Taxane-induced scleroderma: report of two cases / Esclerodermia inducida por taxanos: informe de dos casos

ABSTRACT Taxanes are a class of chemotherapeutic agents with common associated dermatologic adverse events, such as skin hyperpigmentation, hand-foot skin syndrome, paronychia and onycholysis. Taxane-induced scleroderma is rare. Few cases with skin findings resembling systemic sclerosis, have been reported after the administration of these agents. We report two cases with stage IV breast cancer, aged 66 and 71 years, who developed sclerodermic skin lesions in their extremities after starting treatment with placlitaxel and nabplaclitaxel respectively.

Los taxanos son agentes quimioterapéuticos cuyo uso se asocia a problemas dermatológicos tales como hiperpigmentación, síndrome manos-pies, paroniquia y onicolisis. La esclerodermia inducida por taxanos es rara, con pocos casos informados en la literatura. Informamos los casos de dos pacientes con cáncer de mama en estado IV, de 66 y 71 años, que desarrollaron lesiones esclerodérmicas en las extremidades después de ser tratadas con placlitaxel y nabplaclitaxel, respectivamente.

Escrofuloderma: tuberculosis cutánea y pulmonar asociada a coinfección COVID-19: reporte de caso / Scrofuloderma, cutaneous and pulmonary tuberculosis associated with COVID-19: report of one case

Cutaneous tuberculosis represents 1-1.5% of extrapulmonary tuberculosis, including a variety of clinical conditions. Scrofuloderma and lupus vulgaris are the most common forms. We report a 49-year-old woman who sought medical attention through tele-dermatology concerning a cervical nodule associated with suppuration and cutaneous involvement. The diagnoses of scrofuloderma and pulmonary tuberculosis were confirmed, and during her evolution she presented a coinfection with SARS-CoV-2. The possible associations between tuberculosis and COVID-19 were reviewed.

Sospecha diagnóstica de síndrome de Ehlers Danlos tipo vascular: reporte de un caso y revisión de literatura / Suspected vascular ehlers danlos syndrome: case report

Ehlers Danlos Syndrome comprises a heterogeneous group of genetic disorders of the connective tissue, due to defects in collagen or its modifying enzymes. We report a 21 years old male presenting with translucent skin revealing the subcutaneous venous pattern. He had a thin face, large-appearing eyes, thin lips, thin nose, joint hypermotility and history of hip dysplasia. A vascular Ehlers Danlos Syndrome was suspected. However, the genetic study to confirm the diagnosis was not done.

Porfiria cutánea tarda: caso clínico / Porphyria cutanea tarda: case report

Porphyria cutanea tarda (PCT) is the most common type of porphyria: it is characterized by blistering lesions, erosions and crusts on the back of the hands, associated with photosensitivity and facial hypertrichosis. It is produced by acquired or hereditary deficiency of the enzyme UROD, fifth enzyme in the chain of production of the Heme group. This causes accumulation of porphyrins in the liver, which are subsequently mobilized to the skin, where lesions are generated by photosensitivity. This deficiency can be exacerbated by multiple causes. We report a 51-year-old female presenting with the characteristic dermal lesions described above, which disappeared when she discontinued her hormone replacement therapy with estradiol and dydrogesterone. Urinary and blood uroporphyrin and hexacarboxyl porphyrins were elevated and plasma ferritin was 479 ng/ml. Hormone replacement therapy was discontinued and phlebotomies were attempted but not tolerated by the patient. The dermic lesions have not relapsed.

Linfoma NK/T extra nodal, tipo nasal, con compromiso cutáneo primario: reporte de un caso / Extranodal Natural Killer/T-Cell Lymphoma, nasal-type with primary cutaneous involvement: a case report

INTRODUCCIÓN: El linfoma extranodal natural killer/célula T (NK/T) de tipo nasal, es una neoplasia poco frecuente, con una alta letalidad, caracterizada por destrucción ósea alrededor de los senos paranasales, el septum nasal u obstrucción de la vía aérea. Puede presentar compromiso primario de la piel, vía aérea y otros órganos. OBJETIVO: Presentar un caso ilustrativo de una afección poco frecuente y de curso agresivo en población pediátrica, para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los especialistas. CASO CLÍNICO: Adolescente de 14 años, que consultó por lesiones solevantadas en brazos y piernas, no dolorosas, sugerentes de paniculitis subcutánea, las cuales evolucionaron a máculas violáceas ulceradas. La biopsia de las lesiones fue compatible con linfoma NK/T de tipo nasal. Fue derivada a oncología pediátrica, donde recibió tratamiento quimioterápico. Pese a los esfuerzos médicos, la paciente falleció a los 8 meses producto de una infección pulmonar grave secundaria a inmunosupresión. CONCLUSIONES: El linfoma extranodal NK/T, tipo nasal es una neoplasia poco frecuente, que se comporta de forma agresiva, con una alta mortalidad sin tratamiento. Por lo que su reconocimiento es de gran relevancia para el diagnóstico precoz y rápida derivación a Hemato-Oncología.

INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. OBJECTIVE: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.

Entendiendo las terapias actuales en melanoma metastásico / Understanding current therapies in metastatic melanoma

Cutaneous melanoma is a highly aggressive tumor developing from melanocytes, its incidence is increasing, and prognosis in advanced stages is daunting. New therapies have been approved during the recent years with unprecedented results, including inhibitors of MAPK/ERK pathway and immune checkpoint blockade (anti-cytotoxic T lymphocyte antigen-4 (CTLA-4) as ipilimumab, anti-programmed cell death protein 1 (PD-L1) as pembrolizumab and anti-programmed cell death protein 1 ligand (PD-L1), among many others). The aim of this paper is to review currently available metastatic melanoma therapies focusing mainly on new therapies that have demonstrated effectiveness, after several decades of little progress in the treatment of this disease.

Fotoféresis en el tratamiento de Síndrome de Sézary: Caso clínico / Sézary syndrome treated with extracorporeal photopheresis: Report of one case

Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management of SS depends on its stage, patient comorbidities, and treatment availability. Extracorporeal photopheresis (ECP) is the first line of treatment for patients with T-cell lymphomas in stage IVA1, IVA2 or SS. This treatment comprises three phases: leukapheresis, photoactivation and subsequent reinfusion of lymphocytes. As it is an immunomodulatory therapy it does not produce generalized immunosuppression. We report a 76 year-old male with SS stage IIIb initially treated with 12 sessions of ultraviolet phototherapy without response. After 10 well-tolerated sessions of ECP, itching and skin lesions eventually disappeared.

Linfoma anaplástico de células grandes CD30 positivo cutáneo primario: reporte de un caso / Primary cutaneous anaplastic large cell lymphoma: Report of one case

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is within the spectrum of cutaneous CD30-positive lymphoproliferative disorders. It presents as localized or multifocal tumors or plaques and carries an excellent long-term prognosis even in cases with regional and/or ipsilateral lymph node involvement or in cases of recurrent disease. We report a 34 year-old female with a thigh lesion. Skin biopsy confirmed the diagnosis of PCALCL. The patient was strictly monitored but no treatment was instituted and the tumor regressed spontaneously. After 24 months of follow-up the patient remains free of disease without new lesions.

Actualización en diagnóstico y manejo de micosis fungoide y síndrome de Sézary / Update on diagnosis and management of mycosis fungoides and Sézary's syndrome

Los linfomas cutáneos primarios consisten en una proliferación anormal de linfocitos T o B que muestran tropismo por la piel, sin evidenciarse compromiso extra cutáneo al momento del diagnóstico. Se dividen en linfomas de células T (75 por ciento-80 por ciento) y linfomas de células B (20 por ciento-25 por ciento). La micosis fungoide es una neoplasia de estirpe T y constituye el linfoma cutáneo primario más frecuente. Su presentación clínica clásica consiste en 3 etapas: parche, placa y tumor. Sin embargo, tiene múltiples variantes y un amplio diagnóstico diferencial, por lo que para su diagnóstico se requiere una estricta correlación entre la clínica y la histopatología. El síndrome de Sézary, por su parte, es considerado la variante leucémica de los linfomas cutáneos primarios y forma parte del diagnóstico diferencial de las eritrodermias. En esta revisión profundizaremos en los principales aspectos de la clínica, histopatología, criterios diagnósticos y tratamiento de la micosis fungoide y el síndrome de Sézary.

