ABSTRACT
Introducción: El carcinoma basocelular (CBC)es una de las neoplasias más comunes de la piel. En nuestro país, por su localización, representa una entidad patológica de gran importancia, por la radiación ultravioleta elevada, que es inversamente proporcional a la latitud geográfica en la que nos encontramos en Ecuador. El objetivo del presente trabajo es revisar las características claves que distinguen al Carcinoma basocelular, y actualizar los conocimientos, incluyendo la evidencia disponible en hallazgos histopatológicos, marcadores de inmunohistoquímica y metástasis en esta patología
Introduction: Basal cell carcinoma BCC is one of the most common skin neoplasms. In our country, because of its location, it represents a pathological entity of great importance, due to the high ultraviolet radiation, that is inversely proportional to the geographical latitude we are in Ecuador. This paper objective is to review the key features that distinguish basal cellcarcinoma, and update knowledge, including the available evidence on histopathological findings, immunohistochemical markers and metastasis in this pathology.
Introdução: Carcinoma basocelular O CBC é uma das neoplasias cutâneas mais comuns. Em nosso país, por sua localização, representa uma entidade patológica de grande importância, devido à alta radiação ultravioleta, que é inversamente proporcional à latitude geográfica em que nos encontramos no Equador. O objetivo deste artigo é revisar as principais características que distinguem o carcinoma basocelular e atualizar o conhecimento, incluindo as evidências disponíveis sobre achados histopatológicos, marcadores imunohistoquímicos e metástases nessa patologia.
Subject(s)
Humans , Adult , Immunohistochemistry , Carcinoma, Basal Cell , Skin Neoplasms , Pathology, Molecular , Neoplasm MetastasisABSTRACT
Blastic plasmacytoid dendritic cell neoplasm is a rare hematological malignancy derived from immature plasmacytoid dendritic cells. The tumor cells have an immature blastic appearance, and diagnosis is based on the expression of CD4, CD56 y CD123 in the absence of other lymphoid, natural killer, or myeloid antigens. The majority of affected individuals are older people with a mean age of 66 years. Male to female ratio is approximately 3:1. Common presentation includes cutaneous lesions followed by tumor dissemination. Treatment with conventional chemotherapy is ineffective and allogeneic hematopoietic stem cell transplantation is required to achieve remission. We report three male patients, aged 23, 27 and 51 years with the disease. All had multiple, infiltrated pink plaques and nodules on the skin of their face, neck and thorax, measuring 1 to 12 cm in diameter. All tumors were histologically characterized by a monotonous proliferation of medium size cells with blastic features. Tumor cells were positive for CD123, CD56, CD4 and CD7 in all cases. After a mean of follow-up of 14.6 months, one patient died of the disease, one patient is alive and the disease relapsed after 17 months of remission and one patient is alive with no evidence of the disease.
Subject(s)
Humans , Male , Adult , Middle Aged , Young Adult , Dendritic Cells/pathology , Hematologic Neoplasms/pathology , Skin/pathology , Biopsy , Bone Marrow/pathology , Immunohistochemistry , Fatal OutcomeABSTRACT
La enfermedad de Hailey-Hailey o pénfigo familiar benigno es una enfermedad ampollar rara, con herencia autosómica dominante. Usualmente se inicia en la adolescencia o inicios de la segunda década. Presentamos el caso de una mujer de 40 años que fue referida a nuestro servicio con una historia de cuatro años de erupciones continuas en región axilar y ocasionalmente en zona inguinal, que dejan úlceras dolorosas, y se presentan en forma periódica exacerbándose en épocas de calor. No tuvo fiebre ni malestar previo a la erupción en la piel. Presentaba pápulas vesiculares con base eritematosa y algunas lesiones costrosas en ambas axilas. Respondió adecuadamente a la corticoterapia.
The Hailey-Hailey disease or familial benign chronic pemphigus, is a rare blistering disorder. The onset is usually in the late teens or early twenties. It is inherited in an autosomal dominant manner. A 40 year-old woman with familial benign chronic pemphigus (Hailey-Hailey disease) was referred to our department with a 4 years history of these continuous eruptions occasionally axillary, groin, leaving painful wounds are periodically exacerbated in hot weather. She had no fever and malaise prior to rash. There were based vesicular erythematous papules and somecrusted lesions on both arms. She responded to corticosteroids.
