Anal involvement in patient with pemphigus vulgaris: Case report.

INTRODUCTION AND IMPORTANCE: Pemphigus Vulgaris (PV) is a low incidence autoimmune mucocutaneous bullous disease, in which pathogenic antibodies are directed against the keratinocyte cell surface. Although anal involvement is unusual, correlations between its appearance and disease severity have been recently reported. CASE PRESENTATION: A 42-year-old male presented with an anal fissure and a year of recurrent oral and pharyngeal aphtha. A chemical sphincterotomy with botulinum toxin plus fissurectomy was performed. 3 years later an upper eyelid injury raised suspicion of pemphigus vulgaris (PV), tested negative for specific antibodies. A couple years later, an anal fissure, and suspected Crohn's disease, led to a second intervention. Surgical analysis unveiled characteristics indicative of a vegetating variant of PV. CLINICAL DISCUSSION: Anal involvement in PV varies in prevalence, with studies reporting rates between 2 % and 27.98 %. This manifestation often accompanies late disease stages and may indicate severity, notably in association with oral lesions. Patients might not readily discuss anal symptoms, leading to potential underreporting. Anal PV is linked to other lesions (ocular, nasal, genital) and the severity of oral PV. Even when other sites are in remission, anal recurrence can occur. Early recognition and follow-up are crucial, highlighting the need for routine anal examination and confirmatory diagnostics. CONCLUSION: PV lesions can present on the skin or mucosa, although oral lesions are the most common site of initial presentation. Anal PV is a diagnostic challenge, and this case illustrates the importance of a careful evaluation.

Cutaneous myiasis in skin cancer and malignant wounds: a systematic review.

BACKGROUND: Cutaneous myiasis in patients with malignant wounds or skin cancer is a rare and undesirable event with limited epidemiological data. A subregister of reports, lack of education in the population, inadequate empirical treatments, and medical underestimation are components of a public health problem that threatens patients' lives. METHODS: We conducted a systematic review of the literature of cutaneous myiasis associated with malignant wounds and skin cancer, characterizing sociodemographic variables, risk factors, clinical and histological features, and treatment. Additionally, we present a demonstrative case with the adequate taxonomic evaluation. DISCUSSION: Cutaneous myiasis is an underestimated and poorly managed infestation, which can generate severe complications in oncological patients. This is the first systematic review in the literature about this clinical scenario, which provides information to the physician and clinical researcher about the epidemiological gaps and what has been published so far. CONCLUSIONS: Findings from the current review have helped to display the sociodemographic, epidemiological, and clinical behavior of myiasis in skin cancer and malignant wounds. Its contribution to the greater tumor tissue destruction is clear; however, more studies are required. The therapeutic management in these patients is equally clarified.

What's Eating You? Black Butterfly (Hylesia nigricans).

Lepidopterism refers to the adverse medical effects of contact with insects of the order Lepidoptera, which includes both moths and butterflies. These effects typically result from contact with an insect during the caterpillar (larval) stage. Lepidopterism involves multiple pathologic mechanisms, including direct toxicity of venom and mechanical irritant effects. Clinical patterns associated with contact with lepidoptera include localized stinging reactions, papular urticaria and dermatitis, urticarial wheals, and hemorrhagic diathesis. Accurate diagnosis of symptoms associated with contact with caterpillars, butterflies, or moths is nearly impossible without a reliable history of exposure, as the histology of exposure is nonspecific. Treatment is largely empiric and should be based on symptoms. We report a case of lepidopterism in a patient with acute cutaneous lesions following exposure to an adult-stage black butterfly (Hylesia nigricans).

Angiokeratomas, not everything is Fabry disease.

