Acute Hemorrhagic Edema Of Infancy Research Articles

Accurate diagnosis of acute hemorrhagic edema of infancy: a French multicenter observational study.

The purpose of the study is to highlight clinical signs that are either suggestive of or against the diagnosis of AHEI to improve diagnosis and management. The medical records of children under 3years old diagnosed with AHEI were retrospectively reviewed. Clinical data and photographs were reviewed by three independent experts, and the cases were classified as probable, doubtful, or unclear AHEI. Of the 69 cases of children diagnosed with AHEI included in 22 centers, 40 were classified as probable, 22 as doubtful, and 7 as unclear. The median age of patients with probable AHEI was 11months [IQR 9-15], and they were in overall good condition (n = 33/40, 82.5%). The morphology of the purpura was targetoid in 75% of cases (n = 30/40) and ecchymotic in 70% of cases (n = 28/40) and affected mostly the legs (n = 39/40, 97%), the arms (n = 34/40, 85%), and the face (n = 33/40, 82.5%). Edema was observed in 95% of cases and affected mostly the hands (n = 36/38, 95%) and feet (n = 28/38, 74%). Pruritus was absent in all patients with probable AHEI and described for 6/21 with doubtful AHEI (29%). AHEI was the original diagnosis in only 24 patients (n = 24/40, 60%). The major differential diagnoses were purpura fulminans and urticaria multiforme. Conclusion: AHEI, which the diagnosis is made on clinical findings, is often misdiagnosed. Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in a young child with a good overall condition are highly suggestive of AHEI. What is Known: •Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis affecting children under 3 years old. •Appropriate diagnosis is important to distinguish this benign disease from more serious diseases to avoid investigations and treatments, iatrogenic harm and unnecessary follow-up. What is New: •AHEI is an uncommon disorder often misdiagnosed by pediatricians and dermatologists. •Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in an infant with a good overall condition are highly suggestive of AHEI.

Acute Hemorrhagic Edema of Infancy - Case Report

Abstract Acute hemorrhagic edema of infancy (AHEI) is a rare vasculitis of small dermal vessels with characteristic presentation in infants aged up to 24 months. It manifests as a sudden occurrence of palpable purpuric skin lesions, swellings in hands, feet, face and auricles, and mild fever. The children affected with AHEI are almost always in good general health and with normal laboratory parameters. Approximately 400 cases have been described in the literature so far. However, the etiology is still unknown. Most evidence suggests infections or vaccination as the principal triggers. Extra cutaneous manifestations are recorded in only about 10% of AHEI patients. The majority of the affected children undergoes recovery spontaneously and without any complications within 1-3 weeks, with or without any treatment. AHEI is usually diagnosed on the clinical grounds only and the diagnostic procedure rarely requires a skin biopsy. The current literature indicates the use of corticosteroids and/or antihistamines as a therapy, but there is still a lot of controversy about these therapeutic measures. This paper presents the case of AHEI with its typical clinical manifestations that resolved in a rapid spontaneous recovery without the use of any treatment within a week. AHEI is a rare syndrome that pediatricians should be well familiar with in order to differentiate it from other potentially severe diseases that have similar cutaneous manifestations, but also to avoid unnecessary investigations and therapy.

Acute Hemorrhagic Edema of Infancy Associated With Coronavirus Disease 2019

Acute Hemorrhagic Edema of Infancy Associated With Coronavirus Disease 2019

Acute hemorrhagic edema of infancy (AHEI): Alarming cutaneous presentation of a benign and self‐limited disease

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis. We report on an 11-month-old boy, presenting the classical feature of AHEI with alarming cutaneous presentation, but good clinical condition. Early recognition is crucial to avoid unnecessary medical investigations or therapies, as well as to identify potentially severe complications.

Acute Hemorrhagic Edema following COVID-19 Infection

Background: Acute hemorrhagic edema of infancy (AHEI) is an immune complex-mediated leukocytoclastic vasculitis. Despite its worrisome appearance, it has a benign disease course with rare systemic involvement. Clinical Description: A 25-month-old male patient was brought to the pediatric outpatient clinic with a pink oval-shaped plaque-like rash all over the body and edema on the lower extremities and left auricle. The child was diagnosed as a case of coronavirus disease 2019 (COVID-19) 5 days before the present complaints. History and physical examination were otherwise unremarkable. Because of the patient's age, the purpuric appearance of lesions, distribution pattern of the rashes, localized edema, and no end-organ involvement, a possibility of AHEI, triggered by COVID-19, was considered and the patient was evaluated for the same. Management and Conclusions: The patient recovered in 15 days with no end-organ involvement. He was advised to continue regular follow-ups to look for long-term complications. AHEI is a benign condition which may occur in children following COVID-19 infection. It is essential to recognize the condition to avoid unnecessary investigations and treatment.

