Subcorneal pustular dermatosis type of IgA Pemphigus in a 35-year-old female: A case report

Introduction@#IgA pemphigus is a rare, distinct variant of Pemphigus characterized by vesiculopustular eruptions mediated by IgA autoantibodies targeting keratinocyte cell surface antigens, desmocollins 1-3 and sometimes desmogleins 1 and 3. Its classical features have been described in literature but atypical cases have also been documented. This report presents such case posing a diagnostic dilemma.@*Case Report@#A 35-year-old female presented with a 16-year history of intermittent eruptions of multiple hyperpigmented, annular and circinate, desquamating plaques and coalescing flaccid pustules on erythematous bases on the scalp, neck, trunk, and extremities. Histopathologic examination revealed subcorneal pustular dermatitis, and direct immunofluorescence was positive for granular intercellular IgG and IgA deposits in the epidermis. Antinuclear antibody test was negative and C3 level was normal. Antibody tests against desmogleins 1 and 3 were both negative. Topical potent corticosteroid therapy resulted in complete resolution of all lesions in three weeks.@*Conclusion@#Diagnostic dilemmas arise when laboratory results do not correlate with clinical findings. Findings of IgA autoantibodies in patients with pemphigus-like skin eruptions led to the diagnosis of subcorneal pustular dermatosis type of IgA pemphigus. Dapsone is the treatment of choice although topical potent corticosteroid alone may provide complete remission in some cases, avoiding the potential adverse effects of systemic therapy

Generalized pustular psoriasis (von Zumbusch)

Abstract Generalized pustular psoriasis (von Zumbusch) is a rare and acute eruption characterized by multiple sterile pustules over an erythematous and edematous background, eventually associated with psoriasis vulgaris. Classically, it manifests as a potentially severe systemic picture and demands prompt diagnosis and intervention. The duration of each flare-up and intervals between the pustular episodes is extremely variable. Recently, genetic abnormalities have been identified mainly in the familial and early variants of this disease. The therapeutic arsenal is limited; however, new drugs being evaluated aim to control both pustular flare-ups and disease recurrences.

Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Abstract Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

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.

Gaze vaselinada para prevenção de lesões por pressão em pessoas com dermatoses imunobolhosas / Vaseline gauze for prevention of pressure injuries in people with immunobullous dermatoses / Gasa con vaselina para la prevención de lesiones por presión en personas con dermatosis inmunobulosa

Objetivo: avaliar a mobilidade do cliente com dermatose imunobolhosa antes e após aplicação do curativo com gaze vaselinada. Método: estudo quase experimental, interinstitucional, com clientes com dermatoses imunobolhosas hospitalizados em um hospital estadual e um hospital federal do Estado do Rio de Janeiro e uma instituição do Mato Grosso do Sul. Utilizou-se a lógica fuzzy para classificar a mobilidade dos sujeitos antes, 24 horas após e uma semana após aplicação do curativo. A pesquisa foi aprovada pelo Comitê de Ética em Pesquisa. Resultados: Incluídos 14 participantes, sendo nove com pênfigo vulgar, dois com pênfigo foliáceo e três com penfigóide bolhoso, entre 27 e 82 anos, predominando 11 mulheres. Após 24 horas, nenhum participante se considerou com baixa mobilidade, sete passaram a mobilidade média, e sete, alta, o que foi mantido uma semana após aplicação do curativo. Conclusão: constatou-se significativo aumento da mobilidade logo nas primeiras 24 horas após aplicação do curativo.

Objective: to assess the mobility of clients with immunobullous dermatoses, before and after applying vaseline gauze dressings. Method: in this quasi-experimental, interinstitutional study of inpatients with immunobullous dermatoses at a state hospital and a federal hospital in Rio de Janeiro State and an institution in Mato Grosso do Sul (Brazil), patient mobility before, 24 hours after, and one week after applying the dressing was classified using fuzzy logic. The study was approved by the research ethics committee. Results: 14 participants, nine with pemphigus vulgaris, two with pemphigus foliaceus, and three with bullous pemphigoid, aged between 27 and 82 years old, and predominantly (11) women. After 24 hours, none of the participants considered their mobility to be poor, seven began to be moderately mobile, and seven were highly mobile, and continued so one week after applying the dressing. Conclusion: mobility increased significant in the first 24 hours after applying the dressing.

