Lesiones ampollares en una paciente con morfea panesclerótica discapacitante de la infancia / Bullous lesions in a patient with panesclerotic morphea of the childhood

La morfea ampollar es un tipo enfrecente de esclerodermia localizada que se caracteriza por presentar ampollas sobre placas escleróticas. La presencia de este tipo de lesiones obliga a descartar la variante extraenital de liquen esclerodemias localizadas, es posible hallar ambas afecciones. Se describe el caso de una paciente de 19 años con diagnóstico de morfea panesclerótica y liquen escleroso ampollar.

Bollous morphea is an infreqent type of morphea characterized for developing bullae on sclerodermiformic plaques. The presence of bullae forces to discard lichen sclerosus, a disease that usually develops in the genital zone, the extragenital variant could belong to the same spectrum that localized sclerodermiformic diseases. We present a 19 year old female patient with the diagnosis of panesclerotic morphea and bullous lichen sclerosus.

Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Diagnostic approach of eosinophilic spongiosis

Abstract Eosinophilic spongiosis is a histological feature shared by some distinct inflammatory disorders, and is characterized by the presence of intraepidermal eosinophils associated with spongiosis. Most often, isolated eosinophilic spongiosis indicates the early stages of a subjacent autoimmune bullous dermatosis, such as the pemphigus group and bullous pemphigoid. Herein, the main causes of eosinophilic spongiosis are discussed, as well as the supplementary investigation needed to elucidate its etiology.

Adult linear IgA bullous dermatosis: report of three cases

Abstract: Linear immunoglobulin A bullous dermatosis is a rare autoimmune disease that usually has an excellent prognosis in childhood; however, its control is more difficult in adults. It presents heterogeneous clinical manifestations and is frequently confused with other bullous diseases such as bullous pemphigoid and Duhring's dermatitis herpetiformis. Dermatologists' awareness of this disease contributes to early diagnosis and appropriate treatment. We thus report three cases of linear immunoglobulin A dermatosis in adults.

Subcorneal pustular dermatosis in the pediatric age

Abstract: Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.

Epidermólisis bullosa: piel de mariposa. A propósito de un caso / Epidermolysis bullosa: butterfly skin. Apropos of a case

La epidermólisis bullosa comprende un grupo heterogéneo de enfermedades ampollosas de la piel y las mucosas, son de origen congénito y hereditario. Hacer el diagnóstico no es difícil si se tiene experiencia dermatológica, pero su clasificación es compleja y para ello se necesita considerar la clínica, la genética, la microscopia y la evaluación de laboratorio. El tratamiento de esta enfermedad es también dificultoso y son necesarias ciertas medidas, para proteger al paciente, evitar la aparición de lesiones y las complicaciones derivadas de ellas. Se describe el tratamiento de estas lesiones en un recién nacido, al que se administraron antibióticos profilácticos y se colocaron vendajes en las lesiones. Se describieron todos los cuidados y recomendaciones para evitar, especialmente los roces y las presiones en estas lesiones, así como las temperaturas altas. Para la confección del presente trabajo se consultaron 18 materiales entre revistas y libros de Pediatría. El caso reportado fue un recién nacido con epidermólisis bullosa atendido en el Hospital Universitario "Dr. Mario Muñoz Monroy" de Colón, Matanzas. Se demostró lo poco frecuente y raro de esta patología para los especialistas del tema (AU).

The epidermolysis bullosa includes a heterogeneous group of bullous skin and mucous diseases of congenital and hereditary origin. Diagnosing them is not difficult if the specialist has dermatologic experience, but their classification is complex and it is necessary to take into account the clinical, genetic and microscopic factors, and the laboratory assessment. The treatment of this disease is also difficult and it is necessary to take certain measures to protect the patient, avoid the onset of lesions and the complications derived from them. The treatment of these lesions in a newborn is described. Prophylactic antibiotics were administered and bandages were put on the lesions. All the cares and recommendations to avoid rubbings and pressures on these lesions, and also the high temperatures, are described. To develop the current term, 18 materials (journals and pediatric books) were consulted. The reported case was the case of a newborn with epidermolysis bullosa attended in the University Hospital "Dr. Mario Muñoz Monroy" of Colon, Matanzas. It was demonstrated the low frequency and rarity of this pathology for the specialists of the theme (AU).

