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Mycoplasma pneumoniae -induced rash and mucositis (MIRM): An unusual mild skin rash associated with severe mucosal involvement
... Culture or PCR (throat/ sputum) are not always reliable and cannot distinguish (6) MP infection from MP carriage in the upper respiratory tract (up to 56% of healthy children). Therefore, the diagnosis mainly relies on serological methods, which demonstrate rising serial MP-specific IgM antibody titers over time (6,7,8,10). ...
... The umbrella term reactive infectious mucocutaneous eruption (RIME) has been coined to encompass clinically similar mucositis disorders caused by MP, CP and others (6). A diagnosis of RIME should be considered when an acute-onset mucositis, linked to a prodromal respiratory illness and lacking a history of offender medication is seen in children or adolescents (5,7,10). ...
Mycoplasma pneumoniae-Associated mucositis (MPAM) is a novel and rare disorder, both interesting and confusing for physicians who are unaware of its existence. MPAM is described as an atypical milder variant of Stevens-Johnson syndrome (SJS), triggered by Mycoplasma pneumoniae. Compared to drug-induced SJS or toxic epidermal necrolysis (TEN), MPAM tends to affect children and adolescents, shows a predominantly mucosal pattern (two or more mucosal sites), is associated with pneumonia, runs a milder disease course, displays a favorable prognosis, a low recurrence and mortality rate and uncommon long-Term sequelae. Establishing a definitive diagnosis of MPAM may prevent unnecessary hospitalization, offer appropriate management of patients and preclude their stigmatization as victims of drug allergy. Herein we report two children with MPAM, which we believe is increasingly encountered in clinical practice.
... It is also notable that there is a better prognosis of MIRM even with support treatment alone (11,(21)(22)(23)(24)(25). All these clinical features may help physicians distinguish the clinical spectrum of EM, SJS/TEN and MIRM, where skin lesions are different and milder, explaining its previous and obsolete name of incomplete SJS (11,26). ...
... Although antibiotic treatment directed at M. pneumoniae eliminates the causative agent and limits the duration and severity of the pulmonary disease, the paucity of data does not indicate whether the incidence or severity of the mucocutaneous eruption is reduced (16). Additionally, the role of immunomodulatory therapies is not clear, and there is clinical evidence that IVIG may be helpful for MIRM patients with severe mucositis (11,14,17,20,26). In our report, three patients with severe mucositis had been treated with IVIG, and only one patient had genital synechiae and underwent circumcision. ...
Dermatological disorders are the most common extrapulmonary complications of Mycoplasma pneumoniae, of which Mycoplasma-induced rash and mucositis (MIRM) has recently been proposed to be a separate diagnostic entity. MIRM could easily be misdiagnosed as atypical Stevens-Johnson syndrome by clinicians due to the unawareness of this rare disease. We retrospectively reviewed the inpatient database from Jan. 2016 to Dec. 2019 of the Children's Hospital of Fudan University. In total, five patients (mean age 5.5 years, three male) matched the diagnostic criteria of MIRM. All patients had scattered lesions and more than two sites of mucosal involvement. The serum IgA level of three patients was higher than normal. Two patients had a significant decrease in peripheral blood CD3+ T and CD4+ T cells that improved with recovery. The percentage of TCRαβ+ CD4–CD8–T cells of Patient five was higher than normal. All patients received treatments with antibiotics and corticosteroids, 3 patients received intravenous immunoglobulin. Among five patients, three patients complained of dyspigmentation, and two patients had an uneventful recovery. MIRM is a separate entity with predominant mucosal involvement and excellent prognosis that more often affects younger patients. Excessive inflammatory reactions may lead to immune disorders, including lymphopenia and a redistribution of CD4+ T cells. We recommend that pneumonia accompanied by mucocutaneous eruptions, especially in young patients, should raise clinical suspicion of MIRM.
... pneumoniae extrapulmonary diseases, MpEPDs), which may affect a variety of organs, including skin, mucosae, muscles, joints, heart, and central nervous system. Even though the pathogenesis of MpEPDs has not been fully elucidated yet (and is likely to be variable according to different types of extrapulmonary manifestations), it is supposed to be immune-mediated [4][5][6]. Recent studies suggested that MpEPDs, as well as pulmonary complications, may be promoted by delayed antibiotic treatment and/or microbial persistence due to antibioticresistant strains, [7][8][9][10] whereas other authors evidenced some correlation with immunological aspects, such as increased levels of serum IgE and/or the presence of a 2polarized immunological environment [10][11][12][13][14]. erefore, M. pneumoniae seems to have a complex (and not fully elucidated) interaction with the host's immune system. Although M. pneumoniae is one of the leading causes of CAP [1,2], its relevance in the setting of primary immunodeficiencies (PIDs) seems to be poorly investigated so far. ...
