Comportamiento clínico-epidemiológico del eritema nudoso en el Hospital Provincial Docente Clínico Quirúrgico León Cuervo Rubio / Epidemiological clinical behavior of erythema nodosum at the Provincial Teaching Clinical Surgical Hospital Leon Cuervo Rubio

RESUMEN Introducción: el eritema nudoso es la variante clínico-patológica más frecuente de la paniculitis. Es una reacción cutánea inmunológica en respuesta a un amplio espectro de agentes etiológicos. Objetivo: caracterizar el comportamiento clínico-epidemiológico del eritema nudoso. Materiales y métodos: se realizó un estudio descriptivo, prospectivo y longitudinal en pacientes con diagnóstico de eritema nudoso, ingresados en el Hospital Provincial Docente Clínico Quirúrgico León Cuervo Rubio, de Pinar del Río, en el período de enero de 2017 a diciembre de 2018. El universo estuvo constituido por 34 pacientes con diagnóstico clínico de eritema nudoso, hospitalizados en los servicios de Medicina Interna y Dermatología. Para la recogida de la información se utilizaron la historia clínica, el examen físico y los exámenes de laboratorio. Se utilizaron métodos teóricos, empíricos y estadísticos; de los últimos se empleó la estadística descriptiva a través de frecuencias absolutas y porcentaje. Resultados: el mayor grupo de pacientes estuvo entre 30 y 39 años, y fue del sexo femenino. Las lesiones fueron más frecuentes en los meses de invierno. El tipo de eritema nudoso idiopático prevaleció. Predominó el tiempo de evolución de 1 a 3 meses. Como causa secundaria predominaron las infecciones, y los tratamientos más usado fueron los antiinflamatorios no esteroideos. Conclusiones: se trata de una importante enfermedad de salud, cuyo un diagnóstico etiológico temprano permite un tratamiento adecuado. Resulta difícil su identificación y manejo tanto en la atención primaria como en la secundaria (AU).

ABSTRACT Introduction: erythema nodosum is the most common clinical-pathological variant of panniculitis. It is an immunological skin reaction in response to a wide spectrum of etiologic agents. Objective: to characterize the clinical-epidemiological behavior of erythema nodosum. Materials and methods: a descriptive, prospective and longitudinal study was carried out in patients with diagnosis of erythema nodosum, admitted to the Provincial Teaching Clinical Surgical Hospital León Cuervo Rubio, of Pinar del Río, in the period January 2017 to December 2018. The universe was formed by 34 patients with clinical diagnosis of erythema nodosum, hospitalized in the services of Internal Medicine and Dermatology. Medical records, physical examination and laboratory tests were used for the collection of information. Theoretical, empirical and statistical methods were used; descriptive statistics was used through absolute frequencies and percentage. Results: the largest group of patients was between 30 and 39 years, and they were female. Injuries were more frequent in the winter months. The type of idiopathic erythema nodosum prevailed. The time of evolution prevailed from 1 to 3 months. Infections predominated as a secondary cause, and the most commonly used treatments were non-steroidal anti-inflammatory drugs. Conclusions: it is an important health condition, whose early etiological diagnosis allows the proper treatment. Its identification and management in both primary and secondary care is difficult (AU).

A case report and literature review of cutaneous intravascular large B-cell lymphoma presenting clinically as panniculitis: a difficult diagnosis, but a good prognosis,

Abstract Intravascular large B-cell lymphoma is a rare, non-mass-forming, extranodal large B-cell lymphoma subtype characterized by the presence of tumor cells in the lumens of vessels. It is divided into two major types: classical and Asian. Patients presenting only with skin involvement are mostly female, at a younger age than classical intravascular large B-cell lymphoma patients, and have a better prognosis. Since the diagnosis of cases with isolated skin involvement is difficult, keeping this entity in mind, performing a careful microscopic examination, and applying new, effective treatment regimens will make it possible to achieve better clinical outcomes in these cases.

