Mucocutaneous Eruption Research Articles

Stevens-Johnson syndrome in children.

The concept of Stevens-Johnson syndrome (SJS) in children is evolving. This manuscript reviews recent advances with the lens of new terminology namely infection-triggered reactive infectious mucocutaneous eruption and drug-induced epidermal necrolysis, with the objective of integrating this novel terminology practically. Traditionally considered to exist on a spectrum with toxic epidermal necrolysis, SJS in children is more often caused or triggered by infections instead of medications. Proposed pediatric-specific terminology can be applied to literature to gain further insights into blistering severe cutaneous adverse reactions. Distinguishing infection-triggered from drug-triggered blistering reactions is useful for 3 main reasons: (1) early clinically recognizable different features such as isolated or predominant mucositis, (2) different initial management depending on trigger, (3) avoiding the label of a drug reaction on cases triggered by infection.

Reactive infectious mucocutaneous eruption in a young-adult with COVID-19

In this case report, we detail the case of a young adult with a recurrent case of Reactive Infectious Mucocutaneous Eruption (RIME) in the setting of Sars-COV-2 infection, the second time this has been reported in the literature. We review the more common causes of RIME, including Chlamydophila pneumoniae, metapneumovirus, parainfluenza, rhinovirus, enterovirus, and influenza; and we discuss patient characteristics in other reported cases of RIME secondary to COVID-19 and the features of recurrent RIME seen in patients reported in the literature. Our patient had the characteristic severe mucosal involvement seen with RIME, and was treated with supportive care alone and experienced rapid improvement in symptoms.

Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.

Viral exanthems in children

Virale und paravirale Exantheme sind die häufigsten Exantheme des Kindesalters, die auch meist zu einer Vorstellung in der Praxis inklusive der Notaufnahme führen. Die Kenntnis der verschiedenen Krankheitsbilder ist entscheidend für die adäquate Diagnostik und Betreuung der kleinen Patient:innen, die entsprechende Aufklärung der Familie und ggf. das Minimieren des Infektionsrisikos. In der aktuellen Arbeit werden die kürzlich beschriebenen „reactive infectious mucocutaneous eruptions“ (RIME) und das „pediatric inflammatory multisystem syndrome“ (PIMS) vorgestellt. Zusätzlich werden wir einige atypische Manifestationen des Gianotti-Crosti-Syndroms und der Hand-Fuß-Mund-Erkrankung – wie z. B. die mit Mollusken assoziierte „Gianotti-Crosti-like reaction“ und das „eczema coxsackium“ – erläutern.

Reactive infectious mucocutaneous eruption.

Journal of Paediatrics and Child HealthVolume 58, Issue 5 p. 932-932 Images of the Month Reactive infectious mucocutaneous eruption First published: 02 May 2022 https://doi.org/10.1111/jpc.2_15627Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume58, Issue5May 2022Pages 932-932 RelatedInformation

Reactive infectious mucocutaneous eruption.

Journal of Paediatrics and Child HealthVolume 58, Issue 5 p. 931-931 Images of the Month Reactive infectious mucocutaneous eruption Dr Aryan Riahi, Dr Aryan Riahi orcid.org/0000-0001-9318-9356 Faculty of MedicineSearch for more papers by this authorDr Joseph M Lam, Dr Joseph M Lam Department of Paediatrics Dermatology, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this author Dr Aryan Riahi, Dr Aryan Riahi orcid.org/0000-0001-9318-9356 Faculty of MedicineSearch for more papers by this authorDr Joseph M Lam, Dr Joseph M Lam Department of Paediatrics Dermatology, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this author First published: 02 May 2022 https://doi.org/10.1111/jpc.1_15627Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume58, Issue5May 2022Pages 931-931 RelatedInformation

Severe Airflow Obstruction From Constrictive (Obliterative) Bronchiolitis in Paraneoplastic Autoimmune Multiorgan Syndrome

Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as “paraneoplastic pemphigus,” is a rare life-threatening autoimmune disease triggered by an underlying neoplasm. It is characterized by painful erosive stomatitis and a polymorphous mucocutaneous eruption.1 A subset of patients with PAMS manifest pulmonary involvement characterized by progressive airflow obstruction related to constrictive (obliterative) bronchiolitis (also known as bronchiolitis obliterans).2

Immune- and Non-Immune-Mediated Adverse Effects of Monoclonal Antibody Therapy: A Survey of 110 Approved Antibodies.

