Edema hemorrágico agudo da infância: uma vasculite com bom prognóstico / Acute hemorrhagic edema of infancy: a vasculitis with good prognosis

OBJETIVOS: Relatar dois casos de edema hemorrágico agudo da infância, que consiste numa vasculite rara, caracterizada por lesões cutâneas purpúricas e edema periférico, sem envolvimento sistêmico (excetuando- se a febre), que tem início súbito, curso benigno e evolui espontaneamente para a cura. DESCRIÇÃO DOS CASOS: Os autores apresentam dois casos de crianças do sexo masculino, com 11 e 12 meses de idade, que foram atendidos no serviço de urgência por febre e lesões purpúricas exuberantes de agravamento progressivo. O diagnóstico de edema hemorrágico agudo da infância foi feito por exclusão e sustentado pelo fato de que, apesar da impressionante apresentação cutânea, ambos os lactentes apresentavam um ótimo estado geral e a tríade clássica desta entidade: febre, edema e lesões purpúricas da face, orelhas e extremidades. Houve regressão total das lesões cutâneas em aproximadamente uma semana, sem sequelas em ambos os casos. CONCLUSÕES: Existem pouco mais de 100 casos publicados mundialmente de edema hemorrágico agudo da infância. A raridade dessa vasculite pode dever-se a um subdiagnóstico ou diagnóstico equivocado de outras vasculites leucocitoclásticas, principalmente a Púrpura de HenochSchönlein. Além desta, existem outras doenças a considerar no diagnóstico diferencial, como meningococemia, doença de Kawasaki e eritema multiforme, que apresentam similaridades, mas ao mesmo tempo características distintas que permitem excluí-las. É fundamental o diagnóstico oportuno do edema hemorrágico agudo da infância, de modo a evitar exames complementares e terapêuticas desnecessárias, além de tranquilizar a família quanto ao bom prognóstico da doença.

AIMS: To report two cases of acute hemorrhagic edema of infancy, a rare vasculitis characterized by purpuric skin lesions and peripheral edema without systemic involvement (excluding fever), which has a sudden onset and an usually benign course, with spontaneous resolution. CASES DESCRIPTION: The authors describe two cases of male infants, 11 and 12 months old, who were admitted to the emergency department with fever and progressively worsening purpuric lesions. The diagnosis of acute hemorrhagic edema of infancy was made by exclusion and sustained by the fact that, despite the impressive skin presentation, both infants had a good general state and presented the classic triad of this entity: fever, peripheral edema, and purpuric lesions on the face, ears and extremities. Both boys presented total regression of the lesions in about one week, without sequelae. CONCLUSIONS: There are just over 100 cases of hemorrhagic edema of infancy reported worldwide. The rarity of this vasculitis may be due to underdiagnosis or mistaken diagnosis of other leukocytoclastic vasculitis, mostly Henoch-Schönlein purpura. In addition to this, there are other conditions to consider in the differential diagnosis, such as meningococcemia, Kawasaki disease and erythema multiforme, which have similarities, but at the same time distinctive features that allow to exclude them. Timely diagnosis of acute hemorrhagic edema of childhood is crucial to avoid unnecessary therapies and supplementary tests, as well as to reassure the family about the good prognosis of the disease.

Acute hemorrhagic edema of infancy: A report of two cases.

Acute hemorrhagic edema of infancy is one of the clinical forms of leukocytoclastic vasculitis seen in children. The condition runs a benign course. We report two male children who presented with upper respiratory tract infection followed by limb swelling and purpuric and ecchymotic lesions on the skin. Skin biopsy revealed leukocytoclastic vasculitis in both patients. In both cases, the lesions resolved completely without any sequelae.

Edema agudo hemorrágico de la infancia en una niña mayor; una entidad diferente de la púrpura de Henoch-Shönlein / Infantile acute hemorrhagic edema in a female child: a different entity from Henoch-Shönlein purpura

Introducción. El edema agudo hemorrágico de la infancia (EAHI) es una entidad nosológica que se manifiesta en niños pequeños, es autolimitada y de curso benigno. Usualmente su manifestación es secundaria y existe el antecedente de infección de vías aéreas superiores, administración de antibióticos o aplicación de vacunas. Las dos manifestaciones cutáneas principales son lesiones purpúricas en roseta o cocarda y anulares o en tiro al blanco, que se encuentran de manera primaria en cara y extremidades superiores, además hay edema en cara y extremidades. Caso clínico. Se informa el caso de un preescolar femenino de 4 años y 11 meses de edad con manifestaciones clínicas clásicas de esta entidad, pero que se presentó en edad tardía. Conclusión. Se refuerza el concepto de que la púrpura de Henoch-Schönlein y el EAHI son entidades diferentes.

