Atrofia branca de Milian / Atrofie blanche

A vasculopatia livedoide é uma doença cutâneo-vascular que surge devido à oclusão trombogênica de vasos da derme. Apresenta-se por meio de máculas ou pápulas eritematosas e purpúricas, em membros inferiores, que podem levar à ulceração dolorosa crônica e recorrente. Com a evolução, pode haver cicatrização, o que leva ao aparecimento de áreas de fibrose e cicatrizes atróficas, irregulares e esbranquiçadas, dando nome à patologia. Relata-se o caso de uma paciente com vasculopatia livedoide de acometimento de membros inferiores. (AU)

Livedoid vasculopathy is a vascular skin disease resulting from thrombogenic occlusion of dermal blood vessels. It presents with erythematous, purpuric macules or papules on the lower limbs, which can lead to chronic and recurrent painful ulceration. With its progression, there may be scarring leading to the appearance of areas of fibrosis and atrophic, irregular, and whitish scars, which gave this name to the pathology. The case of a patient with livedoid vasculopathy affecting the lower limbs is reported. (AU)

Reporte de un caso: eritema discrómico perstans en paciente con diagnóstico de virus de inmunodeficiencia humana / Case report: dyschromic erythema perstans in a patient diagnosed with human immunodeficiency virus

El eritema discrómico perstans o dermatitis cenicienta, es un trastorno pigmentario de la piel poco común, de etiología desconocida. Se describe el caso de un adulto de 35 años con antecedente de VIH, quien consulta por aparición de lesiones irregulares, bien definidas café-grises localizadas en cuello, área mandibular inferior, espalda y brazos, de borde levemente eritematoso. El diagnóstico de eritema discrómico perstans se realizó con base en los hallazgos clínicos e histopatológicos.

Erythema dyschromicum perstans also known as ashy dermatosis is a rare skin pigmentary disorder of unknown etiology. We describe the case of a 35-year-old man HIV positive who presented irregular, well defined brown-gray lesions with slightly erythematous border located in neck, lower jaw, back and arms. Diagnosis of erythema dyschromicum perstans in this patient was made based on clinical and histopathological criteria.

Eritema gyratum repens idiopático: presentación de un caso en una mujer joven / Idiopathic erythema gyratum repens: presentation of a case in a young woman

El eritema gyratum repens es una dermatosis muy infrecuente incluida dentro del abanico diagnóstico de las lesiones figuradas. Aparece sobre todo en varones entre la sexta y séptima décadas de vida. Clásicamente se ha relacionado con neoplasias, sobre todo de pulmón, esófago y mama. Sin embargo, en los últimos años se ha visto que aproximadamente el 30% de los casos no son paraneoplásicos, pudiendo deberse a fármacos, enfermedades reumáticas, manifestaciones atípicas de procesos cutáneos, e incluso ser idiopáticos, por lo que es importante realizar un adecuado proceso diagnóstico para filiar la etiología, y considerar un amplio diagnóstico diferencial. Presentamos el caso de una mujer de 40 años con lesiones eritematosas anulares concéntricas sugestivas de eritema gyratum repens, del tipo idiopático tras el correspondiente estudio diagnóstico.

Erythema gyratum repens is an unusual dermatosis included within the group of figurate lesions. It usually appears in men at the age of sixty or seventy. Classically it has been related to neoplasms, especially of the lung, esophagus and breast, however recently it has been seen that 30% of cases are not paraneoplastic approximately, and might be caused by drugs, rheumatologic diseases, atypical presentation of common skin diseases, and also it could be idiopathic, so it is important to carry out an adequate diagnostic process to determine the etiology, and to consider a broad differential diagnosis. We present the case of a 40-year-old woman with concentric annular erythematous lesions suggestive of idiopathic erythema gyratum repens after the diagnostic process

Trichoscopy findings in dissecting cellulitis

Abstract Dissecting cellulitis is an inflammatory, chronic, and recurrent disease of the hair follicles that mainly affects young Afro-descendent men. Trichoscopy is a method of great diagnostic value for disorders of the scalp. Clinical and trichoscopic findings of dissecting cellulitis are heterogeneous and may present features common to non-cicatricial and scarring alopecia. This article presents the trichoscopic findings of dissecting cellulitis that help in the diagnosis and consequent institution of the appropriate therapy and better prognosis of the disease.

