Eritema multiforme, una rara presentación de exantema en población pediátrica: a propósito de un caso / Erythema multiforme, a rare occurrence of exanthema in the pediatric population: on the subject of a case

El Eritema multiforme (EM) o eritema polimorfo es una enfermedad aguda de la piel de naturaleza inmunológica con o sin compromiso de mucosas, que puede comportarse como crónica recurrente. Se presenta con lesiones cutáneas en diana distintivas, a menudo acompañado de úlceras o bullas en mucosas (oral, genital u ocular). Entre sus formas clínicas se distingue: una forma menor caracterizado por un síndrome cutáneo leve y su forma mayor que se manifiesta como una afectación cutánea con daño mucoso marcado. Entre sus principales diagnósticos diferenciales se encuentran el Síndrome de Stevens-Johnson (SSJ) y Síndrome de Lyell (Necrólisis epidérmica tóxica (NET)). Tiene una incidencia estimada < 1%, siendo su forma mayor levemente más frecuente que su forma menor (0.8-6 por millón/año). Puede darse a cualquier edad, presentando un peak de incidencia entre los 20 y 30 años, predominando ligeramente el sexo masculino con una proporción 3:2, sin predilección racial. Su presentación en edad pediátrica es rara, más aún en la primera infancia. En esta población es más frecuente el EM menor recurrente. En el presente texto se reporta un caso de EM en población pediátrica como una rara forma de presentación exantemática, abordado en el Servicio de Pediatría del Complejo Asistencial Dr Victor Rios Ruiz (CAVRR)en la ciudad de Los Ángeles, Chile en el presente año.

Erythema multiforme (EM) also known as polymorph erythema is an acute skin disease of immunological nature with or without mucous membrane involvement, which may behave as chronic recurrent. It presents with distinctive targets like skin lesions, often together with ulcers or bullae in mucous membranes (oral, genital or ocular). Among its clinical forms are: a minor form characterized by a mild skin syndrome and its major form that manifests as a skin disease with marked mucosal damage. Among its main differential diagnoses are Stevens-Johnson Syndrome (SJS) and Lyell Syndrome (Toxic Epidermal Necrolysis (TEC)). It has an estimated incidence < 1%, with its major form being slightly more frequent than its minor form (0. 8-6 per million/year). It can occur at any age, presenting a peak incidence at the age between 20 and 30 years, with a slight predominance of males with a 3:2 ratio, without racial predilection. Its presentation in pediatric age is rare, even more so in early childhood. Minor recurrent EM is more common in this population. This paper reports a case of EM in the pediatric population as a rare form of exanthematic presentation, addressed at the Department of Pediatrics of the Complejo Asistencial Victor Rios Ruiz (CAVRR) in the city of Los Angeles, Chile this year.

Erythema Multiforme Versus Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Subtle Difference in Presentation, Major Difference in Management.

Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis are immune-mediated epidermal conditions with variable clinical presentations. Although their clinical presentations often overlap, they have distinct etiologies and potential outcomes, which necessitate specific management strategies. This case is presented to highlight the subtle differences and review management given that Stevens-Johnson syndrome/toxic epidermal necrolysis can rapidly become life-threatening. The need for astute diagnostic work-up and accuracy is magnified in the military setting given operations in austere environments and availability of medical and medical evacuation resources. Herein, we present a less common case of bullous erythema-multiforme, the diagnostic approach, and clinical differential with special attention to the importance of the military physician.

Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment.

Prior to the first international consensus classification published in 1993, the clinical distinction between erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) had been subject to uncertainty and controversy for more than a century. Based on this classification, the three conditions are defined by the morphology of the individual lesions and their pattern of distribution. Etiopathogenetically, the majority of EM cases is caused by infections (primarily herpes simplex virus and Mycoplasma pneumoniae), whereas SJS/TEN are predominantly triggered by drugs. The SCORTEN (score of toxic epidermal necrolysis) can and should be used to assess disease prognosis in patients with SJS/TEN. While supportive treatment is generally considered sufficient for EM, there is still uncertainty as to the type of systemic therapy required for SJS/TEN. Given the lack of high-quality therapeutic trials and (in some cases) conflicting results, it is currently impossible to issue definitive recommendations for any given immunomodulatory therapy. While there is always a trade-off between rapid onset of treatment-induced immunosuppression and an uptick in infection risk, there has been increasing evidence that cyclosporine in particular may be able to halt disease progression (i.e. skin detachment) and lower mortality rates. Assistance in diagnosis and management of the aforementioned conditions may be obtained from the Center for the Documentation of Severe Skin Reactions (dZh) at the Department of Dermatology, University Medical Center, Freiburg, Germany.

Triggers, clinical manifestations, and management of pediatric erythema multiforme: A systematic review.

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.

Erythema Multiforme: Recognition and Management.

