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Ecchymotic Skin Lesions and Hand Edema in a 10-month-old Boy
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Background:
Acute hemorrhagic edema is a skin-limited small-vessel leukocytoclastic vasculitis, which affects infants 4 weeks to 2 years of age and remits within 3 weeks. The diagnosis is made clinically in not-ill appearing children with acute onset of raised annular or nummular eruptions and edema. In this vasculitis, type, distribution, and evolution of the rash have never been systemically investigated. To address this issue, we employed the data contained in the Acute Hemorrhagic Edema Bibliographic Database, which incorporates all reports on acute hemorrhagic edema.
Summary:
Key features of rash were documented in 383 children. Annular eruptions in a strict sense, usually targetoid, were reported in 375 (98%) cases (many children also presented polycyclic or arciform eruptions). Nummular eruptions were also very common (n = 358; 93%). Purpuric eruptions and ecchymoses were reported in the vast majority of cases. Macules and wheals were described in a minority of cases. Edema, detected in all cases, was mostly painful, indurated and nonpitting. The following regions were affected, in decreasing order, by annular or nummular eruptions: legs, feet, face, arms, ears, trunk, and genitals. With the exception of feet, which were very often affected, the same distribution was reported for edema. The initial eruption was often a wheal or a macule that evolved into a nummular or an annular eruption. Nummular eruptions successively evolved into annular ones. Key Message: This study carefully characterizes type, distribution, and evolution of skin eruption in acute hemorrhagic edema. The data help physicians to rapidly and noninvasively make the clinical diagnosis of this vasculitis.
Conclusion:
This is the first prospective evaluation of hemorrhagic edema. Our findings emphasize its distinctive tetrad: a well-appearing child; targetoid lesions that do not change location within hours; non-pitting, sometimes tender edema; complete resolution without recurrence. What is known • Acute hemorrhagic edema of young children is considered a benign vasculitis. • There have been ≈100 cases reported in small retrospective case series. What is new • The first prospective evaluation of this condition emphasizes its features: febrile prodrome; well-appearing child; targetoid lesions not changing location within hours; non-pitting, sometimes tender indurative edema; absent extracutaneous involvement; resolution within 3 weeks. • Antineutrophil cytoplasmic autoantibodies do not play a pathogenic role.
The autoinflammatory diseases are a heterogeneous group of disorders characterized by early appearance of symptoms and characteristic flares of fever plus signs of internal inflammation, including various skin manifestations. We describe a 2.5‐year‐old boy who presented with fever, purpuric skin lesions, and edema, the classic clinical triad of acute hemorrhagic edema of infancy (AHEI). Although AHEI was our initial leading diagnosis, additional history revealed that these episodes were recurrent, and further investigation lead to a diagnosis of tumor necrosis factor receptor–associated periodic syndrome (TRAPS). This case report affirms the broad clinical spectrum of the autoinflammatory diseases and reinforces the importance of obtaining a thorough history of previous episodes of skin and extracutaneous inflammation in patients with a clinical diagnosis of AHEI.
Background:
Erythema multiforme (EM) is an acute inflammatory mucocutaneous condition. EM is rarely described in children and infants.
Objective:
To investigate the triggers, clinical manifestations, and treatment of pediatric EM.
Methods:
Systematic literature review of pediatric EM.
Results:
After full-text article review, we included 113 articles, representing 580 patients. The mean age was 5.6 years, ranging 0.1-17 years. Infectious agents were the main triggers: herpes simplex virus (HSV) in 104 patients (17.9%) and Mycoplasma pneumoniae in 91 patients (15.7%). In total, 140 cases (24.1%) were drug-related and 89 cases (15.3%) had other triggers, such as vaccines (19 patients, 3.2%). In total, 229 patients had EM major (39.5%). Treatment was supportive care only (180 patients, 31.1%), systemic corticosteroids (115 patients, 19.8%), antivirals (85 patients, 14.6%), and antibiotics (66 patients, 11.3%), mostly macrolides (45 patients, 7.7%). Long-term sequelae were rare (1.3%). Pediatric EM was reported in 19 infants (3.2%). The main trigger was vaccination (9 patients). Infantile EM was EM major in 2 cases and EM minor in 17. Infants were less prone to develop EM major than older children (P < .01). Pediatric EM was recurrent in 83 cases (14.3%), which was triggered by HSV in 36 patients (61%). Recurrence affected older children.
