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1.
Arch. argent. pediatr ; 120(1): e34-e38, feb 2022. tab, ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1353770

RESUMO

Los xantomas cutáneos reflejan el depósito de lípidos en la piel y pueden ser la única manifestación temprana de dislipidemias de inicio en la infancia. Las características y distribución de los xantomas orientan a la patología de base; los xantomas tuberosos tienen una fuerte asociación con la hipercolesterolemia homocigota familiar, una patología muy infrecuente. Su detección temprana otorga una ventana terapéutica para prevenir la ateroesclerosis acelerada y la mortalidad. Se presenta el caso de una paciente que comenzó a los dos años con xantomas tuberosos, que fueron la clave diagnóstica para identificar la hipercolesterolemia homocigota familiar subyacente.


Cutaneous xanthomas reflect lipid deposition on the skin and may be the only early manifestation of a childhoodonset dyslipidemia. Characteristics and distribution of the xanthomas signalize the underlying pathology, tuberousxanthomas being strongly associated with homozygous familial hypercholesterolaemia, an extremely rare condition. Its early detection provides a therapeutic window to prevent accelerated atherosclerosis and mortality. We present the case of a patient who started at two years with tuberous xanthomas, which were the diagnostic clue to identify the underlying homozygous familial hypercholesterolaemia.


Assuntos
Humanos , Feminino , Pré-Escolar , Xantomatose/diagnóstico , Xantomatose/etiologia , Xantomatose/tratamento farmacológico , Dislipidemias , Hipercolesterolemia , Pele , Diagnóstico Precoce
2.
Arch Argent Pediatr ; 120(1): e34-e38, 2022 02.
Artigo em Espanhol | MEDLINE | ID: mdl-35068128

RESUMO

Cutaneous xanthomas reflect lipid deposition on the skin and may be the only early manifestation of a childhoodonset dyslipidemia. Characteristics and distribution of the xanthomas signalize the underlying pathology, tuberous xanthomas being strongly associated with homozygous familial hypercholesterolaemia, an extremely rare condition. Its early detection provides a therapeutic window to prevent accelerated atherosclerosis and mortality. We present the case of a patient who started at two years with tuberous xanthomas, which were the diagnostic clue to identify the underlying homozygous familial hypercholesterolaemia.


Los xantomas cutáneos reflejan el depósito de lípidos en la piel y pueden ser la única manifestación temprana de dislipidemias de inicio en la infancia. Las características y distribución de los xantomas orientan a la patología de base; los xantomas tuberosos tienen una fuerte asociación con la hipercolesterolemia homocigota familiar, una patología muy infrecuente. Su detección temprana otorga una ventana terapéutica para prevenir la ateroesclerosis acelerada y la mortalidad. Se presenta el caso de una paciente que comenzó a los dos años con xantomas tuberosos, que fueron la clave diagnóstica para identificar la hipercolesterolemia homocigota familiar subyacente.


Assuntos
Dislipidemias , Hipercolesterolemia , Xantomatose , Criança , Diagnóstico Precoce , Humanos , Pele , Xantomatose/diagnóstico , Xantomatose/tratamento farmacológico , Xantomatose/etiologia
3.
Arch. argent. pediatr ; 119(3): e247-e251, Junio 2021. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1248216

RESUMO

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado.Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.


Drug reaction with eosinophilia and systemic symptoms is a rare and potentially serious skin adverse reaction, with fever, polymorphous skin rash, facial edema, and/or lymphadenopathy. Reactivation of human herpes virus type 6 has been associated with a more severe and/or prolonged course. A 22-month-old infant under phenobarbital treatment developed erythematous-papular lesions, fever, leukocytosis, elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.