INTRODUCTION: Angiokeratoma corporis diffusum are benign capillary malformations typically associated with Fabry disease and other lysosomal storage disorders. Only in a few cases they appear in healthy individuals. METHODS AND CASE: We carried out an exhaustive review of the literature on angiokeratomas and their main clinical, dermoscopy and histological features. Additionally, we reviewed the cases of healthy subjects illustrating the limitations of each case and comparing these results with our case. DISCUSSION: Angiokeratoma corporis diffusum is mostly related to Fabry disease and other lysosomal storage disorders. However, some cases may occur in apparently healthy individuals. Therefore, there is a increasing interest in its etiology, pathogenesis and clinical evaluation. CONCLUSION: This is an academic-clinical review on angiokeratomas and their main implications in daily dermatological practice. Additionally, we report the first case in the literature of angiokeratoma corporis diffusum in a healthy patient with up-to-date laboratory methods currently available. The clinician should remember that not all angiokeratoma corporis diffusum occurs with lysosomal storage disorders.

Extramammary Paget's disease.

The purpose of this report is to describe an unusual case of extramammary Paget's disease with urethral and lymph node infiltration and demonstrate the role of MRI in the pre-operative period for the assessment, management and prognosis of the disease. Although skin wrinkles on MRI may be misinterpreted based on observer's experience, it correlates well with pathology and may provide an accurate assessment before interventional therapy.

Biopsia por congelación: recomendaciones en la práctica clínica y dermatológica / Frozen biopsy: Recommendations in clinical and dermatology practice

Los avances en histotecnología han permitido a lo largo de los años el desarrollo de diferentes técnicas diagnósticas en la práctica de la patología. Entre estas técnicas se encuentra la biopsia por congelación, sin embargo, en el ámbito clínico es común el desconocimiento de esta herramienta diagnóstica. Presentamos una breve revisión acerca de sus limitaciones, sus posibles indicaciones y los casos en los que la biopsia por congelación brinda información importante al clínico para orientar una modificación terapéutica. Al final se hace énfasis sobre las consideraciones a tener en cuenta sobre la biopsia por congelación de piel y las condiciones clínicas en las cuales resulta útil.

The advances in histotechnology over the years have enabled the development of different diagnostic techniques in the pathology practice. These techniques include frozen biopsy; however, in the clinical field use of this diagnostic tool generally unknown. A brief review of their limitations are presented, along with their possible indications, as well as cases in which frozen biopsy provides important information to the clinician to guide therapeutic changes. Finally, emphasis is placed on the considerations to take into account in frozen skin biopsy, and the clinical conditions in which it is useful. © 2013 Instituto Nacional de Cancerología. Published by Elsevier España, S.L.U. All rights reserved.

Tumor pilar proliferante maligno en cuero cabelludo / Malignant proliferating pilar tumor of the scalp

El tumor pilar proliferante maligno es una neoplasia rara que puede confundirse con un carcinoma escamocelular y está caracterizado a la clínica por localizarse en áreas diferentes al cuero cabelludo, crecimiento rápido e infi ltrativo, un diámetro mayor a 5 centímetros y, en algunas instancias, reportes de metástasis. Presentamos el caso de una paciente de sexo femenino, con diagnóstico de tumor pilar proliferante maligno localizado en el cuero cabelludo.

The malignant proliferating pilar tumor is a rare neoplasm that may be confused with squamous cell carcinoma, and is characterized by the clinical location in areas other than the scalp, rapid and infiltrative growth, a diameter greater than 5 centimeters, and in some instances, reports of metastasis. We report the case of a female patient, diagnosed with malignant proliferating pilar tumor, located in the scalp.