Introduction. Infantile acute hemorrhagic edema (IAHE) is an entity manifested in young children and has a self-limiting and benign course. It usually appears secondary to a history of upper respiratory illness, course of antibiotics or vaccination. The two primary cutaneous features include large "cockade" or rosette appearance or annular purpuric lesions found primarily on the face and upper extremities along with edema of the limbs and face. Case report. We report the case of a female patient (age 4 years and 11 months) who manifested all the classic clinical characteristics of this entity at an older age. Conclusion. The concept that Henoch-Schönlein purpura and acute hemorrhagic edema of infancy are different entities is reinforced.

Edema hemorrágico agudo del lactante: a propósito de un caso / Acute hemorrhagic edema of the infant: report of a case

El edema hemorrágico agudo del lactante consiste en una vasculitis leucocitoclástica de vasos pequeños. Las manifestaciones clínicas son llamativas por el intenso púrpura asociado a edema que se desarrolla en el plazo de uno o dos días en zona facial y extremidades. Sin embargo, el diagnóstico es clínico y generalmente no requiere tratamiento, ya que su curso es benigno y autolimitado: presentamos una revisión del tema a raíz de un caso diagnosticado en un niño de seis meses (AU)

Acute hemorrhagic edema of infancy is a small vessel leucocytoclastic vasculitis. The clinical manifestations are dramatic, because the intense purpuric lesions associated with edema develop in one or two days on the face and limbs. However, the diagnosis is based on the history and physical examination and it generally does not require treatment, because of its benign and limited course.We write this review after seeing a case we in a six months old baby (AU)

Edema agudo hemorrágico del lactante: reporte de un caso / Acute hemorrhagic edema of infants: case report

El edema agudo hemorrágico del lactante es una vasculitis aguda leucocitoclástica de pequeños vasos, de curso benigno, autolimitado, que afecta sobre todo a niños menores de dos años de edad. Se caracteriza por lesiones cutáneas con morfología anular o en escarapela, en cara y extremidades, además de edema de las zonas afectadas, sin embargo se conserva el buen estado general de los pacientes. Se consideran como probables desencadenantes las infecciones y la administración de ciertos medicamentos, incluso vacunaciones. Se presenta el caso de un lactante de 18 meses de edad que inicia su cuadro en forma súbita luego de un proceso infeccioso de vías aéreas superiores (AU)

Acute hemorrhagic edema of infancy is a benign variant of leukocytoclastic vasculitis, self-limited condition that affects mainly children under 2 years old. It is characterized by cutaneous lesions with a cockade or target-like pattern, located on the face and limbs, and a variable degree of edema in affected areas; however the general health status is generally preserved.We describe the case of an 18 month old boy whose clinical picture started soon after an upper respiratory tract infection (AU)

A Case of Acute Hemorrhagic Edema of Childhood / 대한피부과학회지

Acute hemorrhagic edema of childhood or infancy (AHE) is a benign form of leukocytoclastic vasculitis with many similarities to Henoch-schonlein purpura (HSP). It occurs in children younger than 2 years and is characterized by abrupt onset of inflammatory edema and ecchymotic purpura in a target or cockade pattern, that resolve spontaneously within 3 weeks. AHE had been considered by some as a variant of HSP which shares certain clinicopathologic features. But most authors have regarded it as an entity distinct from HSP in view of the younger age of patients, lack of visceral involvement, distinctiveness of cutaneous lesions, absence of perivascular IgA on immunofluorescence, and excellent prognosis. We report a typical case of AHE presenting with a cockade or target-like purpura and edema on the face and acral areas with excellent prognosis in a 13-month-old boy.

Acute hemorrhagic edema in an infant mimicking Henoch-Schonlein purpura: a case study / 소아과

Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings of Henoch-Schonlein purpura (HSP). The skin lesions heal spontaneously within one to three weeks and internal organs are rarely affected. We report a case of AHEI occurring in a 23-month-old boy who was initially misdiagnosed as HSP, and was later diagnosed according to his clinical symptoms and histochemical characteristics.