Objetivo: evaluar la movilidad de clientes con dermatosis inmunobullosa, antes y después de la aplicación de apósitos de gasa con vaselina. Método: en este estudio cuasi-experimental, interinstitucional de pacientes hospitalizados con dermatosis inmunobullosa en un hospital estatal y un hospital federal en el estado de Río de Janeiro y una institución en Mato Grosso do Sul (Brazil), la movilidad del paciente antes, 24 horas después y una semana después la aplicación del apósito se clasificó mediante lógica difusa. El estudio fue aprobado por el comité de ética en investigación. Resultados: se incluyeron 14 participantes, nueve con pénfigo vulgar, dos con pénfigo foliáceo y tres con penfigoide ampolloso, con edades comprendidas entre 27 y 82 años, y predominantemente mujeres (n=11). Después de 24 horas, ninguno de los participantes consideró que su movilidad fuera pobre, siete comenzaron a ser moderadamente móviles y siete eran altamente móviles, y así continuaron una semana después de la aplicación del apósito. Conclusión: la movilidad aumentó significativamente en las primeras 24 horas después de la aplicación del apósitoconsideraba con baja movilidad, siete comenzaron a tener movilidad media y siete, alta, que se mantuvo una semana después de aplicar el apósito. Conclusión: hubo un aumento significativo en la movilidad en las primeras 24 horas después de aplicar el apósito.

Acute bullous hemorrhagic prurigo: a diagnostic challenge

Abstract Insect bites and bite wounds are quite common and most often have mild repercussions in humans. Statistics on the incidence of accidents caused by insects are not available, and the skin reactions after the bites are not always known. The authors present two cases of patients with hemorrhagic blisters on their hands after tabanidae bites and discuss the factors that cause the problem and the importance of the differential diagnosis of blisters with hemorrhagic content on human skin.

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)

Impetigo herpetiformis in a 33-year-old Filipino female

@#Introduction: Impetigo herpetiformis is a rare pustular disorder that affects pregnant women. It is also otherwise termed as “pustular psoriasis of pregnancy”, owing to the fact that the pustules are sterile and are not associated with a viral etiology. The classic lesions are erythematous patches or plaques with margins studded with subcorneal pustules spreading centrifugally. A cardinal feature of this disorder is the rapid resolution of lesions after delivery. Case Summary: This is a case report of a 33-year-old female, gravida 3, para 2 (2-0-0-2) at 36 weeks age of gestation who presented with one week history of multiple well defined irregularly shaped erythematous annular patches and plaques with marginal pustules on the trunk and extremities. The lesions began on the trunk spreading centrifugally, sparing the face, palms, soles and mucus membrane. Biopsy showed scale crust in the stratum corneum, the epidermis showed acanthosis, spongiosis and neutrophilic microabscesses with focal vacuolar alteration and small sub-epidermal nests on the basal cell layer. The dermis revealed dilated blood vessels and mild superficial perivascular lymphocytic infiltrates. Patient was given Prednisone and Cetirizine with noted resolution of lesions. Conclusion: Recurrences of impetigo herpetiformis in subsequent pregnancies are common with earlier onset in gestation and are characteristically more severe. The complications are placental insufficiency, stillbirth or neonatal death. Early detection is of utmost importance. Management must be multidisciplinary involving a dermatologist, obstetrician and pediatrician.

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.

