Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infection (PANDAS): a Controversial Diagnosis.

Despite more than a decade of studying pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS), it is still not possible to confirm its existence and whether it is a poststreptococcal autoimmune disorder. Many controversies remain: the diagnostic criteria have not been validated, evidence of autoimmunity remains inconclusive, evidence of a genetic predisposition is weak, and streptococcal infections are common in childhood and could represent only a trigger of exacerbations of tics and obsessive-compulsive disorder. Patients who fit the PANDAS criteria appear to represent a subgroup of children with chronic tic disorder and/or obsessive-compulsive disorder who may experience symptom exacerbations after group A ß-hemolytic streptococci infections; however, those infections are not the sole or even the most common antecedent of exacerbations. There is not enough evidence to support PANDAS as a unique clinical entity.

Cutaneous vasculitis as a paraneoplastic syndrome in childhood.

INTRODUCTION: In children, vasculitis as a paraneoplastic syndrome has never been reported before. In this work we report a vasculitis syndrome as a neoplasm onset manifestation in a child and we discuss our case regarding the data from literature. CASE REPORT: A 7-year-old girl presented with hand and foot ulceration, fixed cyanosis and pallor. During investigation, a central nervous system (CNS) rhabdomyosarcoma with metastasis on multiple sites was diagnosed. DISCUSSION: Rhabdomyosarcomas represent 5 to 8% of child neoplasms, although the CNS seldom is the primary site. In the indexed English language literature there were no published cases of vasculitis associated with rhabdomyosarcoma as a paraneoplastic syndrome in childhood, which suggests that the described report is the first being published. Awareness of this possible coexistence could allow to an earlier diagnosis of neoplasms expressed by vasculitis, leading to an earlier treatment and a longer survival.

Edema hemorrágico agudo da infância: uma variante da púrpura de Henoch-Schõnlein? / Acute hemorrhagic edema of infancy: a variant of Henoch-Schõnlein purpura?

O edema hemorrágico agudo da infância (EHAI) é uma vasculite leucocitoclástica rara, com aproximadamente 100 casos descritos na literatura de língua inglesa. As lesões cutâneas características são púrpuras palpáveis, que se localizam em face, orelhas e extremidades, e lembram a figura de um medalhão. É uma vasculite de pequenos vasos, característica de crianças menores de dois anos de idade. Na maioria das vezes, tem curso autolimitado e benigno, apesar da aparência das lesões. Relatamos o caso de uma lactente, que iniciou edema de mãos e pés, lesões purpúricas na face e febre, e comparamos a outros já descritos, de acordo com a revisão da literatura acerca do assunto. A raridade da doença pode estar associada ao subdiagnóstico ou ao diagnóstico equivocado de púrpura de Henoch-Schõnlein (PHS). EHAI é precedido na maioria dos casos por infecções, imunizações ou drogas. O envolvimento de mucosas e vísceras raramente ocorre. Nenhum tratamento é recomendado atualmente. O alerta para essa vasculite tem como objetivo auxiliar o diagnóstico, tornando-o mais precoce, e evitar tratamentos e preocupações desnecessárias.

Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis and there are around 100 cases described in the English language literature. The typical cutaneous lesion is a palpable purpura localized on the face, ears, and extremities and resembles a medallion. It is a vasculitis of small vessels, mainly seen in children less than 2 years of age. It is usually self-limited and benign, despite its appearance. We report a case of an infant who presented with swelling of hands and feet, purpuric lesions on face and fever, and compare with other previously described cases, accordingly to the literature review. The rarity may be associated with underdiagnosis or mistaken diagnosis of Henoch-Schõnlein purpura. AHEI is preceded in the majority of cases by infections, immunizations or drugs. Mucosal and visceral involvement is seldom seen. No treatment is currently recommended. The alert for this vasculitis aims to help the diagnosis, making it earlier and preventing unnecessary concern and treatment.