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Trichothiodystrophy: a case report of childhood glaucoma associated with non-acquired systemic disease / Tricotiodistrofia: relato de caso de glaucoma infantil associado a doença sistémica não adquirida

ABSTRACT Trichothiodystrophy belongs to a group of rare genetic diseases characterized by DNA repair anomalies. Ocular manifestations can occur in 50% of cases, including cataract, refractive errors, strabismus, microcornea, microphthalmia, dry eye, and pigmentary macular changes. We report a case of childhood glaucoma in a patient with trichothiodystrophy who underwent trabeculectomy in the left eye. To our knowledge, this is the first clinical report of childhood glaucoma associated with trichothiodystrophy.

RESUMO A tricotiodistrofia pertence a um grupo de doenças genéticas raras caracterizadas por anomalias da reparação do DNA. Manifestações oculares podem ocorrer em 50% dos casos, incluindo catarata, erros refrativos, estrabismo, microcórnea, microftalmia, olho seco e alterações maculares pigmentares. Relatamos um caso de glaucoma infantil em um paciente com tricotiodistrofia submetido à trabeculectomia no olho esquerdo. No nosso conhecimento, este é o primeiro caso descrito de glaucoma infantil associado à tricotiodistrofia.

Llega la primavera y florecen los eritemas polimorfos solares / Spring arrives and with it the polymorphous light eruption

El eritema polimorfo solar es la fotodermatosis más frecuente y suele aparecer en primavera con la primera exposición intensa al sol. Sus manifestaciones cutáneas son variadas y el diagnóstico se basa en la clínica junto al antecedente de exposición solar. En los casos leves, la fotoprotección suele ser suficiente para el control de la enfermedad, pero en formas más graves se requieren otras terapéuticas, como corticoides, antihistamínicos, o fototerapia, que genera una "fotoadaptación" de las áreas de piel afectadas. Presentamos un caso típico de erupción polimorfa solar que respondió de forma adecuada a medidas de fotoprotección. (AU)

The polymorphic solar eruption is the most frequent photodermatosis, and usually appears in spring with the first intense exposure to the sun. It has multiple cutaneous manifestations, and its diagnosis is based on the clinic and the antecedent of solar exposition. In mild cases, photoprotection is usually enough to control the disease, but in more severe forms, other therapies are required, such as corticosteroids, antihistamines, or phototherapy to generate a "photo-adaptation" of the affected skin areas. We present a typical case of polymorphic solar eruption that responded adequately to photoprotection measurements. (AU)

Erythema dyschromicum perstans in a child following an enteroviral meningitis

ABSTRACT A healthy 6-year-old boy presented with an erythematous macular exanthema, meningeal signs and fever, initially diagnosed with probable bacterial meningitis and treated with antibiotic and anti-inflammatory drugs. Enteroviral meningitis was confirmed, but the skin lesions continued to evolve and the patient was ultimately diagnosed with erythema dyschromicum perstans. The boy was followed during three years until the spontaneous resolution of the dermatosis.

Eritema flagelado por ingesta de hongos Shiitake: reporte de caso / Flagellated erythema due to Shiitake mushroom intake: report of case

El eritema flagelado es una erupción cutánea poco frecuente con múltiples causas, dentro de las cuales se encuentra la ingesta de hongos shiitake crudos o semicrudos. Se postula que es secundario a una reacción de hipersensibilidad Th-1 producida por el lentinan, un polisacárido termolábil de la pared celular del hongo. En años recientes ha aumentado su incidencia en el mundo occidental debido a la creciente popularidad de la comida asiática, en la que los hongos shiitake son un ingrediente muy utilizado. El cuadro clínico se caracteriza por la aparición de lesiones eritematosas lineales pruriginosas principalmente en tronco y extremidades, de curso autolimitado. El diagnóstico es principalmente clínico, basado en el antecedente de ingesta reciente de hongos shiitake y el rash característico y el tratamiento es sintomático con antihistamínicos y corticoides tópicos u orales. A continuación, presentamos un caso de eritema flagelado en una mujer joven, secundario a ingesta de hongos shiitake, que se resolvió completamente en 2 semanas.