Dermatologic Manifestations of Systemic Diseases in Childhood

Dermatologic Manifestations of Systemic Diseases in Childhood

Acute Hemorrhagic Edema of Infancy With Associated Hemorrhagic Lacrimation: Erratum.

Acute Hemorrhagic Edema of Infancy With Associated Hemorrhagic Lacrimation: Erratum.

Acute Hemorrhagic Infancy of Edema: A Purpuric Rash in 6-Month-Old Infant

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis presenting with purpura, ecchymosis, fever, and edema. Pediatricians must effectively differentiate AHEI from other diseases that have similar presentations but are more severe in order to treat appropriately without overutilizing expensive, unnecessary diagnostic tests. In this article, we describe a case of a 6-month-old previously well male who presented to our institution with a worsening rash and fever. In this case, the patient’s age and benign clinical manifestations without systemic involvement favored the diagnosis of AHEI over more serious conditions. This case is a valuable example of the clinical findings of AHEI and the effectiveness of conservative therapy once a diagnosis is made for pediatricians, especially emergency and urgent care physicians.

Acute Hemorrhagic Edema of Infancy: A Case Series of Three Cases

Acute hemorrhagic edema of infancy (AHEI) is a poorly known benign vasculitis. With a rapid course, self-limited evolution, and without specific diagnostic tests, it is poorly diagnosed. It has differential diagnosis with Henoch–Schonlein’s purpura, multiform erythema, Kawasaki’s incomplete disease, or urticaria. We present a case series of three cases of children with a clinical diagnosis of AHEI, and in two of them, a biopsy was performed which confirmed the diagnosis. The three children have a maculopapular purpuric eruption, fever, and edema at the extremities. They were treated with symptomatic medications and showed improvement in a few days. AHEI is a rare vasculitis, sometimes underdiagnosed, due to the similarity with other differential diagnoses, much more prevalent. As it is not frequently described, and due to the exuberance of symptoms, it is a disease susceptible to diagnostic errors and iatrogenic treatments.

Clinical characteristics of 10 children with a pediatric inflammatory multisystem syndrome associated with COVID-19 in Iran

BackgroundAlthough symptoms and signs of COVID-19 (Coronavirus disease 2019) in children are milder than adults, there are reports of more severe cases which were defined as pediatric inflammatory multisystem syndrome (PIMS). The purpose of this report was to describe the possible association between COVID-19 and PIMS in children.MethodsFrom 28 March to 24 June 2020, 10 febrile children were admitted with COVID-19 infection showing characteristics of PIMS in Buali tertiary hospital of Sari, in Mazandaran province, northern Iran. Demographic and clinical characteristics, laboratory and imaging findings, and therapeutic modalities were recorded and analyzed.ResultsThe mean age of the patients was 5.37 ± 3.9 years (13 months to 12 years). Six of them were boys. Kawasaki disease, myocarditis, toxic shock syndrome, appendicitis, sepsis, urosepsis, prolonged febrile seizure, acute hemorrhagic edema of infancy, and COVID-19-related pneumonia were their first presentation. All of them had increased C-reactive protein levels, and most of them had elevated erythrocyte sedimentation rate, lymphopenia, anemia, and hypoalbuminemia. Three of them had thrombocytopenia(PLT < 106). Six of them were serologically or polymerase chain reaction positive for COVID-19, and 4 of them were diagnosed as COVID-19 just by chest computed tomography scan. Most of the patients improved without a residual sequel, except one who died with multiorgan failure and another case was discharged with a giant coronary aneurysm.ConclusionsChildren with COVID-19 may present symptoms similar to Kawasaki disease and inflammatory syndromes. PIMS should be considered in children with fever, rash, seizure, cough, tachypnea, and gastrointestinal symptoms such as vomiting, diarrhea, and abdominal pain.

Mycoplasma pneumonia and atypical acute hemorrhagic edema of infancy

Acute hemorrhagic edema of infancy is a benign rare presentation of leukocytoclastic vasculitis that affects children between 4 and 24months of age. It usually involves the distal extremities, face, and ears. We report an atypical presentation of AHEI in a 1year 5months old boy starting initially over the trunk and back, then spreading to the face and extremities. Mycoplasma pneumonia IgM was found to be positive. The rash resolved spontaneously within two weeks. Herein we present a case of Mycoplasma induced AHEI with an atypical clinical presentation followed by a review of the literature.

