement, and associated with severe hemophagocytic lymphohistiocytosis. CONCLUSION: A panniculitic T-cell lymphoma with morphological and molecular features of SPTCL, which is limited to mesentery, i.e. does not involve subcutaneous fat, seems to be exceedingly rare.


Assuntos
Linfo-Histiocitose Hemofagocítica/patologia , Linfoma de Células T/patologia , Paniculite/patologia , Linfócitos T/patologia , Idoso , Autopsia , Diagnóstico Diferencial , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma de Células T/diagnóstico , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/patologia , Masculino , Paniculite/diagnóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia
4.
Clin Lab ; 65(7)2019 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-31307165

RESUMO

BACKGROUND: We report a case that presented as fever with positive Epstein-Barr Virus (EBV) IgM antibody combined with subcutaneous nodules on lower extremities and cervical lymphadenopathy firstly misdiagnosed as infectious mononucleosis, which was proven as subcutaneous panniculitis-like T-cell lymphoma by subcutaneous nodule biopsies. METHODS: Appropriate serum and bacteriological laboratory tests were carried out for the cause of fever. An ultrasound and subcutaneous nodule biopsies were performed. RESULTS: EBV IgM antibody was positive. An ultrasound revealed multiple subcutaneous nodules, which were prone to be lipoma on lower extremities and cervical lymphadenopathy. Subcutaneous nodule biopsies were firstly misdiagnosed as lipoma, while pathology consultation for the subcutaneous nodule biopsies diagnosed subcutaneous panniculitis-like T-cell lymphoma. CONCLUSIONS: When patients have persistent fever with positive EBV IgM antibody combined other system involvements, especially lymphadenopathy and multiple subcutaneous nodules, it should differentiate lymphoma from infectious diseases.


Assuntos
Febre/diagnóstico , Imunoglobulina M/imunologia , Mononucleose Infecciosa/diagnóstico , Extremidade Inferior/patologia , Linfadenopatia/diagnóstico , Linfoma de Células T/diagnóstico , Paniculite/diagnóstico , Tela Subcutânea/patologia , Adulto , Anticorpos Antivirais/imunologia , Biópsia , Diagnóstico Diferencial , Feminino , Febre/etiologia , Herpesvirus Humano 4/imunologia , Herpesvirus Humano 4/fisiologia , Humanos , Mononucleose Infecciosa/complicações , Mononucleose Infecciosa/virologia , Extremidade Inferior/virologia , Linfadenopatia/etiologia , Linfoma de Células T/complicações , Pescoço , Paniculite/complicações , Encaminhamento e Consulta , Tela Subcutânea/virologia
5.
Dermatol Online J ; 25(5)2019 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-31220899

RESUMO

Factitious disorder imposed on self is characterized by self-induction. Dermatitis artefacta, the cutaneous subtype of factitious disorder imposed on self, can have a variety of atypical presentations. A 36-year-old woman with an extensive past medical history presented with painful nodules on her abdomen, thighs, and arms. Histologic evaluation identified panniculitis with foreign body material seen under polarization. Chart review from previous hospital visits established a history of factitious disorder imposed on self and upon subsequent search of the hospital room, syringes with an unknown substance were found. Factitial panniculitis should be considered in cases with atypical lesions or locations that do not conform to the presentation of organic causes of panniculitis. Management should include a multidisciplinary approach that prioritizes patient safety and establishes a therapeutic patient-provider relationship.


Assuntos
Transtornos Autoinduzidos/diagnóstico , Corpos Estranhos/patologia , Paniculite/patologia , Pele/patologia , Adulto , Feminino , Humanos , Paniculite/diagnóstico
6.
Rinsho Ketsueki ; 60(5): 372-377, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31167997