Flagellate erythema is a rare skin eruption with many causes, including the ingestion of raw or undercooked shiitake mushrooms. It is thought to be a Th-1 hypersensitivity reaction produced by lentinan, a thermolabile polysaccharide found in the cell wall of the mushroom. In recent years, there has been an increase in the number of cases due to the growing popularity of Asian cuisine, in which shiitake mushrooms are a central ingredient. The clinical presentation is characterized by the appearance of linear, erythematous, pruritic lesions mostly on the trunk and extremities, of self-limited course. Diagnosis is mainly clinical, based on the history of recent shiitake mushroom ingestion and the characteristic rash, and treatment is symptomatic, with antihistamines and topical or systemic steroids. In this report, we present a case of flagellate erythema in a young woman, that appeared after the ingestion of shiitake mushrooms, and resolved completely after 2 weeks.

Syndrome in question

Abstract: Dermatomyositis is a rare inflammatory disease, autoimmune, with proximal myopathy associated with characteristic dermatological manifestations. In adults, 20-50% of the cases are paraneoplastic manifestation, being mandatory the workup for malignancy Herein we report a case of a woman with classic dermatological presentation of dermatomyositis and newly diagnosed breast cancer. In general, the clinical presentation of paraneoplastic dermatomyositis is more exuberant and manifestations may precede, concur or succeed the diagnosis of neoplasia. The prognosis of cases associated with breast cancer is worse than the idiopathic form. Treatment is based mainly on the resolution of the underlying disease, beyond immunosuppressants.

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Abstract Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

Dermatosis cenicienta: revisión y diagnóstico diferencial con otros trastornos pigmentarios idiopáticos / Ashy dermatosis: revision and differential diagnosis with other idiopathic pigmentary disorders

La dermatosis cenicienta es un trastorno pigmentario infrecuente que se presenta en individuos de piel morena, especialmente en centroamericanos. Las lesiones consisten en manchas hiperpigmentadas de coloración gris-azulada, principalmente localizadas en la cara, tronco y brazos. Su principal diagnóstico diferencial debe ser planteado con tres dermatosis pigmentarias de origen idiopático: eritema discrómico perstans, pigmentación macular eruptiva idiopática y liquen plano pigmentoso. Hay consenso en considerar al eritema discrómico perstans como la misma enfermedad, pero con un borde eritematoso transitorio. La pigmentación macular eruptiva idiopática es clínicamente similar, pero sin una dermatitis de interfase al estudio histopatológico, y sólo muestra una pigmentación de la capa basal, incontinencia pigmentaria y melanófagos en la dermis. Finalmente, el liquen plano pigmentoso debe ser considerado como una variedad pigmentada de liquen plano.

Ashy dermatosis is an infrequent disorder of pigmentation, which predominantly occurs in darkly pigmented individuals, specially in central americans. The lesions consist of bluish-gray hyperpigmented patches mainly located on the face, trunk and arms. Its main differential diagnosis must be raised with three idiopathic pigmentary disorders: erythema dyschromicum perstans, idiopathic eruptive macular pigmentation and lichen planus pigmentosus. It has been widely accepted that erythema dischromicum perstans is the same disease, but with a transient erythematous border. Idiopathic eruptive macular pigmentation is clinically similar but without an interfase dermatitis at histopathologic studies, only showing increased pigmentation of the basal layer and pigmentary incontinence and many melanophages in the dermis. Finally, lichen planus pigmentosus must be considered as a pigmented variant of lichen planus.