Hiperplasia angiolinfoide en espécimen de expansión tisular: Reporte de caso / Angiolymphoid hyperplasia in a tissue expansion specimen: A case report

La expansión tisular es una técnica que aprovecha las propiedades viscoelásticas de la piel para distenderla mediante infiltraciones seriadas a través de una válvula de un dispositivo de silicona. dicho tejido se utiliza posteriormente para reconstrucción y para cubrir defectos. La hiperplasia angiolinfoide con eosinofilia es una enfermedad vasoproliferativa que se manifiesta clínicamente por tumores nodulares. se presenta el caso clínico de un paciente con expansión tisular, y en quien se encuentra de forma incidental, en la histopatología de la cápsula, hiperplasia angiolinfoide con eosinofilia. para el conocimiento del grupo a cargo del presente trabajo, este es el primer caso en el cual ambas entidades se relacionan. © 2012 Instituto Nacional de Cancerología. publicado por elsevier españa, s.L. todos los derechos reservados.

Tissue expansion is a technique that takes advantage of the viscoelastic properties of the skin by distending it using a series of injections through a valve of a silicone device. this tissue is subsequently used for repair and to cover defects. angiolymphoid hyperplasia with eosinophilia is a vasoproliferative disease that manifests clinically as nodular tumors. the clinical case is presented of a patient with tissue expansion in whom angiolymphoid hyperplasia with eosinophilia was an incidental finding in the histopathology of the capsule. To the knowledge of the group presenting this work, this is the first in which both conditions are linked.

Adenocarcinoma apocrino en nevus sebáceo de Jadassohn / Apocrine adenocarcinoma arising on a nevus sebaceus of Jadassohn

El nevus sebáceo de Jadassohn es una lesión congénita sobre la cual se pueden desarrollar de manera infrecuente, diversos tumores, en su mayoría benignos. Se presenta el caso de una mujer de 63 años con un nevus sebáceo sobre el cual se desarrolló un adenocarcinoma apocrino primario.

Melanoma en niños: reporte de caso / Melanoma in children: case report

Aunque el melanoma cutáneo es una neoplasia infrecuente entre la población pediátrica, están definidos los factores de riesgo para su desarrollo. El clínico debe pensar en esta entidad al evaluar una lesión pigmentada en niños. Se presenta el caso de un paciente de 14 años y la correspondiente revisión de la literatura.

Melanoma conjuntival en niños / Conjuntival melanoma in children

Las lesiones melanocíticas de la conjuntiva abarcan un espectro que comprende desde nevus benignos y atípicos hasta el melanoma maligno. El melanoma conjuntival es un tumor poco común de personas adultas, con algunos casos reportados en niños. Las lesiones conjuntivales pigmentadas en niños son predominantemente nevus, y en raras ocasiones pueden sufrir transformación maligna. Se describirá el caso de una niña de 8 años con melanoma conjuntival, quien presentó antecedente de lesión pigmentada en la conjuntiva bulbar del ojo izquierdo desde los 3 años de edad, y la cual creció de forma progresiva.

Melanocytic lesions of the conjunctiva range from benign and atypical nevus to malignant melanoma. Conjuntival melanoma is an uncommon tumor among adults, and few cases have been reported in children. Pigmented conjuntival lesions in children are predominantly nevus, and on rare occasion can become malignant. A case is described of an 8-year old girl with conjuntival melanoma with antecedent of pigmented lesion in the bulbous conjunctiva of the left eye from age 3 which underwent progressive growth.

Carcinoma seudolinfoepitelioma cutáneo / Lymphoepithelioma-like carcinoma

El carcinoma seudolinfoepitelioma cutáneo es un tumor maligno poco frecuente que se caracteriza por ser histológicamente similar a los tumores linfoepiteliales de la nasofaringe. Se presenta el caso de un paciente de sexo masculino de 80 años de edad, que presentó este tipo de tumor en el hombro derecho y se destaca la importancia del estudio histológico e inmunohistoquímico para llegar al diagnóstico definitivo.