Primary cutaneous lymphomas represent an abnormal proliferation of T or B-cells with skin-homing ability, with no evidence of extra cutaneous disease at the time of diagnosis. They are divided in T-cell lymphomas (75 percent-80 percent) and B-cell lymphomas (20 percent-25percent). Mycosis fungoides (MF) is a T-cell malignancy, being the most common lymphoma. Classic MF presents 3 clinical phases: patch, plaque and tumor stage. However, it has numerous variants and a wide range of differential diagnosis, so that precise clinicopathologic correlation is necessary for make a correct diagnosis. Sézary syndrome is an aggressive leukemic primary cutaneous T-cell lymphoma variant and it is part of the spectrum of erythroderma. In this review we will analyze the main aspects about clinical presentation, histopathology, diagnosis and treatment of mycosis fungoides and Sézary syndrome.

Melanoma dérmico hiperpigmentado de bajo grado de malignidad (melanoma de tipo animal): reporte de cuatro casos / Hyperpigmented dermal melanoma of low-grade of malignancy (animal-type melanoma): report of four cases

El melanoma animal o hiperpigmentado es un subtipo infrecuente de melanoma con células melanocíticas epitelioideas y fusadas muy pigmentadas. Esta entidad sería similar al llamado “melanoma de tipo equino”, una forma de melanoma de bajo grado de malignidad que afecta a los caballos grises. Se reportan cuatro casos, tres hombres y una mujer, cuyas edades variaron de 22 a 84 años; el estudio histopatológico confirmó melanoma dérmico hiperpigmentado; dos casos mostraron ganglio centinela positivo y un caso evolucionó con metástasis múltiples. Tres casos están en control o tratamiento sin evidencias de recidiva o metástasis. El melanoma animal es considerado un subtipo de melanoma de conducta poco agresiva y de mejor pronóstico, pese a su tendencia a las metástasis ganglionares. Los casos presentados mostraron una conducta menos agresiva que la esperada para el espesor de Breslow y estadio clínico en estos casos. Se requieren más estudios para poder identificar variables que permitan predecir el comportamiento biológico y así protocolizar el tratamiento de esta entidad, considerada por algunos como diferente del melanoma.

Animal type or hyperpigmented melanoma is an infrequent subtype of melanoma with heavily pigmented epithelioid and spindle melanocytes. This entity is similar to the so-called “equine-type melanoma”, an indolent variant of melanoma affecting gray horses. We report four cases, three males and one female, whose ages varied from 22 to 84 years; the histopathological study confirmed the diagnosis of hyperpigmented dermal melanoma; two cases showed positive sentinel lymph nodes and one case evolved with multiple metastases. Three cases are in control or under therapy without evidence of recurrences and/or metastases. Animal-type melanoma is considered a subtype of melanoma with indolent behavior and better prognosis, despite its tendence to develop lymph node metastases. The present cases showed a less aggressive behavior than expected for the Breslow’s thickness and clinical stage. More studies are needed to identify variables to predict its behavior and propose therapy protocols for this tumor, considered by some authors a different type of melanoma.

Uso de inmunoglobulina humana endovenosa en pacientes con necrolisis epidérmica tóxica y síndrome de sobreposición Stevens Johnson necrolisis tóxica epidérmica / Use of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis and Stevens-Johnson/toxic epidermal necrolysis overlap syndrome. Review of 15 cases

Background: Toxic epidemial necrolysis (TEN) is an acute adverse drug reaction, that has an unpredictable progression and a 30 percent mortality. The incidence of TEN in the general population is approximately 0.4 to 1.2 cases/million/year. It is characterized pathologically by keratinocyte apoptosis which leads to epidemial detachment. Keratinocyte apoptosis is triggered by activation of the Fas-FasL, pathway and could be prevented by the use of intravenous immunoglobulin (IVIG). Aim: To report the experience with the use of IVIG in TEN. Material and methods: Retrospective study of 15 patients with a diagnosis of Stevens-Johnson/TEN overlap (SJS/TEN) or TEN, that received a total dose of 23 ± 0.6 mg/kg of IVIG over aperiod of 3 to 4 days. The infusion was initiated during the first 24 hours after diagnosis and was associated with standard care for burn victims. Steroids were avoided if the patient was not in chronic steroidal therapy. Results: All patients responded to IVIG in a lapse of 46.4 ± 14.2 hours from the beginning of infusion. Eighty percent of patients survived, but one developed acute renal failure due to IVIG, and another became blind due to corneal opacities, a complication of TEN. Those who survived were discharged after a lapse of 19-8 ± 6.6 days from the beginning of the disease. Conclusions: Despite the lack of blind, multicentric and randomized trials, we agree with some international studies that IVIG is beneficial as a treatment for SSJ/NET and TEN.