Assuntos
Humanos , Masculino , Lactente , Herpesvirus Humano 6 , Síndrome de Hipersensibilidade a Medicamentos/diagnóstico , Ciclosporina/uso terapêutico , Corticosteroides/uso terapêutico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Eosinofilia , Síndrome de Hipersensibilidade a Medicamentos/complicações , Síndrome de Hipersensibilidade a Medicamentos/terapia
4.
J Ultrasound Med ; 40(2): 407-415, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32725836

RESUMO

Nevus sebaceous of Jadassohn (NSJ) is a cutaneous hamartoma commonly found in the scalp and face and more frequent in children. Clinically, it presents as a yellowish and hairless plaque. In later stages, the NSJ can develop secondary tumors, including skin cancer. We reviewed the ultrasound characteristics of 9 patients (67% female; mean age, 3 years) with NSJ at 18 and 70 MHz. The ultrasound analysis covers the data on the location, thickness, echo structure, and vascularity. The provision of the ultrasound patterns of NSJ can support early diagnosis, avoid unnecessary biopsies, and support monitoring.


Assuntos
Nevo Sebáceo de Jadassohn , Nevo , Neoplasias Cutâneas , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Nevo/diagnóstico por imagem , Couro Cabeludo/diagnóstico por imagem , Neoplasias Cutâneas/diagnóstico por imagem , Ultrassonografia
5.
Rev. méd. Chile ; 148(11)nov. 2020.
Artigo em Espanhol | LILACS | ID: biblio-1389256

RESUMO

Rowell's syndrome is characterized by an association of lupus erythematosus and erythema multiforme, with distinctive laboratory findings. Its treatment is similar to lupus. We report a 16-year-old female presenting with skin lesions, laboratory and histology consistent with this entity. Because of the spread of cutaneous involvement and development of epidermal detachment, she required therapy with intravenous corticosteroids and gamma globulin.


Assuntos
Adolescente , Feminino , Humanos , Lúpus Eritematoso Cutâneo , Eritema Multiforme , Lúpus Eritematoso Sistêmico , Síndrome , Lúpus Eritematoso Cutâneo/diagnóstico , Eritema Multiforme/diagnóstico , Corticosteroides , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico
6.
Medwave ; 20(8): e8021, 2020 Sep 17.
Artigo em Espanhol | MEDLINE | ID: mdl-32956338

RESUMO

A 44-year-old man, previously healthy, consulted for a three-month history of asymmetrical polyarthralgia with inflammatory features involving small and large joints. A few days later, he developed erythema covered by pustules in the nail folds and progressive degeneration of the nail plate of the left thumb, with severe functional limitation. The physical exam showed well-defined erythematous scaly and pustular plaques in the first and third fingers of the left hand, with severe onychodystrophy, which was a key aspect in the diagnosis of acrodermatitis continua of Hallopeau. Other signs of the psoriatic spectrum were observed: erythematous patches of the scrotum, erythematous scaly plaques with hemorrhagic crusts on both knees, and dactylitis. The diagnosis of inverse psoriasis, plaque psoriasis, and pustular psoriasis, respectively, were confirmed by histopathology and, with the CASPAR criteria, psoriatic arthritis. This case is of particular dermatological interest due to the variety of psoriatic manifestations recognized in a single patient and because of the poorly described association between acrodermatitis continua of Hallopeau with psoriatic arthritis.


Un hombre de 44 años, previamente sano, consultó por poliartralgias asimétricas de características inflamatorias de tres meses de evolución, que comprometió pequeñas y grandes articulaciones. Poco tiempo después desarrolló pústulas periungueales sobre una base eritematosa y degeneración progresiva de la lámina ungueal del pulgar izquierdo, limitando severamente su funcionalidad. Destacó al examen físico la presencia de placas eritemato-escamosas y pustulosas bien delimitadas en el primer y tercer dedo de la mano izquierda con onicodistrofia severa, que fue un aspecto clave para establecer el diagnóstico de acrodermatitis continua de Hallopeau. Adicionalmente, se observaron otros hallazgos del espectro psoriático: parches eritematosos en el escroto, placas eritemato-escamosas con costras hemorrágicas en ambas rodillas y dactilitis. Se confirmó histológicamente el diagnóstico de psoriasis inversa, psoriasis en placas y psoriasis pustular, respectivamente y con los criterios de Classification Criteria for Psoriatic Arthritis, CASPAR, el diagnóstico de artritis psoriática.