Calcifying Lupus panniculitis in a patient without manifestations of systemic lupus Erythematosus / Paniculitis Lúpica calcificante en un paciente sin manifestaciones de lupus eritematoso sistémico

Lupus panniculitis or lupus profundus is a variant of lupus Erythematosus cutaneous that primarily affects subcutaneous tissue. Clinically, it is characterized by one or several firm subcutaneous nodules and/or plaques with or without overlying epidermal changes. It is reported to occur with a frequency of 2-3% in patients with Systemic Lupus Erythematosus (SLE). Between 10 and 50 percent of patients with lupus panniculitis will have or eventually develop Systemic Lupus Erythematosus. In nearly all cases there are deep, erythematosus plaques and nodules, and some of them ulcers, which usually involve the proximal extremities, trunk, breasts, buttocks, and face. These lesions may be tender and painful and frequently heal with atrophy and scaring, turning as a chronic condition and subsequently heal with disfigurement. We describe a patient who suffers from lupus panniculitis with no association to SLE symptoms and complicated by several progressive and disabling cutaneous lesions.

La paniculitis lúpica o también llamada lupus profundus es una variante del lupus eritematoso cutáneo que afecta el tejido celular subcutáneo. Se caracteriza clínicamente por uno o varios nódulos subcutáneos que son firmes y/o placa con o sin cambios epidérmicos. Se ha informado su frecuencia en 2% a 3% de casos de Lupus eritematoso sistémico. Entre el 10 al 50% de los casos de paniculitis lúpica va a desarrollar lupus eritematoso sistémico. En casi todos los casos hay placas eritematosas y/o nódulos que en algunos casos se ulceran y que usualmente están localizados en las áreas proximales de las extremidades, tronco, mamas, nalgas y la cara. Estas lesiones pueden ser clínicamente dolorosas y sensibles a la presión y frecuentemente cicatrizan con desfiguración del área circundante. Describimos un paciente que padece de paniculitis lúpica sin asociación de lupus eritematoso sistémico y que se complicó con varias lesiones cutáneas progresivas y discapacitantes.

Neoplasia blástica de células dendríticas plasmocitoides: reporte de un caso / Blastic plasmacytoid dendritic cell neoplasm, a case report

La neoplasia blástica de células dendríticas plasmocitoides es una rara entidad, y la afectación cutánea es la más frecuente. Clínicamente, se caracteriza por pápulas o placas ertiemato-violáceas infiltradas, en cara y parte superior del tronco. Presentamos el caso de un paciente de 61 años con esta entidad y la revisión de la literatura.

Dermatofibrosarcoma protuberans pigmentado (tumor de Bednar) / Pigmented dermatofibrosarcoma protuberans (Bednar tumor)

El dermatofibrosarcoma protuberans es un tumor cutáneo agresivo localmente. Además del tipo convencional, se han descrito múltiples variantes morfológicas, y el tumor de Bednar es una de ellas. Reportamos aquí el caso de un dermatofibrosarcoma protuberans pigmentado, un patrón distintivo. El tumor se presentó en una mujer de 23 años, que consultó por una lesión en el área supraescapular izquierda, de 10 años de evolución.

Manifestaciones cutáneas de la infección por citomegalovirus en un paciente con compromiso inmunitario / Cutaneous manifestations of cytomegalovirus infection in an immunocompromised patient

El citomegalovirus es un patógeno oportunista que frecuentemente afecta pacientes con compromiso inmunitario. Diferentes órganos pueden resultar afectados, y las lesiones en piel son raras e inespecíficas. Se presenta el caso de un hombre de 70 años con diagnóstico de leucemia linfocítica crónica e infección por citomegalovirus con manifestaciones cutáneas.

Melanoma maligno patrón epitelioide: melanoma animal / Malignant epithelioid melanoma: animal type melanoma

El melanoma de tipo animal es una neoplasia cutánea rara. Estas lesiones se caracterizan por una pigmentación intensa, parda oscura, negra o negra azulosa; su apariencia es nodular pero, también, puede aparecer como parche. Puede aparecer en cualquier parte del cuerpo, incluidos los genitales y las mucosas, como el caso que presentamos a continuación de un paciente de 41 años con este diagnóstico.