RESUMEN
BACKGROUND: Due to its rarity, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is often misdiagnosed as benign panniculitis, and there are no standardized treatment guidelines for SPTCL. Aurora kinase A (AURKA) plays a regulatory role in both mitosis and meiosis. Cells treated with an AURKA inhibitor showed severe mitotic delay, which triggered apoptosis. MATERIALS AND METHODS: Ten cases of SPTCL were collected in this study, and immunohistochemistry was performed to detect AURKA expression in the skin tissues of these cases. Control groups were set as follows: 1) 10 cases of inflammatory panniculitis; 2) 9 healthy individuals. Fisher's exact test was used to compare the positive rates of AURKA among various groups. RESULTS: An average onset age of 27.3 years was found in 10 SPTCL cases. Clinically, these patients primarily presented with multiple subcutaneous nodules on the trunk and lower extremities, accompanied by intermittent high fever. One case showed lymph node metastasis, while no other distant organ metastasis being observed in any case. Pathologically, there was an infiltration of a large number of atypical lymphocytes within the fat lobules, characterized as a cytotoxic type. AURKA stanning was positive in 6 out of 10 SPTCL cases, while no positive cases were found in the control groups. CONCLUSION: 1) SPTCL predominantly affects young individuals and can be identified by nodular erythema on the trunk, intermittent high fever, and infiltration of atypical cytotoxic lymphocytes within fat lobules. 2) For early-stage cases without metastasis, monotherapy with glucocorticoids or immunosuppressants such as cyclosporine can be considered. 3) High expression of AURKA in SPTCL tissues suggests that AURKA could be a potential biomarker for disease diagnosis, providing a theoretical basis for further targeted therapy.
Asunto(s)
Aurora Quinasa A , Linfoma de Células T , Paniculitis , Humanos , Aurora Quinasa A/genética , Aurora Quinasa A/metabolismo , Paniculitis/enzimología , Paniculitis/patología , Femenino , Masculino , Adulto , Linfoma de Células T/patología , Linfoma de Células T/enzimología , Linfoma de Células T/genética , Adulto Joven , Diagnóstico Diferencial , Persona de Mediana Edad , Adolescente , Piel/patología , InmunohistoquímicaAsunto(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
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 terapéuticoRESUMEN
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.
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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
ABSTRACT: A 65-year-old woman presented with unexplained weight loss, recurrent fever, and a dermatosis with painful nodules on the extremities. Biopsies showed focal lobular panniculitis with neutrophilic microgranulomas. Comprehensive investigations ruled out infection and hematologic and solid organ neoplasms. Laboratory results showed anti-Ro/SSA and anti-La/SSB antibody positivity, and elevated inflammatory markers. Dry mouth and eye were confirmed. The diagnosis of Sjögren syndrome with cutaneous panniculitis was established. Prednisone treatment with 30 mg/d resulted in remission of fever and pain improvement. This case emphasizes Sjögren syndrome as an autoimmune disease with multiple cutaneous manifestations and highlights its association with granulomatous panniculitis.
Asunto(s)
Paniculitis , Síndrome de Sjögren , Humanos , Femenino , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/tratamiento farmacológico , Anciano , Paniculitis/patología , Paniculitis/etiología , Prednisona/uso terapéutico , Granuloma/patología , Resultado del Tratamiento , BiopsiaRESUMEN
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
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
BACKGROUND: The occurrence of hemophagocytic lymphohistiocytosis (HLH) in patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) may be due to HAVCR2 gene mutation, leading to T-cell immunoglobulin and mucin domain-containing molecule 3 deficiency, T-cell and macrophage activation, and proinflammatory cytokine production. OBSERVATION: We report a patient with SPTCL and HLH for whom ruxolitinib, used as a novel treatment, showed notable therapeutic effects. CONCLUSIONS: Remission of both HAVCR2 mutation-induced high inflammatory characteristics and significant symptoms post-ruxolitinib administration suggested that patients with SPTCL and HLH may not represent typical lymphoma cases. Ruxolitinib, with its relatively low toxic side effects, can provide favorable outcomes.