Identification of new disease-associated biomarkers; specific targeting of such markers by monoclonal antibodies (mAbs); and application of advances in recombinant technology, including the production of humanized and fully human antibodies, has enabled many improved treatment outcomes and successful new biological treatments of some diseases previously neglected or with poor prognoses. Of the 110 mAbs preparations currently approved by the FDA and/or EMA, 46 (including 13 antibody–drug conjugates) recognizing 29 different targets are indicated for the treatment of cancers, and 66, recognizing 48 different targets, are indicated for non-cancer disorders. Despite their specific targeting with the expected accompanying reduced collateral damage for normal healthy non-involved cells, mAbs, may cause types I (anaphylaxis, urticaria), II (e.g., hemolytic anemia, possibly early-onset neutropenia), III (serum sickness, pneumonitis), and IV (Stevens–Johnson syndrome, toxic epidermal necrolysis) hypersensitivities as well as other cutaneous, pulmonary, cardiac, and liver adverse events. MAbs can provoke severe infusion reactions that resemble anaphylaxis and induce a number of systemic, potentially life-threatening syndromes with low frequency. A common feature of most of these syndromes is the release of a cascade of cytokines associated with inflammatory and immunological processes. Epidermal growth factor receptor-targeted antibodies may provoke papulopustular and mucocutaneous eruptions that are not immune-mediated.

Reactive infectious mucocutaneous eruptions associated with SARS-CoV-2 infection

Several dermatological manifestations are described in COVID-19. A 3-year-old boy presented with fever, painful oral ulcers with COVID-19 infection. A diagnosis of reactive infectious mucocutaneous eruptions (RIME) was made. He was worked up for the usual causes of RIME but were all negative. His severe acute respiratory syndrome corona virus-2 immunoglobulin M (SARS-CoV-2 IgM) was positive and he was managed with Intravenous hydrocortisone with which he improved dramatically. RIME might be the first/only manifestation of COVID-19 and it should be kept in mind.

A Systematic Review of Clinical Studies on Mucocutaneous Manifestations of COVID-19: Virus-Related and Drug-Related

Coronavirus could affect almost any part of the body including the skin. In this systematic review, the primary skin lesions resulting from the direct activity of the virus or the medications used for treatment and the changes in the behavior of the virus regarding the occurrence of these symptoms over time were assessed. PubMed/MEDLINE, Embase, PsycINFO, TRIP Cochrane, Cochrane Skin were searched for all published articles from February 19 to July 1, 2020, which met the inclusion criteria. Thirty-six related articles were extracted. Twenty-eight studies reported virus-related mucocutaneous eruptions and 8 articles, the drug-reactions. Data of 583 patients were included. Skin lesions of COVID-19 could be caused by both the virus itself or the influence of drugs used for the treatment. Morbilliform rashes, urticaria, and acral-vasculopathic cutaneous lesions were at the forefront of primary COVID-dependent skin lesions with no significant change during time, Also, Hydroxychloroquine, lopinavir/ritonavir, paracetamol, and antibiotics were reported as the main causes of drug-induced rashes. Since dermatologic manifestations may occur prior or simultaneously/after other COVID clinical symptoms, so they may helpful in patients’ early diagnosis or prediction of internal organ involvements via histopathologic evaluations of skin biopsies especially about vasculopathic and vasculitic, respectively.

M062 STEVENS-JOHNSON SYNDROME OR MYCOPLASMA PNEUMONIAE- INDUCED RASH AND MUCOSITIS: A DIAGNOSTIC DILEMMA

Stevens- Johnson syndrome (SJS) and Mycoplasma pneumoniae- induced rash and mucositis (MIRM) both cause similar muco-cutaneous eruptions. Differences between the two include that SJS affects all ages, is more common in women, and associated with drug and infections, with a mortality rate of about 10%. MIRM affects younger patients, is more common in males, is associated with Mycoplasma infections, and has a milder course, with a mortality rate of around 3%.

Severe and life-threatening COVID-19-related mucocutaneous eruptions: A systematic review.

