RESUMEN
An 11-year-old neutered male large crossbreed dog was presented for investigation because of a 10-day history of progressive lethargy, hyporexia, and pyrexia. Physical and dermatological examinations were unremarkable. Blood biochemical analysis identified a marked total and ionized hypercalcemia and increased C-reactive protein concentration. Bicavitary computed tomography screening for causes of the dog's clinical and biochemical abnormalities identified a diffuse panniculitis. Histopathological examination of full-thickness skin biopsies was consistent with pyogranulomatous inflammation. Extensive histochemical staining revealed no infectious etiology. Complete clinical and biochemical remissions were observed after starting immunosuppressive, followed by tapering, doses of prednisolone, supporting an immune-mediated etiology. Key clinical message: Sterile, immune-mediated pyogranulomatous inflammation should remain a differential diagnosis for hypercalcemia in dogs. Significant dermatological disease may occur without visible abnormalities.
Panniculite pyogranulomateuse à médiation immunitaire avec hypercalcémie chez un chienUn grand chien croisé mâle castré de 11 ans a été présenté pour examen en raison d'antécédents de léthargie progressive, d'hyporexie et de pyrexie depuis 10 jours. Les examens physiques et dermatologiques étaient sans particularité. L'analyse biochimique du sang présentait une hypercalcémie totale et ionisée marquée et une concentration accrue de protéine C-réactive. Le dépistage par tomodensitométrie bicavitaire des causes des anomalies cliniques et biochimiques du chien a identifié une panniculite diffuse. L'examen histopathologique des biopsies cutanées de pleine épaisseur était compatible avec une inflammation pyogranulomateuse. Un examen par coloration histochimique extensive n'a révélé aucune étiologie infectieuse. Les rémissions cliniques et biochimiques complètes ont été observées après le début du traitement immunosuppresseur, suivies d'une diminution progressive des doses de prednisolone, confirmant une étiologie à médiation immunitaire.Message clinique clé:L'inflammation pyogranulomateuse stérile à médiation immunitaire doit rester un diagnostic différentiel de l'hypercalcémie chez le chien. Une maladie dermatologique importante peut survenir sans anomalies visibles.(Traduit par Dr Serge Messier).
Asunto(s)
Enfermedades de los Perros , Hipercalcemia , Paniculitis , Animales , Perros , Enfermedades de los Perros/diagnóstico , Enfermedades de los Perros/tratamiento farmacológico , Enfermedades de los Perros/patología , Masculino , Paniculitis/veterinaria , Paniculitis/diagnóstico , Hipercalcemia/veterinaria , Prednisolona/uso terapéutico , Inmunosupresores/uso terapéuticoAsunto(s)
Paniculitis , Deficiencia de alfa 1-Antitripsina , Humanos , Deficiencia de alfa 1-Antitripsina/complicaciones , Deficiencia de alfa 1-Antitripsina/diagnóstico , Paniculitis/etiología , Paniculitis/diagnóstico , Masculino , Femenino , alfa 1-Antitripsina/sangre , alfa 1-Antitripsina/genéticaRESUMEN
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.