Assuntos
Acrodermatite/diagnóstico , Artrite Psoriásica/diagnóstico , Psoríase/diagnóstico , Acrodermatite/patologia , Adulto , Artrite Psoriásica/patologia , Humanos , Masculino , Psoríase/patologia
7.
Medwave ; 20(1): e7767, 2020 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-31999679

RESUMO

Elephantiasis nostras verrucosa, a rare manifestation of Kaposis sarcoma, is a progressive cutaneous hypertrophy caused by chronic non-filarial lymphedema secondary to obstruction of the lymphatic system that can lead to severe disfigurement of parts of the body that have gravity-dependent blood flow, due to edema, fibrosis, and hyperkeratosis, especially lower extremities. Among the various conditions that can induce chronic lymphedema are tumors, trauma, radiotherapy, obesity, hypothyroidism, chronic venous stasis, and AIDS-related Kaposis sarcoma. Kaposis sarcoma is a vascular tumor associated with the presence of human gammaherpesvirus 8 that is predominantly cutaneous, locally aggressive, with metastasis, and is associated with the production of factors that favor inflammation, lymphatic obstruction, and lymphedema.


Assuntos
Infecções Oportunistas Relacionadas com a AIDS/complicações , Elefantíase/diagnóstico , Sarcoma de Kaposi/complicações , Infecções Oportunistas Relacionadas com a AIDS/tratamento farmacológico , Infecções Oportunistas Relacionadas com a AIDS/patologia , Alcinos , Fármacos Anti-HIV/uso terapêutico , Benzoxazinas/uso terapêutico , Ciclopropanos , Didanosina/uso terapêutico , Quimioterapia Combinada , Elefantíase/etiologia , Elefantíase/patologia , Humanos , Lamivudina/uso terapêutico , Masculino , Pessoa de Meia-Idade , Sarcoma de Kaposi/tratamento farmacológico , Sarcoma de Kaposi/patologia
8.
Rev Med Chil ; 148(11): 1690-1693, 2020 Nov.
Artigo em Espanhol | MEDLINE | ID: mdl-33844778

RESUMO

Rowell's syndrome is characterized by an association of lupus erythematosus and erythema multiforme, with distinctive laboratory findings. Its treatment is similar to lupus. We report a 16-year-old female presenting with skin lesions, laboratory and histology consistent with this entity. Because of the spread of cutaneous involvement and development of epidermal detachment, she required therapy with intravenous corticosteroids and gamma globulin.


Assuntos
Eritema Multiforme , Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Sistêmico , Adolescente , Corticosteroides , Eritema Multiforme/diagnóstico , Feminino , Humanos , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Síndrome
9.
Medwave ; 20(8): e8021, 2020.
Artigo em Espanhol | LILACS | ID: biblio-1128609

RESUMO

Un hombre de 44 años, previamente sano, consultó por poliartralgias asimétricas de características inflamatorias de tres meses de evolución, que comprometió pequeñas y grandes articulaciones. Poco tiempo después desarrolló pústulas periungueales sobre una base eritematosa y degeneración progresiva de la lámina ungueal del pulgar izquierdo, limitando severamente su funcionalidad. Destacó al examen físico la presencia de placas eritemato-escamosas y pustulosas bien delimitadas en el primer y tercer dedo de la mano izquierda con onicodistrofia severa, que fue un aspecto clave para establecer el diagnóstico de acrodermatitis continua de Hallopeau. Adicionalmente, se observaron otros hallazgos del espectro psoriático: parches eritematosos en el escroto, placas eritemato-escamosas con costras hemorrágicas en ambas rodillas y dactilitis. Se confirmó histológicamente el diagnóstico de psoriasis inversa, psoriasis en placas y psoriasis pustular, respectivamente y con los criterios de Classification Criteria for Psoriatic Arthritis, CASPAR, el diagnóstico de artritis psoriática.


