Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients

Kong, Yan; Y, Lim; Chandran, Nisha Suyien

doi:10.1111/pde.12684

Cited by 30 publications

(52 citation statements)

References 22 publications

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“…had a preadolescent onset. [5] The pediatric patients represented 1.9% of all patients diagnosed with PV at our institution during the study period ( n = 106), which is similar to the ratio (2.9%) reported by Yazganoglu et al . from another large university hospital in Istanbul.…”

Section: Discussionsupporting

confidence: 85%

“…In many aspects, this study bears similarities to a more recent single-center retrospective study from Singapore: In both studies, there was a male predominance (female/male: 1/2), and the same number of patients with LAD, PV, and BP were enrolled (5, 2, and 2, respectively). [5] Moreover, no pediatric case of DH was detected in either of the two studies. This is particularly interesting, considering the data from the Western hemisphere indicating that DH is among the most common immunobullous disorders of childhood.…”

Section: Discussionmentioning

confidence: 99%

“…[2] Furthermore, this study did not include any patients with epidermolysis bullosa acquisita or bullous lupus erythematosus, as opposed to the studies by Kirtschig et al . [3] and Kong et al .,[5] respectively.…”

Section: Discussionmentioning

confidence: 99%

“…Of note, most of the patients with juvenile pemphigus reported in the literature had a good prognosis; however, treatment-resistant cases, similar to our patients, have been reported. [58] As it is the case with pemphigus in adult age, rituximab represents a valuable treatment option for pediatric patients, as well. [9] In both of our patients, rituximab was required to obtain remission.…”

Section: Discussionmentioning

confidence: 99%

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Autoimmune bullous disease in childhood

2017

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Background:Autoimmune bullous disorders (AIBDs) are a heterogeneous group of diseases which are rarely seen in children. Studies concerning the immunobullous diseases in pediatric patients are scarce.Aims and Objectives:In this study, we aimed to investigate the clinical features and treatment outcomes of AIBDs in children.Materials and Methods:The electronic records of the patients in our AIBDs outpatient clinic were retrospectively reviewed. All cases diagnosed before the age of 16 years were included in the analysis of clinical features, treatment outcomes, and follow-up data.Results:Of the 196 patients with immunobullous diseases, 9 (4.6%) were diagnosed before the age of 16 years. Mean age of the patients at the time of diagnosis was 7.72 ± 5.66 years. Among nine patients, linear immunoglobulin A disease (LAD), pemphigus vulgaris (PV), and bullous pemphigoid (BP) were seen in 5, 2, and 2 children, respectively. All patients were treated with at least two systemic agents (including methylprednisolone, dapsone, methotrexate, salazopyrine, intravenous Ig [IVIg], and rituximab) leading to clinical remission in all of them after a mean period of 31.77 ± 27.99 months.Conclusion:In line with earlier studies, LAD was the most common immunobullous disease and in general, associated with a favorable response to dapsone. This study was noteworthy in that the patients with PV and BP demonstrated a relatively more recalcitrant course, requiring rituximab and IVIg for remission, respectively. Overall, patients had a good prognosis.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Autoimmune bullous disease in childhood

2017

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“…4,5 Actualmente, el rituximab, un anticuerpo monoclonal dirigido contra el antígeno CD20 de e460 / Arch Argent Pediatr 2016;114(6):e457-e460 / Presentación de casos clínicos los linfocitos B, ha sido propuesto para aquellos casos refractarios a los corticoides. 3,9,10 Es importante también tener en cuenta el control nutricional, la analgesia, la higiene y la curación de heridas. 6 …”

Section: Figura 3 Ampollas Con Contenido Seroso Erosiones Y Costrasunclassified

Pénfigo vulgar de la infancia, a propósito de un caso

et al. 2016

Arch Argent Pediat

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RESUMENEl pénfigo vulgar es una enfermedad crónica que se caracteriza por ampollas fláccidas y erosiones, causadas por un fenómeno autoinmune con autoanticuerpos inmunoglobulina G circulantes contra componentes de la epidermis. Su presentación en la infancia es altamente infrecuente; representa el 1,4%-2,9% de todos los casos de pénfigo vulgar. Presentamos un caso de pénfigo vulgar en una niña de 12 años, que implicó un desafío en su diagnóstico y terapéutica. Palabras clave: pénfigo vulgar, niños. ABSTRACTPemphigus vulgaris is a chronic disease that is characterized by flaccid blisters and erosions, caused by immunoglobulin G autoantibodies against epidermal components. It is highly unusual in the pediatric population; it represents the 1.4 -2.9% of the total cases. We present a case of pemphigus vulgaris in a 12 year old girl, which has implied a challenge both in its diagnosis and in its treatment. Key words: pemphigus vulgaris, children.http://dx.doi.org/10.5546/aap.2016.e457 INTRODUCCIÓNPénfigo es un término que se aplica a un grupo de enfermedades ampollares de origen autoinmune, graves y crónicas, que comprometen la piel y las mucosas.Esta entidad puede clasificarse en pénfigo vulgar, foliáceo, inducido por fármacos, pénfigo inmunoglobulina A (IgA) y paraneoplásico. 1 Pénfigo vulgar de la infancia, a propósito de un caso Childhood pemphigus vulgaris, a case reportEl pénfigo vulgar es una enfermedad ampollar crónica, potencialmente mortal, muy infrecuente en pediatría.Se realiza la presentación de este caso clínico, debido al desafío que representa su sospecha y diagnóstico para el dermatólogo pediatra y que obliga a un abordaje multidisciplinario tanto en el manejo clínico como terapéutico. CASO CLÍNICOPaciente de sexo femenino de 12 años previamente sana que consultó en otra institución por erosiones en la mucosa oral, que fueron interpretadas como de causa herpética y recibió aciclovir por 2 meses sin mejoría.Consultó en nuestro Hospital por la progresión de las lesiones a la piel y refirió un descenso de 5 kg de peso debido a odinofagia. Al momento del examen físico, se observaban erosiones en la mucosa oral y genital intensamente dolorosas; en la piel, ampollas fláccidas con contenido seroso, erosiones y costras distribuidas en forma generalizada con predominio en la cara, el tronco, la región periumbilical, los pliegues inguinales y el dorso (Figuras 1, 2 y 3). El signo de Nikolsky era positivo. Con los diagnósticos presuntivos de: pénfigo paraneoplásico, vulgar, penfigoide ampollar, dermatitis herpetiforme y enfermedad de Behçet, se realizó una biopsia cutánea, inmunofluorescencia directa (IFD) de piel sana perilesional e inmunofluorescencia indirecta (IFI). También se solicitaron exámenes complementarios de laboratorio e imágenes. Se decidió su internación para el diagnóstico y el tratamiento, y se solicitó la interconsulta con los servicios de Cuidados Paliativos y Nutrición para el manejo del dolor y la recuperación de su estado nutricional.En la histopatología, se evidenció epidermis con ampolla...

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Autoimmune Bullous Diseases

Beek

Schmidt

2019

Harper's Textbook of Pediatric Dermatology

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Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients

Cited by 30 publications

References 22 publications

Autoimmune bullous disease in childhood

Autoimmune bullous disease in childhood

Pénfigo vulgar de la infancia, a propósito de un caso

Autoimmune Bullous Diseases

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