Asunto(s)
Linfoma de Células T , Paniculitis de Lupus Eritematoso , Paniculitis , Humanos , Femenino , Paniculitis/diagnóstico , Paniculitis/patología , Paniculitis de Lupus Eritematoso/diagnóstico , Paniculitis de Lupus Eritematoso/tratamiento farmacológico , Paniculitis de Lupus Eritematoso/patología , Linfoma de Células T/diagnóstico , Linfoma de Células T/patología , Adulto , Diagnóstico Diferencial , Biopsia , Prednisolona/uso terapéuticoRESUMEN
Panniculitis is an inflammation that occurs in subcutaneous adipose tissue. Panniculitis includes physical panniculitis (e.g., traumatic) and infectious panniculitis (e.g., bacterial, fungal, subcutaneous panniculitis-like T cell lymphoma [SPCTL], etc.). Accurate diagnosis is crucial due to similar clinical presentation of all types of panniculitis. Here, we report a case of SPCTL which was initially diagnosed with traumatic panniculitis. A 15-year-old male patient was admitted to a previous hospital due to a progressively enlarged right flank and inguinal mass after an abdominal bruise. He was initially diagnosed with traumatic panniculitis, but the mass expanded throughout the chest and abdomen accompanied by a fever of over 11 months. Computed tomography (CT) revealed a subcutaneous mass in the anterior chest and abdominal wall. Fludeoxyglucose F18 (FDG) uptake was observed at those lesions using FDG-positron emission tomography (PET). A biopsy of the mass lesion was performed, during which SPCTL was diagnosed based on pathological examination. He was initially treated with prednisolone and cyclosporine A for two weeks. His fever went down, but subcutaneous mass in the chest and abdominal wall persisted. Therefore, he received a cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen. After 6 courses of CHOP, CT revealed no disease evidence. He remained in complete remission at 30 months of therapy.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica , Ciclofosfamida , Progresión de la Enfermedad , Doxorrubicina , Linfoma de Células T , Paniculitis , Vincristina , Humanos , Masculino , Paniculitis/diagnóstico , Paniculitis/etiología , Paniculitis/tratamiento farmacológico , Paniculitis/patología , Adolescente , Linfoma de Células T/diagnóstico , Linfoma de Células T/patología , Linfoma de Células T/diagnóstico por imagen , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Vincristina/administración & dosificación , Prednisona/administración & dosificación , Tomografía Computarizada por Rayos X , Tomografía de Emisión de Positrones , Fluorodesoxiglucosa F18 , Resultado del Tratamiento , Biopsia , Diagnóstico DiferencialRESUMEN
Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean bag cells"), including phagocytosed erythrocytes, leukocytes, and platelets. We describe a case of a 72-year-old female who presented with a papular eruption that clinically mimicked pityriasis lichenoides et varioliformis acuta (PLEVA). Given that her skin biopsy had multiple features concerning PLEVA, this diagnosis was classified as a superficial pityriasis lichenoides-like variant of CHP. The histopathologic presence of cytophagic histiocytosis prompted workup for a systemic malignancy, leading to a diagnosis of underlying acute monocytic leukemia of myeloid lineage.
Asunto(s)
Paniculitis , Humanos , Femenino , Anciano , Paniculitis/patología , Paniculitis/diagnóstico , Histiocitos/patología , Leucemia Mieloide Aguda/patología , Leucemia Mieloide Aguda/diagnóstico , Leucemia Monocítica Aguda/patología , Leucemia Monocítica Aguda/diagnóstico , Diagnóstico Diferencial , Diferenciación Celular , Monocitos/patologíaRESUMEN
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.
Asunto(s)
Artritis , Pancreatitis , Paniculitis , Humanos , Paniculitis/etiología , Paniculitis/diagnóstico , Paniculitis/complicaciones , Masculino , Artritis/etiología , Artritis/complicaciones , Pancreatitis/complicaciones , Pancreatitis/etiología , Pancreatitis/diagnóstico , Persona de Mediana Edad , Alcoholismo/complicacionesRESUMEN
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.
Asunto(s)
Linfohistiocitosis Hemofagocítica , Linfoma de Células T , Paniculitis , Humanos , Masculino , Biopsia , Diagnóstico Diferencial , Resultado Fatal , Linfohistiocitosis Hemofagocítica/patología , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/complicaciones , Linfoma de Células T/patología , Linfoma de Células T/complicaciones , Linfoma de Células T/diagnóstico , Linfoma Cutáneo de Células T/patología , Linfoma Cutáneo de Células T/complicaciones , Linfoma Cutáneo de Células T/diagnóstico , Paniculitis/patología , Paniculitis/diagnóstico , Piel/patología , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/complicaciones , Adulto JovenRESUMEN
We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and hemophagocytic syndrome (HPS) on evaluation. The histopathological examination of skin punch biopsy from the face and chest wall showed SPTCL. Given the associated HPS, he was started on steroid and multidrug chemotherapy following which he had symptomatic improvement.