Approaching Target and Targetoid Eruptions in Inpatient Dermatology

To summarize the differential diagnosis and describe a clinical approach for the evaluation of target and targetoid lesions in hospitalized patients. Important diagnostic considerations for target and targetoid lesions in an inpatient setting include erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis, mycoplasma-associated rash and mucositis, fixed drug eruption, urticaria multiforme, subacute cutaneous lupus erythematosus, and Rowell syndrome. Less frequent diagnoses may include erythema migrans, arthropod bite, erythema gyratum repens, erythema annulare centrifugum, tinea concentricum, tinea corporis, Granuloma annulare, elastolytic giant cell granuloma, cutaneous T cell lymphoma, linear IgA syndrome, pigmented purpuric dermatosis of Majocchi, hobnail hemangioma, and acute hemorrhagic edema of infancy. Morphology can be a key in initially discriminating among these entities. Classic target lesions should be distinguished from targetoid, gyrate, and annular lesions. The differential diagnosis of target and targetoid lesions in hospitalized patients is broad; however, attention to additional morphologic features, location, concomitant symptoms, mucosal involvement, histopathology, location, and progression can aid in diagnosis. Clinical evaluation should also consider patient demographics and associated systemic symptoms to elucidate the correct diagnosis.

Imaging clinical case

Case report: The authors present the case of a 15-year-old adolescent boy with occipital spur noticed since the previous year and with progressive enlargement. No history of trauma or other associated symptoms was evident. Physical examination showed a hard tumefaction with no inflammatory signs and x-ray revealed occipital hyperostosis. The boy spent most free time playing with mobile gadgets, with poor body posture.Discussion/Conclusion: Although considered a normal feature, occipital spur in adolescents and young adults may be linked to poor posture, for instance associated with prolonged use of handheld technology. Exaggerated technology use may be associated with several complaints and musculoskeletal symptoms should not be neglected.

Acute Hemorrhagic Edema of Infancy: A Diagnostic Challenge for the General Pediatrician.

Acute hemorrhagic edema of infancy (AHEI), a benign and self-limited disease, can be easily mistaken to be a number of diseases with similar dermatological manifestations but with potentially adverse outcomes. This review aimed to familiarize pediatricians with the natural history, clinical manifestations, diagnosis, and management of AHEI. A PubMed search was conducted in February 2020 in Clinical Queries using the key terms "acute hemorrhagic edema of infancy" OR "Finkelstein disease" OR "Seidlmayer disease". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. AHEI, a rare cutaneous leukocytoclastic small-vessel vasculitis, typically presents with palpable purpura, peripheral acral edema, and frequently with fever, most often in children between 4 and 24 months of age. A significant number of children experience prodromal symptoms of an upper respiratory infection. Fever is typically low grade and is present in approximately 50% of cases. The cutaneous lesions are characterized by rapid onset of small erythematous macules or papules that progress to well demarcated, annular, rosette, medallion-like, or targetoid purpuric plaques or ecchymosis in 24 to 48 hours. The skin lesions are typically palpable, nonpruritic, and symmetrically distributed. Sites of predilection include the face, auricles, and extremities. Edema is typically nonpitting and asymmetrical and occurs primarily on the dorsum of the hands and feet, the face, and the auricles. In spite of the acuteness and extent of the cutaneous findings, the child looks well and nontoxic. Systemic and/or visceral involvement are rare. The differential diagnosis is broad and includes, among others, Henoch-Schönlein purpura. It is crucial to distinguish AHEI from the other diseases since the management of these diseases is quite different. The clinical features of mimickers of AHEI are reviewed and clues to differentiate AHEI from these mimickers are highlighted..AHEI is a benign, self-limited disease with complete spontaneous recovery in one to three weeks in the majority of cases. Recognizing this rare disease is important for the pediatrician to rapidly differentiate AHEI from other potentially serious diseases that require prompt therapy and monitoring. With rapid recognition of AHEI, unnecessary investigations and inappropriate interventions can be prevented and parental anxiety can be avoided.