RESUMO

A 66-year-old male presented with fever and erythema at our hospital, and leukoerythroblastosis, anemia, thrombocytopenia, and multiple low-density lesions in the moderately enlarged spleen were detected. Skin tissue revealed CD8+ T cells with the expression of cytotoxic molecule markers involving fat lobules, and subcutaneous panniculitis T-cell lymphoma (SPTCL) was diagnosed. The bone marrow displayed no infiltration of lymphoid tumor cells, but hyperplasia of granulocytes and megakaryocytes with grade 2 stromal fibrosis. In addition, the bone marrow exhibited diffuse 18F-fluorodeoxyglucose (FDG) accumulation on FDG positron-emission tomography/computed tomography (FDG-PET/CT). Although chemotherapy improved SPTCL, the patient died from leukocytosis with leukoerythroblastosis. We obtained negative results for the JAK2 V617F mutation, and CD34+ cells were elevated in the bone marrow compared with the levels at initial examination. The final diagnosis was concurrent myelodysplastic syndrome (MDS) with fibrosis and SPTCL. This report highlights that it is essential to consider MDS or other myeloproliferative neoplasms (MPN) as possible complications when malignant lymphoma complicates myelofibrosis in the absence of bone marrow infiltration of lymphoma cells. Perhaps, the assessment of clonal markers of MPN and FDG accumulation patterns in the bone marrow by FDG-PET/CT could enable differentiation.


Assuntos
Linfoma de Células T/diagnóstico , Síndromes Mielodisplásicas/diagnóstico , Paniculite/diagnóstico , Idoso , Linfócitos T CD8-Positivos , Fluordesoxiglucose F18 , Humanos , Masculino , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons
8.
J Cutan Pathol ; 46(10): 775-777, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31095750

RESUMO

Extramedullary hematopoiesis (EMH) in adults is a rare event, usually associated with myeloid disorders, and can be the first sign of chronic myelomonocytic leukemia. We report a case of EMH presenting as a hemorrhagic panniculitis in a patient who did not apparently present any myeloid disorders. The patient received previous chemotherapy for mantle cell lymphoma localized to the large bowel and had been in complete remission for 3 years. The diagnosis of EMH was achieved after a deep incisional biopsy of a nodule from the thigh, and with pathological examination that included immunohistochemical studies. After a follow-up of 14 months, the patient developed an acute myeloid leukemia, classified as therapy-related myeloid neoplasm, according to the 2016 World Health Organization (WHO) classification of hematological malignancies. As shown by the rare cases described in literature, the presence of cutaneous EMH should always be a trigger for investigating the patient's hematological system; also, in our experience, a long follow-up is mandatory.


Assuntos
Hematopoese Extramedular , Hemorragia , Leucemia Mieloide Aguda , Linfoma de Célula do Manto , Segunda Neoplasia Primária , Paniculite , Neoplasias Cutâneas , Idoso , Hemorragia/diagnóstico , Hemorragia/metabolismo , Hemorragia/patologia , Humanos , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/metabolismo , Leucemia Mieloide Aguda/patologia , Linfoma de Célula do Manto/tratamento farmacológico , Linfoma de Célula do Manto/metabolismo , Linfoma de Célula do Manto/patologia , Masculino , Segunda Neoplasia Primária/diagnóstico , Segunda Neoplasia Primária/metabolismo , Segunda Neoplasia Primária/patologia , Paniculite/diagnóstico , Paniculite/metabolismo , Paniculite/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia
9.
Pathol Res Pract ; 215(8): 152400, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30944066

RESUMO

Sickle cell disease (SCD) is a hereditary blood disorder that often has multiple comorbidities. Patients occasionally develop malignant neoplasms, but the risk of lymphoma in SCD is currently unknown. Here, we report a unique case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 25-year-old male patient with SCD. The patient suffered from episodes of sickling crisis since his initial SCD diagnosis and had been treated with supportive care. Hydroxyurea was added at the age of 23 years old. Two years later, he presented with right cheek swelling, and the biopsy showed a lymphohistiocytic infiltrate within adipose tissue resembling lobular panniculitis. Immunohistochemistry demonstrated CD8/ß-F1-positive T-cells around the fat vacuoles, with a high proliferative index. The histopathologic features suggested a diagnosis of SPTCL. A subsequent TCRß gene rearrangement analysis detected a clonal amplicon, confirming the diagnosis. Because of the lack of systemic symptoms, the patient received conservative therapy with prednisone and responded well with resolution of his right cheek swelling within one month. To the best of our knowledge, this is the first reported case of SPTCL associated with SCD. The proposed lymphomagenesis in the setting of SCD is also discussed.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/patologia , Linfoma de Células T/patologia , Paniculite/patologia , Adulto , Biópsia/métodos , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica/métodos , Linfoma de Células T/diagnóstico , Masculino , Paniculite/diagnóstico
10.
J Cutan Pathol ; 46(7): 538-541, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30957249