Asunto(s)
Angioedema , Linfohistiocitosis Hemofagocítica , Linfoma de Células T , Paniculitis , Masculino , Humanos , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/etiología , Paniculitis/diagnóstico , Paniculitis/tratamiento farmacológico , Paniculitis/etiología , Linfoma de Células T/complicaciones , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamiento farmacológico , Piel/patología , Angioedema/patología , Fiebre/etiologíaRESUMEN
Erythema nodosum (EN) may be idiopathic or secondary, and usually resolves naturally within 1-2 months. In atypical EN cases, the rash extends beyond the lower limbs to the upper limbs and trunk, and histopathological findings may be accompanied by vasculitis in addition to septal panniculitis. Few studies have examined the differences in the clinical characteristics of patients with EN based on rash distribution. We retrospectively examined whether there was a correlation with clinical information, such as the presence or absence of underlying diseases, by classifying the patients into two groups: the lower limbs group (the EN rash was confined to the lower limbs) and the beyond lower limbs group (the EN rash appeared beyond the lower limbs). Among the 86 adult patients diagnosed with EN at the Dermatology Department of Fujita Medical University between 2015 and 2020, there were 65 cases of the lower limbs group and 21 cases of the beyond lower limbs group. The frequency of underlying diseases was significantly higher in the beyond lower limbs group (76.2%, 16 cases) than in the lower limbs group (40.0%, 26 cases; P < 0.005). Vasculitis was more notable in the beyond lower limbs group (P < 0.05). Significantly higher vasculitis was noted in the EN group with underlying diseases (30.2%, 13 cases) than in the idiopathic EN group without underlying diseases (11.6%, 5 cases; P < 0.05). Neutrophil extracellular traps were positive in approximately 40% of cases in both groups. In the beyond lower limbs group, the possibility of severe cases with underlying diseases, vasculitis, and inflammation must be considered for effective treatment.
Asunto(s)
Eritema Nudoso , Extremidad Inferior , Piel , Humanos , Eritema Nudoso/diagnóstico , Eritema Nudoso/patología , Estudios Retrospectivos , Masculino , Femenino , Persona de Mediana Edad , Adulto , Anciano , Piel/patología , Extremidad Inferior/patología , Vasculitis/patología , Vasculitis/diagnóstico , Vasculitis/complicaciones , Adulto Joven , Paniculitis/patología , Paniculitis/diagnóstico , Anciano de 80 o más Años , AdolescenteRESUMEN
Neutrophilic panniculitides are a heterogeneous group of inflammatory disorders encompassing many different entities. This review article focuses on the epidemiology, pathogenesis, clinicopathological features, diagnosis, and treatment of selected diseases. Patients often seek care due to systemic involvement, but the variable presentation of panniculitides can present a diagnostic challenge. Most therapeutic modalities for neutrophilic disorders are anecdotal at best with a notable lack of standardization of the responses to medications. There is an urgent need for a larger multi-institutional collaboration to address the unmet needs of these challenging, yet rare conditions.
Asunto(s)
Paniculitis , Humanos , Paniculitis/diagnóstico , Paniculitis/tratamiento farmacológico , Paniculitis/etiologíaAsunto(s)
Autoanticuerpos , Dermatomiositis , Paniculitis , Humanos , Dermatomiositis/inmunología , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/complicaciones , Dermatomiositis/patología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Paniculitis/patología , Paniculitis/diagnóstico , Paniculitis/inmunología , Paniculitis/tratamiento farmacológico , Enzimas Activadoras de Ubiquitina/inmunología , Enzimas Activadoras de Ubiquitina/antagonistas & inhibidores , Femenino , Piel/patología , Piel/inmunología , Resistencia a Medicamentos , Masculino , Torso , Persona de Mediana EdadRESUMEN
T cell dyscrasias that demonstrate a proclivity for the subcutaneous fat include atypical lymphocytic lobular panniculitis, lupus profundus, and primary subcutaneous T cell lymphoma, including subcutaneous panniculitis-like T cell lymphoma (SPTCL). We encountered two patients who developed fever and indurated abdominal erythema at their peginterferon alfa-2a injection sites. Biopsies showed an atypical CD8 positive, granzyme positive, CD5 negative, MXA negative lymphocytic lobular panniculitis, diagnostic of SPTCL. Peginterferon alfa-2a was held in both patients. One patient received chemotherapy with an excellent response, while the other continued to have progressive disease. Peginterferon alfa-2a is known to significantly elevate serum MXA, which may induce high levels of MXA expression at the injection site, creating a microenvironment for the development of lupus profundus, which may eventuate into SPTCL. In summation, a potential risk of peginterferon alfa-2a injections is the development of SPTCL potentially arising in a background of an exogenous interferon triggered lymphocytic panniculitis.