A 44-year-old man, previously healthy, consulted for a three-month history of asymmetrical polyarthralgia with inflammatory features involving small and large joints. A few days later, he developed erythema covered by pustules in the nail folds and progressive degeneration of the nail plate of the left thumb, with severe functional limitation. The physical exam showed well-defined erythematous scaly and pustular plaques in the first and third fingers of the left hand, with severe onychodystrophy, which was a key aspect in the diagnosis of acrodermatitis continua of Hallopeau. Other signs of the psoriatic spectrum were observed: erythematous patches of the scrotum, erythematous scaly plaques with hemorrhagic crusts on both knees, and dactylitis. The diagnosis of inverse psoriasis, plaque psoriasis, and pustular psoriasis, respectively, were confirmed by histopathology and, with the CASPAR criteria, psoriatic arthritis. This case is of particular dermatological interest due to the variety of psoriatic manifestations recognized in a single patient and because of the poorly described association between acrodermatitis continua of Hallopeau with psoriatic arthritis.


Assuntos
Humanos , Masculino , Adulto , Psoríase/diagnóstico , Acrodermatite/diagnóstico , Artrite Psoriásica/diagnóstico , Psoríase/patologia , Acrodermatite/patologia , Artrite Psoriásica/patologia
10.
Int J Womens Dermatol ; 5(3): 183-186, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31360756

RESUMO

Malignant acanthosis nigricans is a rare paraneoplastic syndrome, usually associated with a gastric adenocarcinoma and less frequently with other neoplasms. In general, its appearance indicates a poor prognosis with a survival of < 2 years. We describe the case of a 40-year-old patient who presented with generalized cutaneous thickening that had a velvety appearance, was rapidly progressing, and involved right axillary adenopathy. Skin and nipple biopsy yielded results consistent with acanthosis nigricans, and palpable adenopathy biopsy results were compatible with mammary adenocarcinoma (human epidermal growth factor receptor 2 positive and estrogen and progesterone receptor negative) without a detectable primary tumor. This case of malignant acanthosis nigricans is presented because of the importance of its early recognition as a paraneoplastic syndrome and its relation with mammary adenocarcinoma, an association infrequently reported in the literature.

11.
Arch. argent. pediatr ; 117(3): 305-308, jun. 2019. ilus, tab
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1001208

RESUMO

La úlcera vulvar aguda o úlcera de Lipschütz corresponde a una entidad poco frecuente, por lo general, subdiagnosticada. Se caracteriza por la aparición súbita o aguda de lesiones ulcerosas y dolorosas en la vulva, la vagina y/o el periné, sin antecedente de contacto sexual en niñas y adolescentes. Su etiología permanece desconocida en la mayoría de los casos, aunque se ha asociado a agentes infecciosos. Para su estudio, se deben sospechar infecciones de transmisión sexual, reacciones adversas a medicamentos, enfermedades autoinmunes e inmunosupresión. Las lesiones ulcerosas desaparecen espontáneamente, de manera habitual, sin secuelas ni recurrencias a largo plazo. Se presenta el caso de una paciente adolescente de 11 años, sin inicio de actividad sexual ni menarquia y que consultó por un cuadro prodrómico de cuatro días, seguido del desarrollo agudo de úlceras genitales. Se discuten sus causas, presentación clínica, diagnósticos diferenciales y tratamiento.


Acute vulvar ulcer or ulcer of Lipschütz corresponds to a rare disease, being usually underdiagnosed. It is characterized by a sudden or acute development of ulcerous and painful lesions in the vulva, vagina and/or perineum, without a previous history of sexual contact in girls and teenagers. Its etiology remains unknown in most cases, although they have been associated with infectious agents. Their study includes the suspicion of sexually transmitted infections, adverse drug reactions, autoimmune diseases and immunosuppression. The ulcerative lesions disappear spontaneously, usually without sequelae or recurrences in the long term. We present the case of an 11-year-old adolescent patient, without sexual initiation, or menarche and who consulted due to a four-day prodromal condition, followed by the acute development of genital ulcers. Its causes, clinical presentation, differential diagnosis and treatment are discussed.