RESUMO

Secondary cutaneous involvement by mantle cell lymphoma (MCL), an uncommon aggressive B-cell malignancy, predominantly involves the dermis, with few reports of pannicular involvement. Lymphocytic infiltration of subcutaneous tissue is associated with inflammatory panniculitides and certain T-cell lymphomas, primarily subcutaneous panniculitis-like T-Cell lymphoma (SPTCL), which is characterized by rimming of adipocytes by tumor cells. We report the case of a 69-year-old man with a history of systemic nodal MCL who presented with subcutaneous nodules on his lower extremities after receiving multi-agent chemotherapy. Biopsies showed a dense infiltrate of atypical, mitotically active, monomorphic, medium-sized lymphoid cells in the subcutaneous fat with prominent rimming of the adipocytes by the tumor cells. These features were not morphologically typical of MCL. Immunohistochemistry showed these cells to be CD20+, CD5+ B-cells with strong cyclin D1 expression; fluorescence in situ hybridization (FISH) analysis was positive for t(11;14)(q13;32), confirming the diagnosis of secondary cutaneous involvement of MCL. This represents an exceptional report of cutaneous MCL presenting clinically and histologically with a panniculitis-type pattern and adipocyte rimming, histomorphologically mimicking SPTCL. Noteworthy examples, such as this report, support the practice of utilizing clinical correlation, immunohistochemistry, and/or molecular cytogenetics to confirm the diagnosis of any case suspicious for cutaneous lymphoma.


Assuntos
Linfoma de Célula do Manto , Linfoma de Células T , Paniculite , Neoplasias Cutâneas , Idoso , Antígenos CD20/genética , Antígenos CD20/metabolismo , Linfócitos B/metabolismo , Linfócitos B/patologia , Antígenos CD5/genética , Antígenos CD5/metabolismo , Cromossomos Humanos Par 11/genética , Cromossomos Humanos Par 11/metabolismo , Cromossomos Humanos Par 14/genética , Cromossomos Humanos Par 14/metabolismo , Ciclina D1/genética , Ciclina D1/metabolismo , Diagnóstico Diferencial , Humanos , Linfoma de Célula do Manto/diagnóstico , Linfoma de Célula do Manto/genética , Linfoma de Célula do Manto/metabolismo , Linfoma de Célula do Manto/patologia , Linfoma de Células T/diagnóstico , Linfoma de Células T/genética , Linfoma de Células T/metabolismo , Linfoma de Células T/patologia , Masculino , Paniculite/diagnóstico , Paniculite/genética , Paniculite/metabolismo , Paniculite/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia , Translocação Genética
11.
Am J Dermatopathol ; 41(9): 667-670, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30908292

RESUMO

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma preferentially localized in the subcutaneous adipose tissue and composed of cytotoxic T cells with an α/ß immunophenotype. The neoplastic T cells can be variably admixed with other inflammatory cells, including histiocytes, which can rarely form noncaseating granulomas. We present a case of SPTCL in which granulomas are the predominant feature, composing 75%-80% of the inflammatory infiltrate. The top differential diagnoses included infectious and autoimmune etiologies. However, special stains for microorganisms were negative, and immunohistochemical analysis of the atypical lymphocytes showed a CD3, CD8, TIA-1+, T-cell receptor (TCR) beta+, and CD4 infiltrate with a high Ki67 proliferation index of approximately 30%. TCR gene rearrangement studies by polymerase chain reaction with confirmation by high-throughput sequencing were necessary to exclude an autoimmune etiology, specifically lupus erythematosus panniculitis. To the best of our knowledge, only 1 other case of SPTCL with prominent granulomas has been reported in the literature. It is important for dermatopathologists to recognize this presentation of SPTCL. SPTCL with predominant granulomas should be included in the differential diagnosis of granulomatous panniculitis along with infectious and autoimmune panniculitides as well as other granulomatous lymphomas.