Asunto(s)
Interferón-alfa , Linfoma de Células T , Paniculitis , Polietilenglicoles , Proteínas Recombinantes , Humanos , Polietilenglicoles/efectos adversos , Polietilenglicoles/administración & dosificación , Interferón-alfa/efectos adversos , Interferón-alfa/administración & dosificación , Proteínas Recombinantes/efectos adversos , Proteínas Recombinantes/administración & dosificación , Paniculitis/inducido químicamente , Paniculitis/diagnóstico , Paniculitis/patología , Paniculitis/etiología , Femenino , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamiento farmacológico , Linfoma de Células T/patología , Persona de Mediana Edad , Masculino , Biopsia , AdultoRESUMEN
INTRODUCTION: Erythema nodosum (EN) is the most common form of panniculitis that predominantly affects the shins. While EN in atypical sites has been described by many authors, there are currently only case studies published on this topic. This study aimed to evaluate clinical differences between patients suffering from EN on the shins, compared to patients with EN in atypical locations. METHODS: We analyzed 105 patients in a retrospective, single-center study at a university hospital in Switzerland. Typical EN was defined as lesions, found only on the lower legs, while atypical EN as lesions on the upper legs, trunk, arms, or face, only or in addition to lesions on the lower legs. The patients were assessed for age, gender, dermatologic history, time until first medical consultation, time to diagnosis, and time until remission. Further, etiology, symptoms, and applied therapies were investigated. Findings were then compared between the typical and atypical EN cohorts. RESULTS: Overall, we included 70 patients (37.99 ± 15.67 [3-81] years) with EN solely on the shins and 35 patients (41.27 ± 16.85 [9-76] years) with EN on other locations. Interestingly, time until diagnosis was significantly shorter in atypical EN (p = 0.034, 1.14 ± 4.68 vs. 0.46 ± 1.14 months). Time to remission was similar in both groups (3.61 ± 2.73 vs. 3.05 ± 2.86 months, respectively). Sarcoidosis was the only etiologic factor significantly more frequent in atypical EN compared to typical EN (23% vs. 9%, p = 0.042). Besides that, solely subtle differences were seen regarding etiology, gender, age at onset, course of the disease, and symptoms. CONCLUSIONS: Our study suggests that only minor alterations between both study populations exist. Significant differences were found in time to diagnosis (shorter for atypical EN), as well as in sarcoidosis as an etiologic factor (more frequent in atypical EN). While adalimumab was only prescribed in atypical EN cases, prognosis seems to be similar for typical and atypical EN (similar time to remission, similar amount of reoccurring cases). Due to the limited sample size, however, our study population may have been too small to detect the relevant differences, and bigger studies may be needed.
Asunto(s)
Eritema Nudoso , Paniculitis , Sarcoidosis , Humanos , Eritema Nudoso/diagnóstico , Estudios Retrospectivos , Paniculitis/complicaciones , Paniculitis/diagnóstico , Paniculitis/patología , Resultado del TratamientoRESUMEN
A 53-year-old man with adult-onset Still's disease developed severe streptococcal toxic shock syndrome (STSS) due to Streptococcus dysgalactiae subsp. equisimilis (SDSE), following retroperitoneal panniculitis. He was receiving tocilizumab (TCZ), an interleukin-6 receptor inhibitor. The modifying effect of TCZ on the immune response and the pathophysiology of SDSE infection may have led to retroperitoneal panniculitis and atypical STSS with delayed shock and flare of soft tissue inflammation.