Assuntos
Humanos , Feminino , Criança , Adolescente , Úlcera , Doenças Vaginais , Doenças da Vulva , Adolescente
12.
Arch Argent Pediatr ; 117(3): e305-e308, 2019 06 01.
Artigo em Espanhol | MEDLINE | ID: mdl-31063323

RESUMO

Acute vulvar ulcer or ulcer of Lipschütz corresponds to a rare disease, being usually underdiagnosed. It is characterized by a sudden or acute development of ulcerous and painful lesions in the vulva, vagina and/or perineum, without a previous history of sexual contact in girls and teenagers. Its etiology remains unknown in most cases, although they have been associated with infectious agents. Their study includes the suspicion of sexually transmitted infections, adverse drug reactions, autoimmune diseases and immunosuppression. The ulcerative lesions disappear spontaneously, usually without sequelae or recurrences in the long term. We present the case of an 11-year-old adolescent patient, without sexual initiation, or menarche and who consulted due to a four-day prodromal condition, followed by the acute development of genital ulcers. Its causes, clinical presentation, differential diagnosis and treatment are discussed.


La úlcera vulvar aguda o úlcera de Lipschütz corresponde a una entidad poco frecuente, por lo general, subdiagnosticada. Se caracteriza por la aparición súbita o aguda de lesiones ulcerosas y dolorosas en la vulva, la vagina y/o el periné, sin antecedente de contacto sexual en niñas y adolescentes. Su etiología permanece desconocida en la mayoría de los casos, aunque se ha asociado a agentes infecciosos. Para su estudio, se deben sospechar infecciones de transmisión sexual, reacciones adversas a medicamentos, enfermedades autoinmunes e inmunosupresión. Las lesiones ulcerosas desaparecen espontáneamente, de manera habitual, sin secuelas ni recurrencias a largo plazo. Se presenta el caso de una paciente adolescente de 11 años, sin inicio de actividad sexual ni menarquia y que consultó por un cuadro prodrómico de cuatro días, seguido del desarrollo agudo de úlceras genitales. Se discuten sus causas, presentación clínica, diagnósticos diferenciales y tratamiento.


Assuntos
Úlcera/diagnóstico , Doenças da Vulva/diagnóstico , Doença Aguda , Criança , Diagnóstico Diferencial , Feminino , Humanos , Úlcera/patologia , Doenças da Vulva/patologia
14.
Medwave ; 19(11): e7740, 2019 Dec 06.
Artigo em Espanhol | MEDLINE | ID: mdl-31891354

RESUMO

Granuloma faciale is an uncommon benign dermatosis, with unknown etiology, usually asymptomatic, characterized by chronic inflammation localized in sun-exposed areas of the face with a characteristic histological pattern. Although response to treatment is variable, there are multiple therapeutic alternatives that have been reported to be effective in some patients, including systemic treatments with steroids and dapsone or also topical treatments like intralesional corticosteroid, cryotherapy and calcineurin inhibitors, such as tacrolimus. We present the case of an adult patient with an asymptomatic erythematous-violet plaque on the right cheek, with progressive slow growth over two years, clinically and histologically and pathologically compatible with a facial granuloma. The patient responded well to intralesional corticosteroids.


El granuloma facial es una dermatosis benigna poco frecuente de etiología desconocida, generalmente asintomática, caracterizada por inflamación crónica y localizada habitualmente en zonas fotoexpuestas de la cara, con un patrón histológico característico. Si bien la respuesta al tratamiento es variable, existen múltiples opciones terapéuticas que han reportado ser efectivas en algunos pacientes. Entre estas alternativas se incluyen tratamientos sistémicos con corticoides y dapsona o tratamientos intralesionales con corticoides, crioterapia e inhibidores de calcineurina tópicos. Describimos el caso de un paciente adulto con una placa eritemato violácea asintomática en mejilla derecha, de crecimiento lentamente progresivo de dos años de evolución, clínica e histopatológicamente compatible con granuloma facial y respuesta favorable a corticoides intralesionales.


