RESUMO
ABSTRACT Objective: To report a rate case of Juvenile xanthogranuloma in a newborn infant. Case description: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. Comments: Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.
RESUMO Objetivo: Descrever um caso raro de xantogranuloma juvenil em recém-nascido. Descrição do caso: Apresentamos o caso de um recém-nascido pré-termo de 31 semanas com múltiplas lesões cutâneas cuja clínica, histologia e imuno-histoquímica permitiram o diagnóstico de xantogranuloma juvenil. Atualmente, com nove meses de idade, não apresenta agravamento das lesões nem evidência de envolvimento extracutâneo, nomeadamente oftálmico. Comentários: O xantogranuloma juvenil é uma patologia rara e benigna, pertencente ao vasto grupo das histiocitoses não Langerhans. Surge tipicamente em idade pediátrica, podendo ter apresentação neonatal. O envolvimento é predominantemente cutâneo sob a forma de pápulas ou nódulos de coloração amarela e/ou eritematosos, assintomáticos, solitários ou múltiplos. O envolvimento extracutâneo é mais frequente em crianças com menos de dois anos e com múltiplas lesões, sendo o olho o local mais afetado. Destacamos este caso clínico pela apresentação no período neonatal e sob a forma de múltiplas lesões, o que lhe confere risco acrescido de envolvimento extracutâneo, sem que, no entanto, tal se tenha verificado.
Assuntos
Humanos , Masculino , Lactente , Diagnóstico Diferencial , Biópsia/métodos , Imuno-Histoquímica , Idade Gestacional , Xantogranuloma Juvenil/imunologia , Xantogranuloma Juvenil/patologia , Assistência ao Paciente/métodosRESUMO
OBJECTIVE: To report a rate case of Juvenile xanthogranuloma in a newborn infant. CASE DESCRIPTION: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. COMMENTS: Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.
Assuntos
Xantogranuloma Juvenil , Biópsia/métodos , Diagnóstico Diferencial , Idade Gestacional , Humanos , Imuno-Histoquímica , Lactente , Masculino , Assistência ao Paciente/métodos , Xantogranuloma Juvenil/imunologia , Xantogranuloma Juvenil/patologiaRESUMO
Cutaneous plasmacytoma is an uncommon observation in clinical practice. It is usually a consequence of direct extension from an underlying bony lesion, in the setting of multiple myeloma. In our case, a 77-year-old woman, with stage IIIA IgG lambda multiple myeloma for two years, presented with firm nodular violaceous cutaneous lesions on the left arm without underlying bone osteolytic lesions or subcutaneous tumors; the biopsy was consistent with plasmacytoma. The patient had suffered two spontaneous left humeral fractures treated with prosthesis replacement just before the initial diagnosis of multiple myeloma. She had also been submitted to radiotherapy for a subcutaneous plasmacytoma, detected some months before, at the same site of the cutaneous lesions. Despite optimal response of the cutaneous lesions to treatment, the disease progressed and the patient died from infectious complications eight months after the appearance of the tumors.