Vesiculobullous exanthema in a 3-month-old child with probable acute chikungunya infection / Exantema vesiculobolhoso em criança de 3 meses com provável infecção aguda por chikungunya

Objective: to report a rare case of a 3-month-old child with acute chikungunya infection presenting as vesiculobullous rash. Case description: herein we report a case of a 3-month-old infant which developed a diffuse erythematous rash involving the trunk and limbs within two days after sudden acute fever, and also presented striking irritability with inconsolable crying, hyporexia, cough, and coryza. Hematological and biochemical investigations were undertaken and were found to be normal. Serum samples were tested for Chikungunya revealing positive IgM. He was treated with supportive therapy, mainly with antipyretics, venous hydration and rest evolving with entirely resolution of the symptoms after four days. Comments: Chikungunya fever is a disease caused by the Chikungunya virus which can be found in endemic areas such as Brazil. Atypical manifestations can affect various systems including the skin and may be more frequent in children. The role of dermatological manifestations related to Chikungunya infection is not completely understood. The reported case is an unusual case of Chikungunya infection which highlights the importance of to know and to be aware of this manifestation in children mainly in new endemic areas such as Brazil.

Objetivo: relatar o caso raro de uma criança de 3 meses de idade com infecção aguda por chikungunya apresentando-se como erupção vesiculobolhosa. Descrição do caso: aqui relatamos o caso de uma criança de 3 meses que evoluiu com erupção eritematosa difusa envolvendo tronco e membros dois dias após febre aguda repentina, além de apresentar irritabilidade marcante com choro inconsolável, hiporexia, tosse e coriza. Investigações hematológicas e bioquímicas foram realizadas e foram normais. Amostras de soro foram testadas para Chikungunya revelando IgM positiva. Ele foi tratado com terapia de suporte, principalmente com antipiréticos, hidratação venosa e descanso evoluindo com resolução completa dos sintomas após quatro dias. Comentários: a febre Chikungunya é uma doença causada pelo vírus Chikungunya, que pode ser encontrada em áreas endêmicas como o Brasil. Manifestações atípicas podem afetar vários sistemas, incluindo a pele, e podem ser mais frequentes em crianças. O papel das manifestações dermatológicas relacionadas à infecção por Chikungunya não é completamente compreendido. O caso relatado é um caso incomum de infecção por Chikungunya, que destaca a importância de conhecer e ter consciência dessa manifestação em crianças, principalmente em novas áreas endêmicas como o Brasil.

Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases' patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires

Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

Bullous systemic lupus erythematosus ­ a case report

Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30­40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE­a rare presentation of SLE­which may evolve with marked clinical presentation

A Case of Pediatric Subcorneal Pustular Dermatosis / 대한피부과학회지

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson's disease, is a rare pustular eruption that occurs mainly in middle-aged women and rarely during childhood. Clinically, the pustules are distributed on the trunk and proximal region of the limbs, and cultures of the pustules consistently do not reveal bacterial growth. Histopathology shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. SPD is a rare condition, especially in children, and only one case has been reported in Korea. Here, we report a case of 4-year-old girl as an educational case for the diagnosis and treatment of pediatric SPD.

A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type / 대한피부과학회지

Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the half-and-half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.

A case of Sneddon-Wilkinson disease with hypersensitivity to dapsone successfully managed with colchicine

Introduction@#Sneddon-Wilkinson disease (SWD) is a rare, recurrent neutrophilic dermatosis presenting as sterile pustules, with a predilection for flexural and intertriginous areas.@*Case summary@#A 49-year-old Filipino female presented with a three-year history of recurrent pustules and papules on the flexural areas of trunk and extremities. Skin punch biopsy was done and histopathology was consistent with subcorneal pustular dermatosis/SWD. She was started on Dapsone but after two weeks of intake, the patient developed generalized erythematous desquamating plaques on the trunk and extremities, with palmoplantar involvement. The patient did not have fever, jaundice, lymphadenopathy, and abdominal tenderness. Laboratory investigation such as complete blood count and liver function tests were normal. The final diagnosis was SWD with hypersensitivity to Dapsone. Dapsone was immediately discontinued and she was shifted to oral colchicine. After six weeks of oral colchicine therapy, the lesions have completely resolved. Patient was in remission for six months thereafter.@*Conclusion@#SWD is rare and the drug of choice is dapsone. In instances where dapsone is not suitable, oral colchicine can be an ideal alternative treatment.