Assuntos
Granuloma/patologia , Linfoma de Células T/patologia , Paniculite/patologia , Neoplasias Cutâneas/patologia , Linfócitos T Citotóxicos/patologia , Biópsia por Agulha , Diagnóstico Diferencial , Feminino , Granuloma/diagnóstico , Humanos , Imuno-Histoquímica , Linfoma de Células T/diagnóstico , Linfoma de Células T/radioterapia , Pessoa de Meia-Idade , Paniculite/diagnóstico , Paniculite/radioterapia , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Prognóstico , Medição de Risco , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/radioterapia , Resultado do Tratamento
13.
Pediatr Dermatol ; 36(2): 258-259, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30746751

RESUMO

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.


Assuntos
Proteínas de Ligação a DNA/deficiência , Síndromes de Imunodeficiência/diagnóstico , Paniculite/diagnóstico , Fatores de Transcrição/deficiência , Antialérgicos/uso terapêutico , Cetirizina/uso terapêutico , Pré-Escolar , Proteínas de Ligação a DNA/genética , Fármacos Dermatológicos/administração & dosagem , Feminino , Humanos , Síndromes de Imunodeficiência/genética , Furoato de Mometasona/administração & dosagem , Mutação , Paniculite/tratamento farmacológico , Paniculite/genética , Pele/patologia , Fatores de Transcrição/genética
16.
Vet Clin North Am Small Anim Pract ; 49(1): 27-36, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30390792

RESUMO

Canine sterile pyogranulomatous dermatitis and panniculitis is an infrequently described syndrome. No autoantigen, or exogenous antigen, inflammatory stimulus has been identified. This syndrome is characterized by pyogranulomatous nodules, plaques, and ulcers of variable extent and severity. Prodromal and concurrent nonspecific clinical and hematologic signs of inflammation may occur. This waxing and waning condition is typically responsive to systemic immunomodulation. Lifelong therapy may be required to prevent relapse. Differential diagnoses include bacterial and fungal nodular dermatoses, neoplasia, and cutaneous reactive histiocytosis. Diagnosis is achieved via diagnostic exclusion of infectious causes and supportive histopathology findings.


Assuntos
Dermatite/veterinária , Doenças do Cão/diagnóstico , Granuloma/veterinária , Paniculite/veterinária , Animais , Dermatite/diagnóstico , Doenças do Cão/microbiologia , Cães , Granuloma/diagnóstico , Paniculite/diagnóstico , Medicina Veterinária/tendências
19.
Nat Genet ; 50(12): 1650-1657, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30374066

RESUMO

Subcutaneous panniculitis-like T cell lymphoma (SPTCL), a non-Hodgkin lymphoma, can be associated with hemophagocytic lymphohistiocytosis (HLH), a life-threatening immune activation that adversely affects survival1,2. T cell immunoglobulin mucin 3 (TIM-3) is a modulator of immune responses expressed on subgroups of T and innate immune cells. We identify in ~60% of SPTCL cases germline, loss-of-function, missense variants altering highly conserved residues of TIM-3, c.245A>G (p.Tyr82Cys) and c.291A>G (p.Ile97Met), each with specific geographic distribution. The variant encoding p.Tyr82Cys TIM-3 occurs on a potential founder chromosome in patients with East Asian and Polynesian ancestry, while p.Ile97Met TIM-3 occurs in patients with European ancestry. Both variants induce protein misfolding and abrogate TIM-3's plasma membrane expression, leading to persistent immune activation and increased production of inflammatory cytokines, including tumor necrosis factor-α and interleukin-1ß, promoting HLH and SPTCL. Our findings highlight HLH-SPTCL as a new genetic entity and identify mutations causing TIM-3 alterations as a causative genetic defect in SPTCL. While HLH-SPTCL patients with mutant TIM-3 benefit from immunomodulation, therapeutic repression of the TIM-3 checkpoint may have adverse consequences.


Assuntos
Mutação em Linhagem Germinativa , Receptor Celular 2 do Vírus da Hepatite A/genética , Linfo-Histiocitose Hemofagocítica/genética , Linfoma de Células T/genética , Paniculite/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Diagnóstico Diferencial , Feminino , Predisposição Genética para Doença , Humanos , Lactente , Linfo-Histiocitose Hemofagocítica/classificação , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma de Células T/classificação , Linfoma de Células T/diagnóstico , Masculino , Pessoa de Meia-Idade , Paniculite/classificação , Paniculite/diagnóstico , Linhagem , Sequenciamento Completo do Exoma , Adulto Jovem
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