ObjectivesEarlier diagnosis and the best management of virus‐related, drug‐related or mixed severe potentially life‐threatening mucocutaneous reactions of COVID‐19 patients are of great concern. These patients, especially hospitalised cases, are usually in a complicated situation (because of multi‐organ failures), which makes their management more challenging. In such consultant cases, achieving by the definite beneficial management strategies that therapeutically address all concurrent comorbidities are really hard to reach or even frequently impossible.MethodsAccording to the lack of any relevant systematic review, we thoroughly searched the databases until 5 October 2020 and finally found 57 articles including 93 patients. It is needed to know clinical presentations of these severe skin eruptions, signs and symptoms of COVID in these patients, time of skin rash appearance, classifying drug‐related or virus‐related skin lesions, classifying the type of skin rash, patients’ outcome and concurrent both COVID‐19 therapy and skin rash treatment.ResultSevere and potential life‐threatening mucocutaneous dermatologic manifestations of COVID‐19 usually may be divided into three major categories: virus‐associated, drug‐associated, and those with uncertainty about the exact origin. Angioedema, vascular lesions, toxic shock syndrome, erythroderma, DRESS, haemorrhagic bulla, AGEP, EM, SJS and TEN, generalised pustular figurate erythema were the main entities found as severe dermatologic reactions in all categories.ConclusionWe can conclude vascular injuries may be the most common cause of severe dermatologic manifestations of COVID‐19, which is concordant with many proposed hypercoagulation tendencies and systemic inflammatory response syndrome as one of the most important pathomechanisms of COVID‐19 so the skin may show these features in various presentations and degrees.

Reactive infectious mucocutaneous eruption in children diagnosed with COVID-19.

We describe the cases of two immunocompetent children who developed mucositis with oral, ocular, and genital involvement during acute COVID‐19 illness. The pattern of mucosal involvement with no other cutaneous involvement was consistent with reactive infectious mucocutaneous eruption (RIME). No other intercurrent infections or new medications were identified, suggesting that COVID‐19 was causative. Both patients noted improvement with systemic corticosteroid therapy.

Dapsone Induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome - A Case Report

A 50-year-old male Sudanese patient presented with a three-week history of jaundice, high-grade fever, and mucocutaneous eruption. For last months he was on compound therapy for leprosy, which had been confirmed recently. The patient’s face was prominent, along with the erythematous dusky morbilliform rash covering all the body. On examination, we detected hepatosplenomegaly and generalized lymphadenopathy. Laboratory investigations revealed hepatorenal impairment, and hematological analysis revealed leukocytosis mainly due to eosinophilia. The clinical and laboratory findings interpretation ranked DRESS or Drug-Induced Hypersensitivity Syndrome (DIHS) on top of possible causes before Dapsone Hypersensitivity Syndrome (DHS) and lepra reactions. We promptly discontinued MDT, admitted him to the dermatological ward. Two skin biopsies were sent to two different histopathologists, MF was suggested by one and Sezary syndrome by the other one. Besides the general conservative measures and vital functions monitoring, he received systemic and topical steroids. However, unfortunately, within the next three weeks, his condition deteriorated, and passed away from multi-systems failure.

26344 Recurrent reactive infectious mucocutaneous eruption (RIME) caused by human metapneumovirus

Reactive infectious mucocutaneous eruption (RIME) was recently proposed by Ramien et al to encompass mucocutaneous eruptions previously diagnosed as Mycoplasma pneumoniae induced rash and mucositis (MIRM) that are caused by nonmycoplasma pathogens. The pathogenesis is thought to involve immune complex deposition, complement activation, and molecular mimicry that leads to mucositis with limited skin involvement. We describe a unique case of recurrent RIME in a teenage patient with two different triggering pathogens. A healthy 13-year-old male presented with cough and fever for two weeks and sores of the throat and lips for one day. Upon examination, there was extensive eschars and sloughing with bleeding of lips and diffuse sloughing of the oral mucosa and tongue. He also had noncoalescing, purple eschars on the scrotum, penile length, and meatus. Mycoplasma titers were positive, and the patient was diagnosed with MIRM without skin involvement—symptoms improved with supportive measures and azithromycin. Four years following the initial episode, the 17-year-old patient presented with throat and mouth pain for five days with fever. He endorsed flu-like symptoms a week prior to presentation. On examination, there were confluent erosions of the mucosal lips and numerous ill-defined erosions on the buccal cheek and palate. Ill-defined erosions were also present on the penile head. Azithromycin was initiated for presumed recurrent MIRM, but Mycoplasma titers returned negative. Further workup demonstrated human metapneumovirus (hMPV) infection and the patient was diagnosed with reactive mucositis secondary to hMPV. To our knowledge, this is the first case of recurrent RIME caused by hMPV.