Pemphigus foliaceus as a differential diagnosis in vesicobullous lesions / Pênfigo foliáceo como diagnóstico diferencial em lesões vesicobolhosas

ABSTRACT Given the challenge of clinical diagnosis of bullous skin lesions, this report aimed to discuss the histological changes, the presentation and clinical reasoning for diagnosis of these lesions. At the same time, the importance of the pathology was reviewed to identify these clinical scenarios. In this case report, we highlighted the clinical progression of a case of pemphigus foliaceus.

RESUMO Considerando o desafio do diagnóstico clínico de lesões cutâneas de apresentação bolhosa, o presente trabalho procurou discutir as alterações histológicas, a apresentação e o raciocínio clínico para o diagnóstico de tais lesões. Paralelamente, a importância da patologia foi revisada na identificação destes quadros. Neste relato de caso, destaca-se a evolução clínica de um quadro de pênfigo foliáceo.

Enfermedad de Sneddon-Wilkinson / Sneddon-Wilkinson disease

Introducción: la dermatosis pustulosa subcórnea o enfermedad de Sneddon-Wilkinson, es una enfermedad poco común, que se distingue por pústulas flácidas, recurrentes y coalescentes; su mecanismo etiológico no está del todo dilucidado, pero se relaciona con la presencia de enfermedades neoplásicas o inmunológicas. Presentación del caso: se presenta el caso de un niño de 4 años de edad, que acude a consulta de Dermatología del Hospital Andino de Chimborazo, con manifestaciones clínicas que permiten hacer el diagnóstico de dermatosis pustulosa subcórnea. Conclusiones: la enfermedad de Sneddon-Wilkinson es una afección rara que se presenta frecuentemente en pacientes femeninas de 40 años y más. Se caracteriza por una amplia diversidad de manifestaciones cutáneas que dificultan el pronóstico y la evolución del paciente. Esta enfermedad, cuando se presenta en niños, puede confundirse con otras afecciones que cursan con exantemas cutáneos(AU)

Introduction: subcorneal pustular dermatosis or Sneddon-Wilkinson disease is a rare illness characterized by recurrent, coalescent and flaccid pustules; its etiological mechanism is not totally known, but it is related to neoplastic or immunological diseases. Case presentation: a four-year old child who went to the dermatology service of Hospital Andino de Chimborazo. He presented with clinical manifestations that allow making the diagnosis of subcorneal pustular dermatosis. Conclusions: Sneddon-Wilkinson disease is a rare illness that frequently occurs in 40 years-old and older women. It has a wide range of skin manifestations that makes prognosis and recovery of patient difficult. When it appears in children, it may create confusion with other illnesses having skin exanthemas(AU)

Nursing diagnoses in patients with immune-bullous dermatosis / Diagnósticos de enfermagem em clientes com dermatoses imunobolhosas / Diagnósticos de enfermería en pacientes con inmuno-dermatosis ampollosa

ABSTRACT Objective: identify nursing diagnoses in patients with immune-bullous dermatosis. Method: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. Results: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11). 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. Conclusion: the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology.

Resumo Objetivo: identificar diagnósticos de enfermagem em clientes com dermatoses imunobolhosas. Método: pesquisa quantitativa e descritiva, realizada em três instituições localizadas no Rio de Janeiro e no Mato Grosso do Sul-Brasil, aplicando o Protocolo de Avaliação do Cliente em Dermatologia, durante consulta de enfermagem. Utilizou-se a estatística descritiva simples para análise dos dados. Resultados: participaram do estudo 14 sujeitos, nove com diagnóstico médico de pênfigo vulgar, dois de foliáceo e três de penfigoide bolhoso. A idade variou entre 27 e 82 anos, predominando 11 pessoas do sexo feminino. Foram discutidos 14 diagnósticos de enfermagem identificados a partir do raciocínio clínico, em todos os participantes do estudo, representando as respostas humanas mais frequentes nesta amostra. A aplicação do Protocolo de Avaliação do Cliente em Dermatologia facilitou a avaliação integral, além de propiciar a identificação dos diagnósticos de acordo com a North American Nursing Diagnosis Association International. Conclusão: os diagnósticos de enfermagem apresentados ratificam a necessidade do trabalho interdisciplinar durante atendimento a esta clientela. Para melhor descrição dos fenômenos relacionados à clientela em questão, sugere-se a inclusão de dois fatores de risco/relacionados em três diagnósticos desta taxonomia. Cabe ressaltar a contribuição dos achados para o cuidar/educar/pesquisar em enfermagem em dermatologia.