Erythema multiforme is an immune-mediated reaction that involves the skin and sometimes the mucosa. Classically described as target-like, the erythema multiforme lesions can be isolated, recurrent, or persistent. Most commonly, the lesions of erythema multiforme present symmetrically on the extremities (especially on extensor surfaces) and spread centripetally. Infections, especially herpes simplex virus and Mycoplasma pneumoniae, and medications constitute most of the causes of erythema multiforme; immunizations and autoimmune diseases have also been linked to erythema multiforme. Erythema multiforme can be differentiated from urticaria by the duration of individual lesions. Erythema multiforme lesions are typically fixed for a minimum of seven days, whereas individual urticarial lesions often resolve within one day. Erythema multiforme can be confused with the more serious condition, Stevens-Johnson syndrome; however, Stevens-Johnson syndrome usually contains widespread erythematous or purpuric macules with blisters. The management of erythema multiforme involves symptomatic treatment with topical steroids or antihistamines and treating the underlying etiology, if known. Recurrent erythema multiforme associated with the herpes simplex virus should be treated with prophylactic antiviral therapy. Severe mucosal erythema multiforme can require hospitalization for intravenous fluids and repletion of electrolytes.

Erythema multiforme: Differences between HSV-1 and HSV-2 and management of the disease-A case report and mini review.

Erythema multiforme (EM) is an immune-mediated reaction characterized by target lesions and with possible mucosal involvement. Its most frequent cause is HSV, with HSV-1 more common than -2. It is usually self-limited but it can show recurrences. We report a peculiar case of recurrent herpes-associated erythema multiforme (HAEM) in a 35-year-old man. The patient was affected by both herpes labialis and genitalis, but the typical target lesions were only associated with recurrent herpes labialis. Here, we hypothesize about the pathogenic differences between HSV-1 and HSV-2, and discuss the therapeutic management of HAEM.

Painful Oral Lesions.

Painful oral vesiculoerosive diseases (OVD) include lichen planus, pemphigus vulgaris, mucous membrane pemphigoid, erythema multiforme, and recurrent aphthous stomatitis. OVD lesions have an immunopathic cause. Treatment is aimed at reducing the immunologic and the following inflammatory response. The mainstay of OVD management is topical or systemic corticosteroids to include topical triamcinolone, fluocinonide, and clobetasol, whereas systemic medications used in practice can include dexamethasone, prednisone, and prednisolone. Oral herpetic lesions can be primary or recurrent. If management is desired, they can be treated by topical or systemic antiviral drugs. Topical antiviral creams include prescription acyclovir, penciclovir and over-the-counter docosanol.

Current Perspectives on Erythema Multiforme.

Recognition and timely adequate treatment of erythema multiforme remain a major challenge. In this review, current diagnostic guidelines, potential pitfalls, and modern/novel treatment options are summarized with the aim to help clinicians with diagnostic and therapeutic decision-making. The diagnosis of erythema multiforme, that has an acute, self-limiting course, is based on its typical clinical picture of targetoid erythematous lesions with predominant acral localization as well as histological findings. Clinically, erythema multiforme can be differentiated into isolated cutaneous and combined mucocutaneous forms. Atypical erythema multiforme manifestations include lichenoid or granulomatous lesions as well as lesional infiltrates of T cell lymphoma and histiocytes. Herpes simplex virus infection being the most common cause, other infectious agents like-especially in children-Mycoplasma pneumoniae, hepatitis C virus, Coxsackie virus, and Epstein Barr virus may also trigger erythema multiforme. The second most frequently identified cause of erythema multiforme is drugs. In different studies, e.g., allopurinol, phenobarbital, phenytoin, valproic acid, antibacterial sulfonamides, penicillins, erythromycin, nitrofurantoin, tetracyclines, chlormezanone, acetylsalicylic acid, statins, as well as different TNF-α inhibitors such as adalimumab, infliximab, and etanercept were reported as possible implicated drugs. Recently, cases of erythema multiforme associated with vaccination, immunotherapy for melanoma, and even with topical drugs like imiquimod have been described. In patients with recurrent herpes simplex virus-associated erythema multiforme, the topical prophylactic treatment with acyclovir does not seem to prevent further episodes of erythema multiforme. In case of resistance to one virostatic drug, the switch to an alternative drug, and in patients non-responsive to virostatic agents, the use of dapsone as well as new treatment options, e.g., JAK-inhibitors or apremilast, might be considered.

[Targeting the skin - erythema multiforme in dogs and cats]. / Die Haut als Zielscheibe ­ Erythema multiforme bei Hund und Katze.