Subject(s)
Humans , Adult , Female , Axilla/pathology , Pemphigus, Benign Familial , Pemphigus, Benign Familial/pathology , Pemphigus, Benign Familial/therapy , Case ReportsABSTRACT
El liquen plano hipertrófico generalizado es una dermatosis subaguda o crónica e inflamatoria, autolimitada de etiología desconocida, que afecta a la piel, mucosas y anexos cutáneos (uñas y pelo). Es una enfermedad relativamente frecuente en adultos, pero extremadamente rara en niños y se presenta en la población general con una prevalencia menor al 1%. La forma infantil es clínicamente similar a la del adulto, aunque la afectación mucosa es excepcional. Se comunica el caso de un niño de 8 años de edad, con lesiones cutáneas generalizadas de liquen plano, con buena evolución y respuesta a tratamiento con corticoides tópicos.
Generalized hypertrophic lichen planus is asubacute or chronic dermatoses, self-limited, inflammatory of unknown etiology that affects the skin, mucous membranes and skin appendages (nails and hair). It is a relatively common disease in adults, but extremely rare in children and occurs in the general population with a prevalence below 1%. The infantile form is clinically similar to adult, although the mucosal involvement is exceptional. We report the case of a child of 08 years of age, with generalized skin lesions of lichen planus, with good outcome and response to treatment with topical corticosteroids.
Subject(s)
Humans , Male , Child , Lichen Planus , Lichen Planus/pathology , Lichen Planus/therapy , Case ReportsABSTRACT
La epidermólisis bulosa distrófica es un trastorno hereditario poco frecuente y clínicamente heterogéneo. Una variante clínica inusual es la epidermolisis bulosa pruriginosa (EBP), que se caracteriza por prurito intenso y lesiones similares a prurigo nodular o liquen simple crónico; y también por la fragilidad de la piel puede conducir a hipertrofia, liquenificación, nódulos y placas. Como en las otras formas de epidermólisis bulosa distrófica, las lesiones se localizan principalmente en extremidades; la patología molecular implica mutaciones en el gen que codifica la proteína fibrilar de anclaje, el colágeno tipo VII (COL7A1). Reportamos el caso de un paciente adulto sin antecedentes patológicos con compromiso cutáneo cuyo tratamiento resultó insatisfactorio, siendo pocos los aportes de la literatura en el manejo exitoso de esta patología.
Dystrophic epidermolysis bullosa is a rare, hereditary, clinically heterogeneous skin disorder. Epidermolysis bullosa pruriginosa is an unusual clinical variant characterized by severe pruritus and simplex lichenoid or nodular prurigo-like lesions; and also by the skin fragility that may lead to hypertrophic, lichenified nodules and plaques. Like other forms of dystrophic epidermolysis bullosa, lesions are located primarily in extremities; molecular pathology involves mutations in the gene encoding the anchoring fibril protein, type VII collagen (COL7A1). We report a case of a previously healthy male adult patient with skin lesions, whose treatment was unsatisfactory, with few contributions from the literature in the successful management of this disease.
Subject(s)
Humans , Male , Adult , Genetic Diseases, Inborn , Epidermolysis Bullosa DystrophicaABSTRACT
Se reporta el caso de un paciente varón de 68 años de edad que presentó tumoración exofítica en planta de pie derecho que al estudio histopatológico fue compatible con melanoma acral amelanótico desmoplásico. El melanoma desmoplásico es una variante histológica de presentación poco frecuente, más aún el tipo amelanótico, y de localización acral, por lo que requiere de un diagnóstico precoz para el tratamiento oportuno, mejorando así el pronóstico del paciente.
We report the case of a male patient aged 68, who presented an exophytic tumor in his right foot plant that, by histopathological study, was compatible with acral amelatonic desmoplastic melanoma. Desmoplastic melanoma is a subtype of rare representation, and even more the amelatonic type of acral location, therefore it requires early diagnosis for prompt treatment and thereby improving patient prognosis.