RESUMEN Objetivo: identificar los diagnósticos de enfermería en pacientes con inmuno dermatosis ampollosa. Método: investigación cuantitativa y descriptiva, realizada en tres instituciones ubicadas en Río de Janeiro y Mato Grosso do Sul, Brasil, utilizando el Protocolo de Evaluación del Cliente en Dermatología en la consulta de enfermería. Se utilizó estadística descriptiva simples para el análisis de datos. Resultados: 14 sujetos participaron en el estudio, nueve con diagnóstico de pénfigo vulgar, dos de pénfigo foliáceo y tres de penfigoide ampolloso. La edad osciló entre 27 y 82 años, predominio femenino con 11 mujeres. Se discutieron 14 diagnósticos de enfermería identificados desde el razonamiento clínico, en todos los participantes en el estudio, que representa las respuestas humanas más comunes en esta muestra. La aplicación del Protocolo de Evaluación de Dermatología facilitó la evaluación global, además de proporcionar la identificación de los diagnósticos de acuerdo con la North American Nursing Diagnosis Association International. Conclusión: los diagnósticos de enfermería presentados confirman la necesidad del trabajo interdisciplinario en el servicio a estos clientes. Para una mejor descripción de los fenómenos relacionados con los clientes en cuestión, se sugiere la inclusión de dos factores de riesgo/relacionados en tres diagnósticos de esta taxonomía. Vale la pena señalar la contribución de los hallazgos para el cuidado/educación/investigación en enfermería en dermatología.

Pustulosis neonatales / Neonatal pustulosis

Las pustulosis neonatales son un grupo de enfermedades frecuentes, que deben ser reconocidas en forma precoz ya que pueden conllevar a una gran morbimortalidad en un grupo etario muy vulnerable. Realizamos una revisión de los conceptos de algunas de las entidades dentro de este grupo a partir de cinco casos clínicos estudiados en nuestro servicio (AU)

Neonatal pustulosis is a group of common diseases, which should be recognized at an early stage and that can lead to a high morbidity and mortality in a very vulnerable age group. We review the concepts of some of the entities within this group due to five clinical cases studied in our service (AU)

Detección de anticuerpos circulantes en suero para el diagnóstico de epidermólisis ampollar adquirida / Detection of circulating antibodies in serum for the diagnosis of acquired blister epidermolysis

Objetivo: realizar una revisión, apreciación crítica y síntesis de la evidencia disponible sobre la validez diagnóstica de la detección de anticuerpos circulantes para el diagnóstico de epidermolisis ampollar adquirida. Metodología: se realizó una búsqueda de evidencia en las bases de datos: MEDLINE, EMBASE, la Librería Cochrane y LILACS. Adicionalmente, se hizo se indagó por estudios locales a través del motor de búsqueda Google. Dos evaluadores de manera independiente, tamizaron las referencias obtenidas, resolviendo las discrepancias por consenso. Resultados: se identificaron 49 publicaciones. Con los resultados obtenidos, no fue posible identificar revisiones sistemáticas de la literatura ni estudios de validez diagnóstica de la IFD. Se hizo una preselección de 7 estudios observacionales. Fue incluido 1. Se presentan los datos descriptivos sobre la positividad de las pruebas usadas para detectar anticuerpos circulantes en personas con sospecha clínica de epidermólisis ampollar adquirida. Conclusiones: con los hallazgos presentados en este informe, no es posible conocer el desempeño de las pruebas para detectar anticuerpos circulantes en suero de pacientes con sospecha clínica de epidermolisis ampollar adquirida.(AU)

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...

Tumourlike Lesions of Skin - A Clinicopathological Analysis of 175 Cases.

