Epidermolysis Bullosa Acquisita: A Rare Autoimmune Blistering Disease in Children.

A 7-year-old girl presented with a 2-year history of recurrent blisters on the skin and oral mucosa. The patient was otherwise healthy, and her family history was unremarkable for any dermatologic or other medical disease. Examination revealed multiple tense vesicles, milia, and atrophic scars present over the extensor surface of the extremities and erosions on the oral mucosa (Figure 1). A skin biopsy established a pauci-inflammatory subepidermal blister (Figure 2a). Direct immunofluorescence (DIF) evidenced the linear deposition of immunoglobulin G (IgG), immunoglobulin M (IgM), and κ and λ chains at the dermal-epithelial junction (DEJ). Indirect immunofluorescence (IIF), using the salt-split technique, established anti-epithelial antibodies on the dermal side (Figure 2b). An enzyme-linked immunosorbent assay (ELISA) was positive for Collagen Type VII (COL7) antibodies. A diagnosis of epidermolysis bullosa acquisita (EBA) was made, and treatment with azathioprine and deflazacort was administered for 8 months with progressive lessening of her symptomatology and complete clinical response at 2-year follow-up. (SKINmed. 2022;20:460-462).

Bronchiolitis Obliterans With Anti-Epiplakin Antibodies in a Boy With Paraneoplastic Pemphigus.

Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by a recalcitrant and severe mucositis, and polymorphic cutaneous lesions, associated with benign and malignant neoplasms. Paraneoplastic pemphigus is caused by production of autoantibodies against various epidermal proteins involved in cell adhesion. Bronchiolitis obliterans (BO) is one of the leading causes of mortality in these patients. Recent advances have associated the presence of anti-epiplakin antibodies with the development of BO in adult patients. Here we describe the first pediatric patient in whom the association of anti-epiplakin antibodies and BO have been reported so far.

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy of the skin and hematopoietic system. There are few pediatric cases reported in the literature. Management of primary cutaneous BPDCN is challenging because, despite an apparently indolent clinical presentation, rapid dissemination with high mortality can occur. We describe a child with isolated cutaneous involvement who had a good response to chemotherapy as first-line treatment of BPDCN.

Frequency and findings of the acquired anorectal disease in the pediatric population with chronic constipation.

Sanchez-Avila MT, Garcia-Valencia OA, Rivas-Calderon M, Morales-Garza LA, Jacobo-Velazquez P, Chavez-Caraza KL. Frequency and findings of the acquired anorectal disease in the pediatric population with chronic constipation. Turk J Pediatr 2018; 60: 547-553. To determine the frequency of anorectal disease associated with chronic constipation in children we conducted an observational, descriptive, retrospective study. One hundred and sixty eight patients were evaluated by anorectal manometry over a 10-year period. The population was divided into organic constipation (OC) and functional constipation (FC) per ROME III criteria. Of those: 95 (56.54%) presented with anorectal disease. The age range was 1-17 (mean 5.3). Eighteen presented with OC and 77 with FC. Acute anal fissure was found in 45 patients (38 FC, 7 OC), chronic anal fissure in 38 (30 FC, 8 OC), perianal erythema in 26 (23 FC, 3 OC), scar tissue was found in 28 (24 FC, 4 OC), anal mucosa congestion in 27 (22 FC, 5 OC), hemorrhoidal disease in 26 (20 FC, 6 OC) and perianal venous dilatation in 21 (16 FC, 5 OC). Anal fissure, perianal erythema, and venous dilation occurred more frequently in patients with an anterior ectopic anus. An anal fissure was present in more than 80% of patients who had puborectalis muscle and external anal sphincter dysfunction. We concluded that the frequency of acquired anorectal disease in children with chronic constipation is high; early diagnosis and treatment are priorities for the successful management of these patients.

Modificación de la conducta y alimentación balanceada en niños con déficit de atención y desorden de hiperactividad / Conduct modification and balanced diet in children with attention deficit hyperactivity disorder

La relación entre conducta hiperactiva y dieta ha sido estudiada en el pasado por Feingold, sin embargo en Venezuela hay poca información. El objetivo de esta investigación fue evaluar el efecto del Reforzamiento de Conductas Incompatibles (RDO) y alimentación balanceada en el control de las conductas de los Trastorno por Déficit de Atención e Hiperactividad (TDAH). El estudio se realizó en seis niños con edades entre 6 y 9 años con el diagnòstico de TDAH . Se utilizó un diseño cuasi-experimental de comparación estática, intrasujeto, combinado con un diseño balanceado conductual de forma A - B - C: Fase A: línea base (Grupo 1 y 2), Fase B: Alimentación balanceada (Grupo 1) y RDO (Grupo 2); Fase C: Alimentación Balanceada + RDO (Grupo 1) y RDO + alimentación balanceada (Grupo 2). Los resultados permitieron evidenciar que ambos tratamientos (RDO y alimentación balanceada) lograron disminuir las conductas de TDAH en el hogar, el RDO ocasionó las disminuciones más inmediatas y estables de las conductas de TDAH en comparación con la alimentación balanceada, ante la cual los cambios fueron más lentos y la sumatoria de los tratamientos ocasionó las reducciones más evidentes de las conductas. La alimentación balanceada parece ser una herramienta de intervención clínica efectiva para controlar el TDAH. Es necesario más investigaciones que incorporen como parte fundamental del tratamiento a la dieta, en muestras más representativas y con mejor control de otras variables