Assuntos
Dermatoses Faciais/diagnóstico , Granuloma/diagnóstico , Dermatopatias Vasculares/diagnóstico , Dermatoses Faciais/tratamento farmacológico , Dermatoses Faciais/patologia , Glucocorticoides/administração & dosagem , Granuloma/tratamento farmacológico , Granuloma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Dermatopatias Vasculares/tratamento farmacológico , Dermatopatias Vasculares/patologia
15.
Rev. chil. dermatol ; 35(1): 14-17, 2019. ilus
Artigo em Espanhol | LILACS | ID: biblio-1103302

RESUMO

La Dermatosis neutrofílica de las manos es consi-derada una variante localizada acral del Síndrome de Sweet, más frecuente en mujeres y principal-mente asociada a enfermedades hematológicas. Las lesiones aparecen como pápulas, vesículas, nó-dulos, placas, úlceras y ampollas, principalmente en el dorso de las manos. Aproximadamente la mi-tad de los pacientes presenta fenómeno de patergia como factor desencadenante.En el presente caso clínico se describe una derma-tosis neutrofílica de las manos posterior a morde-dura de perro, asociado a mielofibrosis primaria y desarrollo de lesiones faciales.


Neutrophilic dermatosis of the hands is conside-red an acral localized variant of Sweet Syndrome, more frequent in women and mainly associated with hematological diseases. The lesions appear as papules, vesicles, nodules, plaques, ulcers, and blisters, mainly on the back of the hands. Appro-ximately half of the patients present a phenome-non of pathergy as a triggering factor. Herein we describe a case of neutrophilic dermatosis of the hands after a dog bite, associated with primary myelofibrosis and development of facial lesions.


Assuntos
Humanos , Animais , Feminino , Idoso , Mordeduras e Picadas/complicações , Cães , Dermatoses Faciais/etiologia , Dermatoses da Mão/etiologia , Síndrome de Sweet/etiologia , Síndrome de Sweet/patologia , Dermatoses Faciais/patologia , Mielofibrose Primária/etiologia , Mielofibrose Primária/patologia , Dermatoses da Mão/patologia
16.
Rev. chil. dermatol ; 35(2): 36-43, 2019. tab, ilus
Artigo em Espanhol | LILACS | ID: biblio-1103479

RESUMO

INTRODUCCIÓN: Las enfermedades acatólicas son un grupo heterogéneo de enfermedades que presentan como característica central histopatológica la acantosis. Generalmente presentan un curso de evolución crónica y recidivante, con variadas manifestaciones clínicas. OBJETIVO: caracterizar los pacientes con diagnóstico de enfermedad acantolítica, bajo 5 criterios clínicos y realizar una revisión de la literatura. MÉTODOS: Se realizó una revisión de la base de datos del Servicio Anatomía Patológica del Hospital San Borja Arriaran (HSBA) entre los años 2007 y 2017 y se complementaron con los antecedentes clínicos extraídos de las fichas clínicas.RESULTADOS: Se obtuvo un total de 13 casos. el 53,8% correspondieron a enfermedad de Darier, 20,6% a enfermedad de Hailey-Hailey y un 20,6% a enfermedad de Grover, obteniendo un promedio de edad al momento del diagnóstico de 22,5 años, 44,3 años y 47,6 años respectivamente. Los antecedentes familiares estuvieron presentes en el 53,8% del total de pacientes, ninguno de ellos presentaba estudio genético. El 61,5% de la muestra correspondió a pacientes de sexo femenino y el promedio de años de evolución previo al diagnóstico fue de 7,4 años para Darier, 8,6 para Hailey-Hailey y para Grover. El 100% de los pacientes con enfermedad de Darier y Grover estaban con terapia sistémica y el 66,6% de enfermedad de Hailey-Hailey con terapia tópica, todos con adecuada respuesta clínica. DISCUSIÓN y conclusiones: las enfermedades acantolíticas corresponden a genodermatosis poco frecuente cuyo diagnóstico y tratamiento constitu-yen un desafío para el dermatólogo