Necrosis grasa del recién nacido / Fat necrosis of the newborn

Resumen: Introducción: Las paniculitis constituyen un grupo de enfermedades que afectan al tejido graso subcutáneo y se manifiestan clínicamente como nódulos. Su patogenia no es del todo clara, por lo general es asintomático. El diagnóstico confirmatorio es histológico. Objetivo: describir las características clínicas e histopatológicas de un caso de necrosis grasa, una forma especifica de paniculitis en el recién nacido (RN). Caso Clínico: RN femenino de 40 semanas, parto cesárea de urgencia por taqui cardia fetal más meconio, APGAR 7-8-9. Requirió oxigeno y presión positiva por 5 min. Al quinto día de vida presentó aumento de volumen en tronco posterior, de coloración eritematosa - violácea, remitente a la palpación, no doloroso. Ecotomografría de piel y partes blandas informó aumento de la ecogenecidad del tejido celular subcutáneo y pérdida de definición de las celdillas adiposas de aproximadamente 42.3 x 9.7 x 20.1 mm, sin presencia de vascularización. Biopsia de piel: epidermis con acantosis irregular y ortoqueratosis en cestas; dermis papilar con infiltrado inflamatorio y dermis reticular y tejido adiposo con presencia de infiltrado linfohistocitiario con tendencia a la formación de nódulos, sin compromiso vascular, leves depósitos de colesterol; compatible con necrosis grasa del recién nacido (NGRN). Paciente tuvo regresión completa de la lesión a los tres meses de vida. Conclusiones: se describe un caso compatible clínica e histológicamente con NGRN; sin complica ciones durante su observación. Por lo general esta patología tiene buen pronóstico, con resolución espontánea, como en nuestro caso.

Abstract: Introduction: Panniculitis is a group of diseases that affect subcutaneous fat tissue and clinically manifest as nodules. Its pathogenesis is not entirely clear, and it is usually asymptomatic. The confirma tory diagnosis is histological. Objective: To describe the clinical and histopathological characteristics of a case of fat necrosis, a specific form of panniculitis in the newborn (NB). Clinical Case: 40-week female NB, born by emergency cesarean section due to fetal tachycardia with meconium, Apgar score 7-8-9. She required oxygen and positive pressure for five minutes. On the fifth day of life, she presen ted an increased volume in the posterior trunk region, with an erythematous - purplish discoloration, which is soft and non-tender to palpation. Skin and soft tissues ultrasound showed increased echo genicity of the subcutaneous cellular tissue and loss of definition of the adipocytes of 42.3 x 9.7 x 20.1 mm approximately, without vascularization. Skin biopsy showed epidermis with irregular acanthosis and basket-weave orthokeratosis; papillary dermis with inflammatory infiltrate, and reticular dermis and adipose tissue with presence of lymphohistiocytic infiltrate with a tendency to form nodules, without vascular involvement, and small cholesterol deposits, compatible with subcutaneous fat ne crosis (SBFN) of the newborn. The patient at three months of age had complete regression of the lesion. Conclusions: a clinically and histologically compatible case with SBFN is described, that did not present complications during observation. In general, this pathology has a good prognosis, with spontaneous resolution as in our case.

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.

Oligoartritis, paniculitis y pioderma gangrenoso como manifestaciones de un Linfoma no Hodgkin / Oligoarthritis, panniculitis and pyoderma gangrenosum associated with non-Hodgkin lymphoma: report of one case

An assortment of clinical and laboratory abnormalities may occur as paraneoplastic syndromes in lymphomas. Rheumatological and dermatological manifestations such as paraneoplastic arthritis and pyoderma gangrenosum must be underscored. We report a 28 years old woman who developed pyoderma gangrenosum and two years later presented with arthritis of knees and ankles associated with panniculitis interpreted as erythema induratum that was pathologically confirmed. She developed a reactivation of pyoderma gangrenosum, that was refractory to treatment. Complementary studies showed a pulmonary nodule and a right paravertebral mass with involvement of the psoas muscle. Biopsies of both masses and a new pathological skin study demonstrated a large B-cell non-Hodgkin's lymphoma.

Primer caso reportado en cuba de paniculitis mixta idiopática / First case reported in Cuba of Idiopathic mixed panniculitis

Las paniculitis son un grupo de enfermedades caracterizadas por la presencia de infiltrado inflamatorio en el tejido celular subcutáneo o panículo adiposo. Se presenta el caso de una paciente femenina de 42 años de edad, de piel blanca con lesiones de color oscuro en la piel de ambos miembros superiores e inferiores con induración dolorosa por debajo de la piel, desde hace unos 15 años. Por biopsia cutánea había sido diagnosticada como una vasculitis por hipersensibilidad que fue tratada con prednisona, azatioprina, danazol y gammaglobulina endovenosa, sin mejoría evidente. Fue remitida a nuestro centro donde se le realizó biopsia quirúrgica de piel y tejido celular subcutáneo que dio como resultado paniculitis septolobular a predominio septal, con signos de vasculitis. Por la clasificación histopatológica utilizada y el cuadro clínico que presentaba se concluyó que la paciente padece de paniculitis mixta idiopática, entidad no descrita antes en Cuba...