Asunto(s)
Receptor 2 Celular del Virus de la Hepatitis A , Linfoma de Células T , Mutación , Nitrilos , Paniculitis , Pirazoles , Pirimidinas , Humanos , Pirazoles/uso terapéutico , Paniculitis/genética , Paniculitis/tratamiento farmacológico , Paniculitis/patología , Linfoma de Células T/tratamiento farmacológico , Linfoma de Células T/genética , Linfoma de Células T/patología , Receptor 2 Celular del Virus de la Hepatitis A/genética , Pirimidinas/uso terapéutico , Linfohistiocitosis Hemofagocítica/genética , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/patología , Masculino , Niño , FemeninoRESUMEN
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.
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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/patologíaAsunto(s)
Artritis Reumatoide , Paniculitis , Piperidinas , Pirimidinas , Pirroles , Humanos , Piperidinas/uso terapéutico , Pirimidinas/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Paniculitis/tratamiento farmacológico , Paniculitis/patología , Resultado del Tratamiento , Femenino , Pirroles/uso terapéutico , Neutrófilos/patología , Inhibidores de Proteínas Quinasas/uso terapéutico , Persona de Mediana Edad , BiopsiaRESUMEN
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.
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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
BACKGROUND: Subcutaneous fat necrosis of the newborn (SCFN) is a rare form of panniculitis manifesting as erythematous plaques or nodules at sites of brown fat in neonates. Surgical management may be indicated in severe cases; however, there is a paucity of literature compiling presentations and outcomes of these surgical patients. METHODS: The authors performed a systematic review, in consultation with a licensed librarian, on MEDLINE and Embase for studies including patients with SCFN who were surgically managed. RESULTS: The search strategy generated 705 results, among which 213 (30.2%) were excluded for lack of discussion on surgical management. Twenty-two studies discussed surgical management of SCFN in 26 patients, but in 6 of these studies the patients were not surgically managed. Ultimately, 16 articles with 16 patients who were surgically managed were included in the study. Average age at diagnosis was 11.8 ± 9.8 days; average age at surgery was 39.5 ± 70.4 days. The most common etiologies were "unknown" (6, 37.5%), therapeutic hypothermia (4, 25.0%), and birth complications (4, 25.0%). Patients harbored nodules on the back (14, 87.5%), upper extremities (7, 43.8%), lower extremities (7, 43.8%), buttocks (5, 31.3%), and head or neck (3, 18.8%). Linear regression models revealed the presence of back lesions and predicted concomitant medical complications (ß = 2.71, p = 0.021). CONCLUSIONS: Patients undergoing surgical management for SCFN most commonly harbor lesions on the back and extremities that are secondary to therapeutic hypothermia or of unknown origin. Reporting of additional cases is needed to further elucidate surgical management and outcomes.
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Necrosis Grasa , Grasa Subcutánea , Humanos , Necrosis Grasa/cirugía , Necrosis Grasa/etiología , Recién Nacido , Grasa Subcutánea/cirugía , Grasa Subcutánea/patología , Paniculitis/cirugía , Paniculitis/etiología , Paniculitis/patología , Resultado del TratamientoRESUMEN
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.
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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
ABSTRACT: Gamma/delta T-cell lymphoma is a rare and aggressive subtype of primary cutaneous lymphoma. Clinical manifestations typically include the development of subcutaneous nodules and ulcerated plaques. Some forms present as panniculitis with hemophagocytic syndrome. Prognosis is bleak, with a 10% 5-year survival rate. In this report, we present the case of a 20-year-old man from French Polynesia, referred for 18 F-FDG PET/CT because of the progressive worsening of febrile cutaneous-mucosal infiltration on the face persisting for 1 month. PET examination guided a biopsy from the right deltoid muscle, and expert histological analysis confirmed a CD8 + not otherwise specified T-cell lymphoma, granzyme+ and TCR gamma/delta.
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Linfoma Cutáneo de Células T , Linfoma de Células T , Paniculitis , Neoplasias Cutáneas , Humanos , Masculino , Adulto Joven , Fluorodesoxiglucosa F18 , Linfoma de Células T/patología , Linfoma Cutáneo de Células T/diagnóstico por imagen , Paniculitis/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Neoplasias Cutáneas/diagnóstico por imagen , Neoplasias Cutáneas/patología , Linfocitos T/patologíaAsunto(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
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.