Reactive infectious mucocutaneous eruption.

Journal of Paediatrics and Child HealthEarly View Image of the Month Reactive infectious mucocutaneous eruption Dr Aryan Riahi, orcid.org/0000-0001-9318-9356 Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this authorDr Joseph M Lam, Department of Paediatrics, University of British Columbia, Vancouver, British Columbia, Canada Department of Dermatology, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this author Dr Aryan Riahi, orcid.org/0000-0001-9318-9356 Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this authorDr Joseph M Lam, Department of Paediatrics, University of British Columbia, Vancouver, British Columbia, Canada Department of Dermatology, University of British Columbia, Vancouver, British Columbia, CanadaSearch for more papers by this author First published: 01 July 2021 https://doi.org/10.1111/jpc.15627Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

Evaluation of Etanercept for Treatment of Reactive Infectious Mucocutaneous Eruption

This cohort study evaluates the association of etanercept treatment with outcomes of reactive infectious mucocutaneous eruption in hospitalized children and adolescents.

Adenoviral-induced rash and mucositis: Expanding the spectrum of reactive infectious mucocutaneous eruption.

Mucocutaneous eruptions associated with respiratory pathogens, specifically Mycoplasma pneumoniae (MP), has recently been described as a MIRM (MP-induced rash and mucositis). The term reactive infectious mucocutaneous eruption (RIME) has been proposed, since non-MP pathogens may also cause a similar rash and mucositis. We report two cases with clinical manifestations suggestive of MIRM/RIME, both with documented adenovirus infection.

Reactive infectious mucocutaneous eruption: Mycoplasma pneumoniae-induced rash and mucositis and other parainfectious eruptions.

Mycoplasma pneumoniae-induced rash and mucositis is the most accurate diagnosis for patients with blistering mucocutaneous disease provoked by an infection. Recent literature suggests expansion of the name is required, as other infections have caused a clinically similar presentation. This review provides a concise update on current understanding of M.pneumoniae-induced rash and mucositis and other reactive infectious mucocutaneous eruptions.

Deficits in ascending and descending pain modulation pathways in patients with postherpetic neuralgia

Postherpetic Neuralgia (PHN), develops after the resolution of the herpes zoster mucocutaneous eruption, is a debilitating chronic pain. However, there is a lack of knowledge regarding the underlying mechanisms associated with ascending and descending pain modulations in PHN patients. Here, we combined psychophysics with structural and functional magnetic resonance imaging (MRI) techniques to investigate the brain alternations in PHN patients. Psychophysical tests showed that compared with healthy controls, PHN patients had increased state and trait anxiety and depression. Structural MRI data indicated that PHN patients had significantly smaller gray matter volumes of the thalamus and amygdala than healthy controls, and the thalamus volume was negatively correlated with pain intensity (assessed using the Short-form of the McGill pain questionnaire) in PHN patients. When the thalamus and periaqueductal gray matter (PAG) were used as the seeds, resting-state functional MRI data revealed abnormal patterns of functional connectivity within ascending and descending pain pathways in PHN patients, e.g., increased functional connectivity between the thalamus and somatosensory cortices and decreased functional connectivity between the PAG and frontal cortices. In addition, subjective ratings of both Present Pain Index (PPI) and Beck-Depression Inventory (BDI) were negatively correlated with the strength of functional connectivity between the PAG and primary somatosensory cortex (SI), and importantly, the effect of BDI on PPI was mediated by the PAG-SI functional connectivity. Overall, our results provided evidence suggesting deficits in ascending and descending pain modulation pathways, which were highly associated with the intensity of chronic pain and its emotional comorbidities in PHN patients. Therefore, our study deepened our understanding of the pathogenesis of PHN, which would be helpful in determining the optimized treatment for the patients.