Panniculitis is a group of diseases characterized by the presence of inflammatory infiltration in subcutaneous cell tissue or adipose panniculous. We describe a 42 year-old white female patient with dark skin lesions in arms and feet with painful induration under the skin, lasting about 15 years. By cutaneous biopsy diagnose of vasculitis for hipersensibility was made which was treated with prednisone, azathioprine, danazol and endovenous gammaglobulin with no evidence of recovery. The patient was sent to our institute and a surgical skin and subcutaneous cell tissue biopsy was made resulting in septolobular panniculitis with septal predominance and signs of vasculitis. Considering the histopathological classification used and the clinical findings final diagnosis was mixed idiopathic pannicultis, entity not described before in Cuba...

Arteriolopatía calcificante urémica / Uremic calcifying arteriopathy

La arteriolopatía calcificante urémica es una complicación poco frecuente de la enfermedad renal crónica y se asocia con elevada tasa de mortalidad. A propósito se describió el caso clínico de un hombre de 59 años de edad, con insuficiencia renal crónica terminal que se hallaba en programa de hemodiálisis convencional, en régimen de 3 veces/semana, desde el año 2007. Se le diagnosticó arteriolopatía calcificante urémica grave tras sospecha clínica y biopsia de lesiones cutáneas, con un desenlace fatal. Reconocemos que se requiere un alto índice de sospecha clínica para su diagnóstico precoz y no siempre se asocia a hiperparatiroidismo secundario grave, en su patogenia se imbrican múltiples factores

The calcifying arteriolopathy is an uncommon complication of chronic renal disease and it is associated with a high rate of mortality. Authors present the clinical case of a man aged 59 presenting with terminal chronic renal failure under a three times/week conventional hemodialysis program from 2007. He was diagnosed with a severe uremic calcifying arteriolopathy after a clinical suspicion and biopsy of cutaneous lesions with a fatal outcome. It is recognized that it is necessary a high rate of clinical suspicion for its early diagnosis and not always it is associated with a severe secondary hyperparathyroidism where multiple factors are involved in its pathogenesis

case of lipomembranous panniculitis associated with rheumatoid arthritis

Lipomembranous panniculitis is a special type of fat necrosis which has been associated with several clinical conditions, mainly chronic venous insufficiency and connective tissue disorders such as lupus profundus, morphea, systemic sclerosis, and dermatomyositis .We report a 50 year old woman with known rheumatoid arthritis who presented with a painful indurated erythematous plaque on her right leg. Histologic examination demonstrated cystic areas of fat necrosis lined by wavy eosinophilic hyaline membrane with convoluted projections of the membrane into the cysts. This finding is diagnostic of lipomembranous panniculitis. To our knowledge, this is the first report on lipomembranous panniculitis associated with rheumatoid arthritis

Subcutaneous panniculitis-like T-cell cutaneous lymphoma.

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.

Paniculitis neutrofílica asociada a la leucemia promielocítica aguda / Neutrophilic panniculitis associated with acute piomyelocytic leukemia

La paniculitis neutrofílica (PN) asociada a síndrome mielodisplásico es una condición muy rara. Presentamos un paciente con PN como parte de una manifestación inicial de síndrome mielodisplásico (Leucemia promielocítica aguda). La PN aparece como una erupción nodular subcutánea, dolorosa, acompañada de síntomas sistémicos, cuyo estudio histopatológico evidencia un infiltrado neutrofílico lobular sin vasculitis, limitado al tejido subcutáneo. Debe ser diferenciada de otros tipos de dermatosis neutrofílicos y de otras hipodermitis lobulares. La PN se asocia significativamente a procesos mielodisplásicos y es altamente sensible al tratamiento con cortidoides sistémicos.