This review article will describe the complex nature of erythema multiforme in dogs and cats. The disease pattern will be illustrated in terms of etiology, pathogenesis, clinical signs, diagnostics and therapy. Erythema multiforme is a rare, immune-mediated skin disorder with an acute specific reaction pattern of skin and mucous membranes against different underlying causes. Reported triggering factors include drugs, food ingredients and neoplasias, but viral or bacterial infections and vaccine reactions were also identified. In over 20% of the cases the cause cannot be identified. The German Shepherd dog and Pembroke Welsh Corgi seem to be predisposed. Erythematous macules, slightly elevated and peripherally spreading papules as well as circular to arciform patterns were described frequently in the dog. Wheals, plaques, vesicles and bullae that develop into ulcers, are also seen. Frequently affected areas in the dog are the ventrum, mucocutaneous junctions, mouth, pinnae and foot pads. Histopathological findings include single cell necrosis in all layers of the epidermis with lymphocytic satellitosis and a cell-poor interface dermatitis. Elimination of the causative factors and concurrent symptomatic therapy are recommended. The prognosis varies from guarded to fair, depending on the underlying cause, presenting clinical signs and response to therapy.

Paediatric Erythema Multiforme: Epidemiological, Clinical and Laboratory Characteristics.

Erythema multiforme (EM) is an immune-mediated reaction presenting as acrofacial target lesions. Most studies utilize the outdated classification, which includes EM, Stevens-Johnson syndrome and toxic epidermal necrolysis as related entities. We describe here epidemiological, aetiological, clinical, laboratory and treatment characteristics of paediatric EM. This is a retrospective single-centre study, performed between 2000 and 2013. Of 119 children given a diagnosis of EM, only 30 met clinical criteria and were included in this study. Most misdiagnosed cases were non-specific eruptions and urticaria multiforme. Mean age was 11.3 years. Fifty percent had mucosal involvement. An aetiology was observed in half of the patients. Seventy percent of patients were admitted to hospital, 46.7% were treated with systemic steroids. Sixteen percent had recurrent EM. The most common identified infectious agent associated with EM in this study was Mycoplasma pneumonia and the cases associated with this infection may represent the recent entity, mycoplasma-induced rash and mucositis. Association with herpes simplex virus was not observed. Despite being a benign, self-limiting condition, children were over-treated in terms of hospitalization and therapy.

[Childhood postinfectious erythema multiforme]. / Érythème polymorphe postinfectieux de l'enfant.

Postinfectious erythema multiforme is an uncommon skin disease in childhood that can have a strong impact, especially in infants if there is involvement of the mucous membranes. The lesion is targeted (central bullous lesions with three concentric circles). Its diagnosis is typically made clinically. Atypical forms are the highly inflammatory, mainly bullous type, with exclusive involvement of the mucous membrane, or recurrent erythema multiforme. The diagnosis of erythema multiforme is frequently suspected in children with urticaria multiforme. Kawasaki disease and toxic epidermal necrolysis may have a target-like skin aspect with mucosal involvement, and should be considered when planning treatment. The two major infectious etiologies in children are Mycoplasma pneumoniae and Herpes simplex. More recently, postvaccination erythema multiforme has been reported with the majority of vaccines used in pediatric practice. The prognosis is usually good but requires observation of the mucosal involvement because of the risk of serious complications. The treatment of erythema multiforme is essentially symptomatic, with etiology-related treatment.

[Features of dynamics of sera cytokines in patients with erythema multiforme during immunotherapy].

AIM: Study the features of cytokine profile in patients with exudative erythema multiforme (EME) and dynamics of basal level of pro-inflammatory and anti-inflammatory cytokines during immunotherapy. MATERIALS AND METHODS: 39 adult patients with erythema multiforme were examined. The patients were split into groups based on therapy variant. One group (14 individuals) received Immunovac-VP-4 against the background of basic therapy; the other (12 individuals)--cagocel against the background of basic therapy; comparison group (13 individuals) received only basic therapy; 15 individuals composed a group of healthy individuals. All the patients had the level of pro-, anti-inflammatory and regulatory cytokines determined in blood sera by solid-phase EIA method by using Biosource (Austria) test-systems at the beginning of the study and after the therapy. RESULTS: In patients with exudative erythema multiforme Immunovac-VP-4 therapy facilitated a significant (p < 0.05) increase of serum IFN-gamma level, insignificant (p > 0.05) increase of IL-1beta and decrease of IL-17. Whereas cagocel lead to an increase of IL-4 (p > 0.05), IL-2, IFN-gamma (p < 0.05) and decrease of TGF-beta and IL-12 (p < 0.05). At the same time basic therapy facilitated a significant increase of IL-5 and decrease of IL-6, IL-12, IFN-gamma. CONCLUSION: Immunovac-VP-4 facilitates the increase of secretion of IFN-gamma, IL-1beta against the background of TGF-beta that facilitates normalization of cooperation of cells in immune response including against viral infections, and thereby influencing the trigger factor in EME patients.