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Paniculitis , Choque Séptico , Infecciones Estreptocócicas , Streptococcus , Humanos , Choque Séptico/etiología , Choque Séptico/tratamiento farmacológico , Choque Séptico/diagnóstico , Choque Séptico/microbiología , Masculino , Persona de Mediana Edad , Infecciones Estreptocócicas/diagnóstico , Infecciones Estreptocócicas/tratamiento farmacológico , Infecciones Estreptocócicas/complicaciones , Infecciones Estreptocócicas/microbiología , Paniculitis/diagnóstico , Paniculitis/etiología , Paniculitis/microbiología , Paniculitis/tratamiento farmacológico , Streptococcus/inmunología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Anticuerpos Monoclonales Humanizados/efectos adversos , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/complicaciones , Enfermedad de Still del Adulto/tratamiento farmacológico , Receptores de Interleucina-6/antagonistas & inhibidores , Resultado del Tratamiento , Espacio RetroperitonealRESUMEN
BACKGROUND: Primary cutaneous lymphoma represents 0.2%-3% of all feline lymphomas, with nonepitheliotropic lymphomas being the most common. In humans and dogs, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a primary nonepitheliotropic lymphoma with a T-cell phenotype developing in the subcutis and often mimicking inflammation. OBJECTIVE: The aim of this report is to describe pathological, phenotypical and clonal features of SPTCL in cats. ANIMALS: Six cats with SPTCL were included in this study. MATERIALS AND METHODS: Skin biopsies were formalin-fixed, routinely processed and stained. Histological and immunohistochemical investigation for anti-CD18, CD204, CD79a, CD20, CD3, FeLVp27and FeLVgp70 and clonality assessment were performed. RESULTS: Four male and two female domestic shorthair cats, mean age 11.2 years, developed SPTCL in the abdominal (three), inguinal (two) and thoracic (one) regions. Variably pleomorphic neoplastic lymphoid cells were present in the panniculus in percentages, expanding the septa (six of six) and extending into fat lobules in one of six cats. Tumours were associated with elevated numbers of neutrophils (five of six), lesser macrophages (six of six) and variable necrosis (six of six). Neoplastic cells expressed CD3+ (six of six), with clonal T-cell receptor rearrangement detected in five of six cats. CONCLUSIONS AND CLINICAL RELEVANCE: This is the first description of SPTCL in cats. Lesions can be confused with panniculitis, leading to delay in diagnosis and therapy. Awareness of this neoplastic disease is relevant to avoid misdiagnoses and to gain greater knowledge about the disease in cats.
Asunto(s)
Enfermedades de los Gatos , Enfermedades de los Perros , Linfoma Cutáneo de Células T , Linfoma de Células T , Linfoma , Paniculitis , Humanos , Gatos , Masculino , Animales , Femenino , Perros , Linfoma de Células T/diagnóstico , Linfoma de Células T/veterinaria , Linfoma de Células T/patología , Paniculitis/diagnóstico , Paniculitis/veterinaria , Linfoma/veterinaria , Piel/patología , Linfoma Cutáneo de Células T/patología , Linfoma Cutáneo de Células T/veterinaria , Enfermedades de los Gatos/diagnósticoRESUMEN
Dermatomyositis constitutes a heterogeneous group of autoimmune inflammatory conditions with a wide variety of clinical outcomes. The symptomatic heterogeneity carries skin, muscle, and joint manifestations; pulmonary and cardiac involvements; and concomitant malignancy. Any of these symptoms often appear at different combinations and time courses, thus posing difficulty in early diagnosis and appropriate treatment choice. Recent progress in laboratory investigations explored the identification of several myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies, allowing precise characterization for a clinical perspective of the disease. MSAs can be detectable in approximately 80% of patients with whole dermatomyositis, some of which closely reflect unique clinical features in the particular disease subset(s), including the distribution and severity of organ involvement, treatment response, and prognosis. However, only limited evidence has been available in dermatomyositis-associated panniculitis, mostly that in anti- melanoma differentiation-associated protein 5 antibody-positive disease. We present a rare case of a patients with dermatomyositis with extensive panniculitis on the trunk whose serum IgG autoantibodies reacted with both subunits of small ubiquitin-like modifier activating enzymes (SAEs), SAE1 and SAE2. The onset of panniculitis coincided with increased disease activity, including disease-related skin manifestations, fever, dysphagia, and muscle weakness in the extremities. These symptoms responded well to a high dose of systemic steroid, but even upon receiving a high-dose intravenous immunoglobulin, the panniculitic lesions and pruritic erythema flared with tapering of steroid dose, further requiring tacrolimus and mycophenolate mofetil to achieve disease remission. To our knowledge, this is the third reported case of anti-SAE autoantibody-positive dermatomyositis with panniculitis. We aim to extend the understanding of the current limitation and further perspective in the clinical management of the extremely rare skin manifestation associated with dermatomyositis.