Systemic lupus erythematosus presenting as lupus panniculitis

Systemic lupus erythematosus [SLE] is an autoimmune disease that presents in diverse ways. Panniculitis is not a common presentation of SLE. Its overall incidence is estimated to be 1-3%, occurring predominantly in women. The lesions have predilection for the fatty areas of body such as shoulders, hips, thighs and face. We present here a case of a young female who visited us with the complaint of soft tissue swellings over buttocks, shins, arms and face. The swellings appeared to be ordinary abscesses at first sight. She also had history of arthralgia and low grade fever. Laboratory examination demonstrated high litres of antinucleal antigen and anti-double-stranded DNA antibodies in serum which led us to suspect lupus panniculitis. Histopathology revealed features of lobular panniculitis with lymphohistiocytic infiltrate deep in the subcutis. We report this case because this is a rare presentation of lupus erythematosus

Panniculitis-like T-cell lymphoma with fatal termination.

A 58-year-old male presented with three weeks history of multiple subcutaneous nodules and fever. Histopathological feature of the nodule was characteristic of panniculitis. Three months later, the patient presented with carvical lymphadenopathy and compressive thoracic myelopathy and a diagnosis of diffuse mixed- cell lymphoma was established. Immunohistochemical study of subcutaneous lesions confirmed their T-cell origin. Chemotherapy was started but patient succumbed to his disease.

Subcutaneous panniculitis-like T cell lymphoma in a HIV positive patient.

Immunocompromised patients are at increased risk for developing certain malignant tumors, particularly aggressive B cell lymphomas and extranodal lymphomas like primary central nervous system lymphoma and primary effusion lymphoma. T cell lymphomas are uncommon in these patients. We report a rare case of subcutaneous panniculitis-like T cell lymphoma in a HIV positive patient who presented with multiple subcutaneous nodules.

Panniculitides--a clinicopathologic study.

OBJECTIVES: To describe the clinical and pathologic features of panniculitides, an uncommon group of diseases, where the available Indian literature is sparse. MATERIAL AND METHODS: Prospective four years study of patients presenting with panniculitis to a large teaching hospital in North India. RESULTS: Erythema nodosum (EN) was the commonest panniculitis (79.3% cases) in our cohort. Other causes included erythema induratum, Weber Christian disease, cutaneous polyarteritis nodosa and panniculitis associated with Takayasu's arteritis and dermatomyositis-lupus overlap. Elevated ESR and leukocytosis were the common laboratory abnormalities encountered. Septal panniculitis without vasculitis was the most frequent histopathologic pattern observed. Patients of EN did well on nonsteroidal anti-inflammatory drugs while corticosteroids with or without azathioprine were used to treat other panniculitides. CONCLUSIONS: Physician awareness, clinical pattern recognition and histopathological confirmation, all play a key role in the identification and management of panniculitis.

Paniculitis por gota / Panniculitis for gout

Presentamos el caso de un paciente de 39 años con multiples nodulos subcutaneos en rodillas, codos, cuellos de pie y pequeñas articulaciones de manos de seis años de evolucion asociado con la presencia de paniculitis por cristales de urato monosodico. La presencia de paniculitis en la gota es inusual, ha sido informada pocas veces en la literatura y creemos que debe considerarse a la gota dentro del diagnostico diferencial de ésta

Paniculite eosinofílica induzida por ibuprofeno: relato de um caso / Eosinophilic panniculitis induced by ibuprofen: a case report

A paniculite eosinofílica é dermatose rara, associada a várias causas e caracterizada por nódulos subcutâneos normocrômicos, assintomáticos, do tronco, membros e/ou couro cabeludo. A histopatologia revela infiltrado eosinofílico no tecido celular subcutâneo. É feito o relato do caso de um paciente com quadro característico após ingestäo de ibuprofeno

Intraabdominal panniculitis. report of three cases and review of the literature

Intraabdominal panniculitis is a rare, benign idiopathic disorder of the mesentery. Patients usually present with abdominal pain and a papable mass. The cross-sectional imaging findings are characteristic and consist of a fibrofatty central mesenteric mass lesion encapsulating the mesenteric vessels with displacement of the bowel loops that can suggest the diagnosis. Imaging is also important to establish a definitive diagnosis by an image-guided percutaneous biopsy, assess extent of the disease for selection of appropriate therapy, exclude associated abnormalities namely malignancies and for follow-up