Cuidados de enfermagem para conforto de pessoas com dermatoses imunobolhosas: avaliação pela lógica fuzzy / Nursing care to comfort people with immunobullous dermatoses: evaluation by fuzzy logic / Cuidados de enfermería para la comodidad de personas con dermatosis inmunoampollares: evaluación por la lógica fuzzy

Objetivos: avaliar se as intervenções propostas na tecnologia de cuidados de enfermagem ao cliente com dermatoses imunobolhosas contribuem para reduzir o desconforto, reconhecer padrões de desconforto antes e após aplicação da tecnologia. Método: aplicação do protocolo de avaliação em 14 clientes hospitalizados em enfermarias de dermatologia do Rio de Janeiro e Mato Grosso do Sul, Brasil. Os cuidados foram realizados mediante diagnósticos identificados e recomendações da tecnologia. A subjetividade em reco¬nhecer padrões de conforto em clientes com doenças raras direcionou o uso da lógica fuzzy em função dos atributos dor, mobilidade, padrão de sono, exposição do corpo/lesões. Aprovado conforme CAAE: 0258.0.228.000-11. Resultados: dos 14 participantes, oito verbalizaram redução da dor após 24 horas. Após uma semana, três declararam maior redução, cinco manutenção e cinco aumento. Conclusão: a análise inferencial fuzzy propiciou avaliar padrões de desconforto, apontando para a veracidade da hipótese de que a tecnologia contribui para promover o conforto da clientela.

Objectives: to evaluate whether interventions proposed in nursing care technology for clients with immunobullous dermatoses contribute to reducing discomfort, and to recognize patterns of discomfort before and after application of the technology. Method: the evaluation protocol was applied to 14 patients hospitalized in dermatology wards in Rio de Janeiro and Mato Grosso do Sul, Brazil, from June 2012 to April 2013. Care was performed by way of identified diagnoses and recommendations in the technology. The role of subjectivity in rec¬ognizing comfort patterns in clients with rare diseases indicated the use of fuzzy logic with attributes of pain, mobility, sleep pattern, and body exposure/lesions. The study was approved under CAAE: 0258.0.228.000-11. Results: of the 14 patients, eight reported diminished pain after 24 hours. After one week, three reported larger reductions; five, no change; and five, increased pain. Conclusion: evaluation of patterns of discomfort by fuzzy inferential analysis supported the hypothesis that the technology contributes to promoting client comfort.

Objetivos: evaluar si las intervenciones propuestas en la tecnología de cuidados de enfermería al cliente con dermatosis inmunoampollares contribuyen a reducir el incómodo; reconocer patrones de incomodidad antes y después de la aplicación de la tecnología. Método: aplica¬ción del protocolo de evaluación en 14 pacientes hospitalizados en enfermerías de dermatología de Río de Janeiro y Mato Grosso do Sul, Brasil, de junio/2012 a abril/2013. Los cuidados se han realizado mediante diagnósticos identificados y recomendaciones de la tecnología. La subjetividad en reconocer patrones de comodidad en pacientes con enfermedades raras dirigió el uso de la lógica fuzzy en función de los atributos dolor, movilidad, patrón de sueño, exposición del cuerpo / lesiones. Aprobado conforme CAAE: 0258.0.228.000-11. Resultados: de los 14, ocho reportaron reducción del dolor después de 24 horas. Tras una semana, tres declararon una mayor reducción, cinco mantu¬vieron y cinco aumentaron. Conclusión: el análisis inferencial fuzzy propició evaluar patrones de incomodidad, señalando la veracidad de la hipótesis de que la tecnología contribuye a promover la comodidad de los pacientes.

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.

Atypical Gianotti-Crosti syndrome

Abstract: We report the case of a male 22-month-old child, with atypical presentation of Gianotti-Crosti syndrome after infection with Epstein-Barr virus.