A 13-year-old boy was referred with a 10-month history of an asymptomatic dark violaceous infiltrated nodular lesion on the left cheek (Figure 1). He had been previously treated with topical and systemic steroids with partial improvement. A skin biopsy revealed a dense dermal infiltrate of monomorphous, poorly differentiated medium-sized cells with blastic morphology (Figure 2A-B). Immunohistochemical staining revealed positive CD4, CD56, and CD123 cells that were negative for CD3, CD8, CD20, CD10, PAX5, TdT, LMP1, and MPO; Ki67 index was 40% (Figure 2C). The diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was made. Bone marrow (BM) biopsy and cerebrospinal fluid (CSF) examination were normal. Positron emission tomography-computed tomography (PET/CT) revealed hypermetabolic activity (SUVmax 4.68) localized to the skin. After careful risk-benefit deliberation with a multidisciplinary team, systemic treatment with a high-risk acute lymphoblastic leukemia (ALL) chemotherapy protocol was initiated (vincristine, dexamethasone, asparaginase, daunomycin, cytarabine, cyclophosphamide, and methotrexate). The patient is currently receiving treatment and has had good response without complications after 18 months' follow-up. How to cite this article: Rivas-Calderón MK, Cheirif-Wolosky O, Rosas-Romero ME, et al. Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management.
Background: Atopic dermatitis (AD) is children's most frequent chronic inflammatory skin disease. In most patients, this condition is controlled with topical treatments; however, some patients with severe AD do not respond to these treatments, requiring systemic therapy. There is insufficient information about the ideal dose, time of use, clinical response, and safety of systemic therapy in children with severe AD. This study described the clinical characteristics of patients with severe AD who required systemic treatment, drugs used, their clinical course, adverse effects, and associated complications. Methods: We conducted a retrospective review of the records of pediatric patients with severe AD treated in the Dermatology Clinic, Instituto Nacional de Pediatría (2000Pediatría ( to 2018, who required systemic treatment for more than 3 months. Results: We included 21 patients. The mean age at disease onset was 3.31 years. The drugs used were methotrexate (57.1%), thalidomide (38%), prednisone (42.8%), azathioprine (19%), mycophenolate mofetil (9.5%), cyclosporine (4.7%), and systemic steroids as bridging therapy (42.8%). Adverse effects were mild and were observed in two patients (9.5%) treated with methotrexate and mycophenolate mofetil. Conclusions: Methotrexate was the most frequently used drug in > 50% of the patients, and most patients attained remission. Cyclosporine, azathioprine, and mycophenolate mofetil were also effective. Side effects were mild and infrequent. Comparative studies of systemic treatments for severe AD in the pediatric population are necessary.
Los hemagiomas infantiles (HI) son los tumores de tejidos blandos más frecuentes de la infancia. Se caracterizan por un crecimiento significativo durante los primeros meses de vida, seguido de una involución lenta y espontánea a lo largo de un periodo que puede durar algunos años. Usualmente, la regresión de la mayor parte del tumor termina a los 4 años de edad. Sin embargo, algunos de los HI desarrollan complicaciones, lo que resulta en alteraciones funcionales, dolor y desfiguramiento. La decisión de administrar tratamiento a un paciente con HI y elegir la mejor opción terapéutica para ese paciente (tratamiento tópico o sistémico) debe ser individualizada, dependiendo de varios factores: el tamaño de la lesión, la localización, la presencia de complicaciones como ulceración, el riesgo de cicatrización o desfiguramiento, la edad del paciente, la tasa de crecimiento o de involución al momento del diagnóstico, los riesgos y beneficios de administrar el tratamiento, la disponibilidad del medicamento, los costos y la experiencia del médico tratante.
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