As the skin is the largest and complex organ, a wide variety of tumourlike lesions are encountered in the clinical practice. The ability to properly diagnose & treat these common lesions & to distinguish them from malignant tumours is the vital skills for all clinicians. Objective: To analyse retrospectively tumourlike lesions of skin with respect to age, sex, clinical features and histopathological features in a tertiary referral centre in Maharashtra, India. Methods : The present study consisted of analysis of tumourlike lesions of skin received in the histopathology section of department of pathology over a period of 5 years that is from August 2005 to July 2010 .The material comprised of biopsies and excision specimens. The clinical and histopathological details were noted. The findings were compared with those reported by other authors. Results : One hundred and seventy five (175) cases of tumourlike lesions of skin were seen .These lesions presented as skin swellings or tumours. Maximum cases (34.8%) of tumourlike lesions occurred in fourth & fifth decades with male preponderance (1.35:1). The maximum number of cases was encountered in the head & neck region (63.4%). Their size ranged from 0.4 to 9 cms. Epidermal cyst was the commonest tumourlike lesion (59.4%) followed by dermoid cyst (13.1%).Other lesions were trichilemmal cyst, fibroepithelial polyp, keloid, hypertrophic scar and epidermal nevus. Conclusion : Tumourlike lesions are clinically diagnosed by their presentation. However, the histopathological examination confirms the clinical diagnosis. The pathologic evaluation of all tumourlike lesions is mandatory to avoid patient’s and family’s anxiety.

Widespread eosinophilic pustular folliculitis in a nonimmunocompromised patient

We present a case of eosinophilic pustular folliculitis, a rare dermatosis which is often associated with HIV infection or internal malignancies. Clinical Presentation and Intervention: We report the case of a 66-year-old man with a medical history of hypertension. Histopathological examination showed a dense follicular inflammatory infiltrate with abundant eosinophils. The clinical response to indomethacin was excellent with no recurrence during the follow-up. The patient responded well to indomethacin treatment

Dermatoses neutrofílicas: parte II / Neutrophilic dermatoses: part II

Neste artigo são abordadas as dermatoses neutrofílicas, complementando o artigo anterior (parte I). São apresentadas e comentadas as seguintes dermatoses: pustulose subcórnea de Sneddon-Wilkinson, dermatite crural pustulosa e atrófica, pustulose exantemática generalizada aguda, acroder matite contínua de Hallopeau, pustulose palmoplantar, acropustulose infantil, bacteride pustular de Andrews e foliculite pustulosa eosinofílica. Uma breve revisão das dermatoses neutrofílicas em pacientes pediátricos também é realizada.

This article addresses neutrophilic dermatoses, thus complementing the previous article (part I). The following dermatoses are introduced and discussed: subcorneal pustular dermatosis (Sneddon-Wilkinson disease), dermatitis cruris pustulosa et atrophicans, acute generalized exanthematous pustulosis, continuous Hallopeau acrodermatitis, palmoplantar pustulosis, infantile acropustulosis, Andrews' pustular bacteride and eosinophilic pustular folliculitis. A brief review of neutrophilic dermatoses in pediatric patients is also conducted.

Acropustulosis infantil / Infantile acropustulosis

La acropustulosis infantil (AI) es una patología benigna, autorresolutiva, que se presenta con mayor frecuencia en los primeros años de la vida, caracterizada por episodios recurrentes de pápulas, vesículas o pústulas, muy pruriginosas y de localización acral. Su etiología es desconocida y en ocasiones terapéuticas, entre las que se destacan los glucocorticoides tópicos de mediana y alta potencia. Se realizó un estudio retrosptectivo de 22 pacientes con diagnóstico de AI atendidos en nuestra institución durante un período de 12 años; se analizaron variables epidemiológicas, clínicas y terapéuticas. El 63,64% de los pacientes eran de sexo femenino; la edad promedio de comienzo de los síntomas fue 11 meses; 8 pacientes referían haber realizado tratamiento previo para escabiosis, pero sólo en 3 se pudo confirmar el diagnóstico. El tratamiento utilizado consistió en glucocorticoides tópicos asociado a antihistamínicos con mejoría de los síntomas en cada brote.