A Case of Acute Hemorrhagic Edema of Childhood Accompanied by Varicella / 대한피부과학회지

Acute hemorrhagic edema of childhood (AHEC) is a distinctive entity among the group of the leukocytoclastic vasculitides in childhood. AHEC is characterized by ecchymotic patches in a cockade pattern and a tender inflammatory edema on the face or upper extremities in children younger than 3 years old. It is usually confined to the skin and runs a benign course. We report a case of AHEC preceded by varicella. A 3-year-old boy presented with a 4-day history of medallion-like purpuric patches on both arms and the abdomen, along with varicella lesions. The purpuric lesions resolved completely without any complication after one week, following the resolution of the varicella. AHEC is an immune complex-mediated vasculitis that can be preceded by infection, and varicella might be a triggering factor in this case.

A Case of Acute Hemorrhagic Edema of Infancy

Acute hemorrhagic edema of infancy is an unusual form of leukocytoclastic vasculitis occuring in children from the age 4 months to 2 years. The etiology remains unknown. Numerous studies, however, suggest acute hemorrhagic edema of infancy as an immune-mediated vasculitis in response to a variety of antigenic stimuli. We report a case of an acute hemorrhagic edema of infancy; 11-month-old boy with a history of fever for 3 days and a history of purpuric rash on the extremities, trunk, buttock and oral mucosa for 2 days.

A Case of Acute Hemorrhagic Edema of Childhood / 대한피부과학회지

Acute hemorrhagic edema of childhood is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in children younger than 3 years. This is characterized by tender edema and rosette-shaped purpuric patches that generally resolve without intervention. We describe a 26-month-old boy with acute hemorrhagic edema of childhood, in whom the disease appeared after an acute respiratory illness. Skin lesions presented with acral edema, and rosette-shaped purpuric plaques on the face and limbs. Some authors consider the disease as a purely cutaneous form of Henoch-Schoenlein purpura, but it is more likely that acute hemorrhagic edema of childhood could be regarded as a distinct entity within leukocytoclastic vasculitis.

A Case of Infantile Acute Hemorrhagic Edema

Infantile acute hemorrhagic edema(IAHE) is a variant of leukocytoclastic vasculitis which is characterized by painful edematous purpura of cockade pattern on the head and distal extremities. The lesions are limited to the skin and complete recovery occurs within 1 to 3 weeks. Recently, it is accepted as a separate entity from Henoch-Sch nlein purpura. A nine month-old boy presented multiple cockade ecchymotic plaques and inflammatory edema on the face and extremities. He had recent upper respiratory infection and was in good general condition except mild fever. His skin lesions markedly improved within 7 days of treatment. We report a typical case of IAHE with analysis of the domestic case reports and review of the literatures.

Infantile Acute Hemorrhagic Edema

We report a case of infantile acute hemorrhagic edema in a 15-month-old boy. The patient showed multiple, bean to walnut-sized, red to purple-colored, indurated purpura with a cockade pattern on the face, earlobes, and extremities. Edema of the hands, feet, and earlobes was also observed. Laboratory examinations showed thrombocytosis, decreased serum CH50, and ketone in the urine. A skin biopsy taken from indurated purpura on the lower leg exhibited findings of acute leukocytoclastic vasculitis. Direct immunofluorescence examination showed deposition of IgM along the dermo-epidermal junction and C3 in the dermal vessel walls. With-out specific management except for fluid therapy to correct dehydration, the skin lesions cleared completely in 2 weeks, and recurrence has not observed.

Infantile Acute Hemorrhagic Edema of the Skin / 대한피부과학회지

Infantile acute hemorrhagic edema of the skin(IAHE) is a benign disease which affects infants between 4 months and 2 years of age and is characterized by palpable ecchymotic purpura and edema on the limb and face. We report a typical case of IAHE, which was presenting a cockade, annular, reticulated, and iris-like purpura and edema on the face and extremities in a 19-month-old male infant. We consider it to be a new disease category because its characteristics different markedly from HenochSchoenlein purpura in several clinical and histopathologic findings.

A Case of Infantile Acute hemorrhagic Edema / 대한피부과학회지

Infantile acute hemorrhagic edema is a benign disease followed by a spontaneous eomplete recovery without sequelae, characterized by cockade purpura and inflamrntory edema on the face and extremities without any internal origin involvement in 4-month to 2-year-old infants. We report a case of infaniile acute hemorrhagic edema which occared in a 2-year-old female after an upper respiratory illness and compare the clinical and histologic features of this disorder with Henoch-Schonlein purpura.