INTRODUCTION: Atytolic diseases are a heterogeneous group of diseases that present acanthosis as a histopathological central characteristic. They usually present a course of chronic and recurrent evolution, with varied clinical manifestations. OBJECTIVE: to characterize the patients diagnosed with acantholytic disease, with 5 clinical criteria and to carry out a review of the literature. METHODS: A review of the database of the Pathological Anatomy Service of the San Bor-ja Arriaran Hospital (HSBA) between 2007 and 2017 was carried out and complemented with the clinical records extracted from the clinical files. RESULTS: A total of 13 cases were obtained. 53.8% corresponded to Darier's disease, 20.6% to Hailey-Hailey's disease and 20.6% to Grover's disease, obtaining an average age at diagnosis of 22.5 years, 44.3 years and 47.6 years respectively. Family history was present in 53.8% of the to-tal patients, none of them had a genetic study. 61.5% of the sample corresponded to female patients and the average of years of evolution prior to diagnosis was 7.4 years for Darier, 8.6 for Hailey-Hailey and for Grover. 100% of the pa-tients with Darier and Grover's disease were on systemic therapy and 66.6% of Hailey-Hailey's disease with topical therapy, all with adequate clinical response. DISCUSSION AND CONCLUSIONS: acantholytic diseases correspond to rare genodermatosis whose diagnosis and treatment constitute a challenge for the dermatologist.


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Acantólise/epidemiologia , Pênfigo Familiar Benigno/epidemiologia , Ictiose/epidemiologia , Doença de Darier/epidemiologia , Chile , Estudos Retrospectivos , Acantólise/diagnóstico , Pênfigo Familiar Benigno/diagnóstico , Ictiose/diagnóstico , Doença de Darier/diagnóstico
17.
Rev. méd. Chile ; 147(1): 114-118, 2019. graf
Artigo em Espanhol | LILACS | ID: biblio-991381

RESUMO

Drug rash with eosinophilia and systemic symptoms or DRESS Syndrome is a rare, serious and potentially fatal adverse drug reaction. It is characterized by widespread morbilliform and edematous skin lesions, associated with eosinophilia, lymphadenopathy and internal organ involvement and unusually associated with pulmonary symptoms. We report a 47-year-old male with DRESS syndrome, manifested with typical skin lesions and extensive pulmonary involvement, responding satisfactorily to systemic corticosteroids.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Doenças Pulmonares Intersticiais/induzido quimicamente , Doenças Pulmonares Intersticiais/patologia , Síndrome de Hipersensibilidade a Medicamentos/patologia , Penicilina G Benzatina/efeitos adversos , Dipirona/efeitos adversos , Resultado do Tratamento , Corticosteroides/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Antipiréticos/efeitos adversos , Síndrome de Hipersensibilidade a Medicamentos/tratamento farmacológico , Antibacterianos/efeitos adversos
19.
Rev. chil. infectol ; 34(6): 603-606, dic. 2017. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-899766

RESUMO

Resumen La infección por VIH puede presentarse con distintas manifestaciones cutáneas, que en algunas ocasiones son consideradas marcadores de infección. Una posible manifestación es la eritrodermia psoriática, que corresponde a una forma generalizada de psoriasis. Presentamos un caso clínico de un hombre joven en que se sospechó una infección por VIH por un cuadro de eritrodermia psoriática confirmada por biopsia, asociado a un sarcoma de Kaposi. Posteriormente, la infección por VIH fue confirmada por serología. Se manejó con terapia antirretroviral, con buena respuesta al mes de tratamiento. La eritrodermia psoriática se puede considerar un marcador cutáneo de infección por VIH cuando ocurre en pacientes previamente sanos o con psoriasis recalcitrante.


HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis.


Assuntos
Humanos , Masculino , Adulto , Psoríase/virologia , Infecções por HIV/complicações , Dermatite Esfoliativa/virologia , Psoríase/patologia , Pele/patologia , Biópsia , Infecções por HIV/patologia , Infecções por HIV/tratamento farmacológico , Dermatite Esfoliativa/patologia , Antirretrovirais
20.
Rev Chilena Infectol ; 34(6): 603-606, 2017 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-29488557

RESUMO

HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis.


Assuntos
Dermatite Esfoliativa/virologia , Infecções por HIV/complicações , Psoríase/virologia , Adulto , Antirretrovirais , Biópsia , Dermatite Esfoliativa/patologia , Infecções por HIV/tratamento farmacológico , Infecções por HIV/patologia , Humanos , Masculino , Psoríase/patologia , Pele/patologia
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