Limitations:
Potential confusion between Steven Johnson syndrome and EM major in addition to publication bias.
Conclusion:
Pediatric EM is a rare disease, mainly triggered by infections. This condition can affect all mucosal surfaces, most commonly the oral mucosae. The diagnosis is clinical, and management relies on supportive care. Vaccines are a particular trigger in infants. Recurrent cases are most commonly linked to HSV. Dermatologists and pediatricians should be aware of this potentially recurrent and severe condition.
Cutaneous hypersensitivity reactions in infants present in a variety of patterns. These skin eruptions can be dramatic, causing alarm in parents and medical personnel. Many of these syndromes have overlapping features, which adds to the confusion and uncertainty regarding diagnosis and management. This review discusses the spectrum of hypersensitivity responses with a focus on their presentation in infants. The clinical findings, pathophysiology, histopathology, management, and complications of these conditions will be reviewed.
We report a case of acute hemorrhagic edema of infancy (AHEI) occurred in an 11-month-old male infant after upper respiratory tract infection. The onset was dramatic with petechiae, ecchymosis, and annular, nummular, or targetoid purpuric plaques on the extremities, face, and ears. Acute hemorrhagic edema of infancy is a benign form of leukocytoclastic vasculitis that typically affects children between 4 and 24 months of age. The etiology remains still unknown. The potential triggers of AHEI include preceding bacterial or viral infections, immunizations, and drugs. Although the clinical picture is fearful, in the majority of cases, it involves only cutaneous small vessels. Recognizing this as a distinct clinical entity allows to establish an appropriate prognosis for this rare benign disease in children.
This report could be a helpful reminder, especially for emergency physicians, to discriminate AHEI from other more severe diseases, such as meningococcal sepsis.
Acute hemorrhagic edema is an uncommon leukocytoclastic small-vessel vasculitis of young children.
To determine clinical features and outcome of acute hemorrhagic edema of young children.
Seven new cases are reported. A search of the literature revealed 287 published cases.
The 294 children (boys, 67%) ranged in age between 2 and 60 months (median, 11 months) and were in good general condition. In 195 children the disease developed after a simple acute infection. The exanthemata included large, round, red to purpuric plaques predominantly over the cheeks, ears, and extremities and mostly tender edema of the distal extremities, ears, and face. Involvement of body systems other than skin was rare. The children recovered spontaneously without sequelae.
Results of this review must be viewed with an understanding of the limitations of the analysis process, which incorporated data exclusively from single case reports or case series.
Acute hemorrhagic edema of young children is a very benign vasculitis. Physicians might rapidly develop the skills necessary to diagnose this condition.
Acute hemorrhagic edema of infancy (AHEI) is a benign and self-limiting disease that usually resolves completely without sequelae and without the need for active therapy. Most consider AHEI to be a separate entity from Henoch-Schonlein purpura (HSP), but others believe that they are different clinical presentations of the same disease.
We report a patient with AHEI who resolved with unusual scarring. We also present a review and update of AHEI synonyms, epidemiology, pathogenesis, clinical features, laboratory findings, histopathology, diagnosis and differentials, management, and prognosis.
A comprehensive Medline search using the Pubmed search engine in the English-language literature, for relative articles addressing AHEI disease. Thirty-three articles were cited, in addition to our case report.
Controversy still exists with regard to the separation of AHEI and HSP as distinct entities, as well as with regard to a conservative or active approach for AHEI treatment, and its effect on the course of the disease.
“Urticaria multiforme”: a case series and review of acute annular urticarial hypersensitivity syndromes in children
- Shah
Acute hemorrhagic edema of infancy and common mimics
- Homme