A case of acute hemorrhagic edema of infancy

A case of acute hemorrhagic edema of infancy

A rare vasculitis with a dramatic presentation: Acute hemorrhagic edema of infancy

Acute hemorrhagic edema of infancy is a disease marked by its dramatic presentation of purpuric plaques in a cockade or targetoid pattern. A 1-year-old boy was brought by his mother to our outpatient department with large purpuric plaques in annular and cockade pattern over the cheeks and bilateral lower limbs. Both the pinna showed purpura in a peculiar reticulate pattern, with vesiculation seen at the margin of the lesions along with mild edema. The skin biopsy showed cutaneous small-vessel vasculitis; direct immunofluorescence showed fibrinogen deposits around the small cutaneous vessels. The unusual features of our case were the reticulate pattern of purpura seen over the ears.

Acute Hemorrhagic Edema of Infancy: A Diagnostic Challenge for the General Pediatrician

Acute Hemorrhagic Edema of Infancy: A Diagnostic Challenge for the General Pediatrician

Role of systemic steroids in acute hemorrhagic edema of infancy: report of two cases

Acute hemorrhagic edema of infancy (AHEI) is an unusual form of leukocytoclastic vasculitis with dramatic distinguishing skin lesions that occurs in infants who age 4 to 24 months old. Clinical presentation at onset requires clinical and, less often, histological evaluation to distinguish it from more serious diseases and other vasculitis. Treatment of AHEI remains controversial; and since it is a self-limiting disease, a conservative approach should be considered first. Although some authors have reported that the use of steroids may be beneficial if started early on, we think their use, based on our presented cases here, may accelerate the healing and improves the color of preexisting skin lesions but does not significantly alter the course of the disease. Authors reported here two clinical Cases of AHEI (Diagnosed based on characteristic skin lesions and confirmed with Histological evaluation) who were treated initially with systemic steroids, besides we reviewed the role of systemic steroids in the treatment of AHEI.

Acute hemorrhagic edema of infancy

In infants less than 2 years of age, acute hemorrhagic edema of infancy (AHEI) is characterized by the rapid onset of annular purpuric lesions associated with initially localized edemas affecting the extremities. This disorder is usually benign, without visceral involvement. Diagnosis is based on clinical examination, no specific laboratory findings exist and the histological examination of the lesions (which is unnecessary in the majority of cases) is most often nonspecific (sometimes patients have nonspecific leukocytoclastic vasculitis). Nosological status of AHEI remains unclear, but some reports suggest it is a clinical manifestation of rheumatoid purpura. Rhinopharyngeal episodes in the days before its onset have sometimes been reported, suggesting a viral cause. There is a net mismatch between patient's good general condition and the remarkable dissemination of the lesions. Infants monitoring must be rigorous in the first days, even though complications are exceptional (acute intussusception). Patients recover spontaneously within 12 days. Therapeutic management is based on accurate monitoring of the infant's general status. Fever, the extension of the purpuric lesions and, in particular, signs of impaired general condition may suggest the diagnosis of purpura fulminans. Another differential diagnosis is acute hemorrhagic or ecchymotic urticaria. We here report the case of a 3-month old infant with diffuse pseudoannular lesions within a context of apyrexy and preservation of patient's general condition. Patient's outcome was favorable without any treatment, confirming the diagnosis of AHEI.

Acute hemorrhagic edema of infancy

In infants less than 2 years of age, acute hemorrhagic edema of infancy (AHEI) is characterized by the rapid onset of purpuric annular lesions associated with initially local edemas of the extremities. In most cases, this disorder is benign, without visceral involvement and diagnosis is based on clinical examination. There are no specific laboratory data and the histological analysis of these lesions (which is unnecessary in the majority of cases) is usually unspecific (the patient sometimes has an unspecified leucocytoclasic vasculitis). This condition is of uncertain nosologic status, mimiking rheumatoid purpura. Sometimes the patient had had rhino-pharyngeal episode in previous days, suggesting a viral cause. There is a clear discrepancy between the good general condition and the profuse and spectacular appearance of the lesions. Children's monitoring in the first days must be rigorous but complications are exceptional (acute intussusception'). Patient's outcome is marked by spontaneous regression within 12 days. Treatment is based on careful monitoring of the child's general status. Fever, purpuric lesions extension and, especially, signs of alteration of the general state may suggest the diagnosis of purpura fulminans. Another differential diagnosis is acute hemorrhagic or ecchymotic urticaria. We report the case of a 3-month old infant with diffuse pseudo-roundel like lesions in a context of apyrexy and preservation of the general state. Patient's outcome was favorable, without receiving any treatment. This confirm the diagnosis of AHEI.