Enfermedad de Morbihan: exitoso tratamiento con isotretinoína oral / Morbihan disease: successful treatment with oral isotretinoin

Se presenta a un paciente de 18 años de edad, con antecedentes de hipotiroidismo y acné leve. Consulta por edema facial persistente, a predominio de la mitad superior del rostro, de 7 meses de evolución. El mismo es indoloro, con un leve eritema, principalmente en párpados, y con temperatura local conservada. Se le solicitan estudios complementarios y se lo trata con antihistamínicos sin mejoría. Se toma biopsia de piel y con el diagnóstico de enfermedad de Morbihan se inicia tratamiento con isotretinoína a dosis de 20 mg/día durante seis meses, con respuesta muy favorable (AU)

An 18-year-old male patient with hypothyroidism and mild acne is reported. He consults for a 7-month history of persistent facial edema, predominantly on the upper half of the face. Edema is painless, with eyelids mild erythema and local temperature preserved. Additional tests are requested and treatment with antihistamines is unsuccessful. Skin biopsy is undertaken with Morbihan disease diagnosis. Patient is treated with oral isotretinoin in a daily dose of 20 mg/day for six months, with significant clinical improvement (AU)

Eritema discromicum perstans (EDP) o dermatosis cenicienta: revisión a propósito de dos casos / Erythema discromicum perstans (EDP) or cystic dermatosis: review of two cases

El eritema discromicum perstans (EDP) o Dermatosis cenicienta es una entidad clínica poco común, de curso crónico e irreversible. Se presenta como máculas hiperpigmentadas color gris-ceniza, de tamaño y forma variable, distribuidas en cara, tronco, y extremidades. En Chile existen pocos casos reportados. La etiología es actualmente desconocida. Los hallazgos histopatológicos no son específicos de EDP, por lo que el diagnóstico es clínico e histopatológico. Aunque se han reportado múltiples opciones de tratamiento, ninguno ha demostrado ser efectivo de manera consistente. Presentamos dos casos de EDP y una revisión de los principales aspectos clínicos, epidemiológicos e histopatológicos de esta entidad.

Erythema dyschromicum perstans (EDP) or cinderella Dermatosis is an uncommon clinical entity, of chronic and irreversible course. Clinically it appears as hyper pigmented gray-ashy macules, of variable size and shape, distributed on face, trunk, and extremities. There are few cases reported on Chile. Etiology is currently unknown. The histopathological features are not characteristic of EDP, so the diagnosis is based upon clinical findings plus histopathology. Although there are several treatment options, none of them have been demonstrated to be consistently effective. We present two cases of EDP and a review of the clinical, epidemiological, and histopathological features of this entity.

Dermoscopy as an auxiliary tool for the diagnosis of furuncular myiasis

Furuncular myiasis occurs after larvae penetrate on the skin. The disease is characterized by the presence of a nodule with a central hole through which there is serosanguinous exudate drainage. The authors present a case of furuncular myiasis by Dermatobia hominis in which late diagnosis made it necessary to have the orifice margins surgically enlarged in order to extract the larva. They also emphasize that dermoscopy is a useful auxiliary tool in this diagnosis.

Eritema Ab Igne como dermatosis laboral: revisión de la literatura a partir un caso clínico / Erythema ab igne as a dermatosis labor: case report and literature review

El eritema ab igne es una entidad que fue descrita por primera vez por Buschke como “hitze melanosis” (melanosis inducida por calor), en piernas de personas que trabajaban frente a fuegos abiertos o estufas de carbón. Se define como lesiones cutáneas eritematosas o hiperpigmentadas en un patrón reticulado característico, secundario a la exposición crónica de bajos niveles de radiación infrarroja. A pesar que la aparición de calefacción centralha significado una dramática disminución en su incidencia, aún se describen casos de causa cultural, médica o laboral. Una de susconsecuencias más graves es la posibilidad de aparición de un carcinoma espinocelular sobre la lesión. A continuación se presenta un caso clínico de etiología laboral y se realizará una revisión de la literatura.

Erythema ab igne is an entity that was first described by Buschke as “hitze melanosis” (heat-induced melanosis), in the legs of people working in front of open fires or coal stoves. It is defined as erythematous or hyperpigmented skin lesions in a reticulated pattern. The main cause is the chronic exposure tolow levels of infrared radiation. Although the occurrence of central heating has meant a dramatic decrease in incidence, cultural, medical or occupational causes are still described. One of the most serious consequences is the possibility of develoging an squamous cell carcinoma. We present a case of occupational aetiology and a review of the literature.