Mycoplasma pneumoniae (M. pneumoniae) is one of the leading causes of community-acquired pneumonia in children and is also implicated in a variety of reactive extrapulmonary diseases. Recurrent and/or severe respiratory infections are one of the most frequent manifestations of several types of primary immunodeficiency. Here, we reviewed the medical literature to assess the potential relevance of M. pneumoniae in the infections observed in children affected with combined, humoral, and innate primary immune deficiencies. M. pneumoniae does not result to be epidemiologically prevalent as a cause of pneumonia in children affected by primary immunodeficiencies, but this infection can have a persistent or severe course in this category of patients. Indeed, the active search of M. pneumoniae could be useful and appropriate especially in children with humoral immune deficiencies. Indeed, most cases of M. pneumoniae infection in primary immunodeficiencies are described in patients affected by a/hypo-gammaglobulinemia.
... Several dermatologic manifestations of M. pneumoniae have been described. These include non-specific urticarial and maculopapular rashes, erythema multiforme, Stevens-Johnson syndrome, and M. pneumoniae-induced rash and mucositis [18]. The mechanisms of these skin manifestations are thought to be via direct inoculation, secondary immunemediated damage, vascular occlusion, or direct invasion of the vascular tissue, depending on the presentation [10,19]. ...
Background
Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder associated with a broad symptom presentation that is still being characterized. We report a rare case of recurrent mycoplasma skin abscesses in a patient with a history of autoimmune disorders and prolonged mycoplasma pneumonia who was diagnosed with CVID.
Case presentation
A 34-year-old woman presented with a history of recurrent abscesses previously confirmed positive for Mycoplasma pneumoniae . Her past medical history of recurrent mycoplasma abscesses, prolonged mycoplasma pneumonia, and autoimmune disorders (mixed connective tissue disease and immune thrombocytopenia) raised suspicion of CVID. Workup included negative anti-mycoplasma antibody titers, hypogammaglobulinemia, and negative anti-pneumococcal antibody titers despite prior vaccination, solidifying the diagnosis of CVID. The patient was discharged on antibiotic and intravenous immunoglobulin therapy and now follows allergy and immunology long-term for treatment.
Conclusions
Her diagnostic history underscores the importance of considering the various criteria of CVID for diagnosis, and her unique presentation of M. pneumoniae skin abscesses highlights the broad sequelae patients with CVID can manifest.
... pneumoniae-induced rash and mucositis. 18 The mechanisms of these skin manifestations are thought to be via direct inoculation, secondary immune-mediated damage, vascular occlusion, or direct invasion of the vascular tissue, depending on the presentation. 10,19 Some manifestations for example, such as the bullous lesions, are thought to be due to hematogenous transfer of the bacteria to the dermis, with damage caused by generation of in ammatory cytokines. ...
Background: common variable immunodeficiency (CVID) is a primary immunodeficiency disorder associated with a broad symptom presentation that is still being characterized. We report a rare case of recurrent mycoplasma skin abscesses in a patient with a history of autoimmune disorders and prolonged mycoplasma pneumonia who was diagnosed with CVID.
Case presentation: a 34-year-old woman presented with a history of recurrent abscesses previously confirmed positive for Mycoplasma pneumoniae. Her past medical history of recurrent mycoplasma abscesses, prolonged mycoplasma pneumonia, and autoimmune disorders (mixed connective tissue disease and immune thrombocytopenia) raised suspicion of CVID. Workup included negative anti-mycoplasma antibody titers, hypogammaglobulinemia, and negative anti-pneumococcal antibody titers despite prior vaccination, solidifying the diagnosis of CVID. The patient was discharged on antibiotic and intravenous immunoglobulin therapy with improvement and now follows allergy and immunology long-term for treatment.
Conclusions: her diagnostic history underscores the importance of considering the various criteria of CVID for diagnosis, and her unique presentation of M. pneumoniae skin abscesses highlights the broad sequelae patients with CVID can manifest.
... Discussion MIRM is usually characterized by prominent mucositis with skin lesions. Isolated severe mucositis with oral and urogenital lesions without skin rash is also quite common [5]. The clinical presentation is mostly found in young adolescents and children. ...
Pulmonary infections are often caused by Mycoplasma pneumoniae. However, extra pulmonary manifestations are also reported. Mycoplasma pneumonia associated severe mucositis with relative few skin rash has recently been described as a new clinical disease named Mycoplasma Pneumoniae-Induced Rash and Mucositis (MIRM). A 14-year-old girl who developed exuberant mucositis, as well as multiple tense bullae and several targetoid lesions were found on the face, trunk and 4 limbs, but no obvious pulmonary manifestation was found. The laboratory examinations showed elevated M. pneumoniae IgM antibodies. Due to this unique clinical presentation, MIRM was considered and differentiated from Toxic Epidermal Necrolysis (TEN), Stevens-Johnson Syndrome (SJS), or Erythema Multiforme (EM). MIRM may be reconsidered as a specific cutaneous manifestation with or without pulmonary infection.
... Another aspect of the natural history of this disease is that recurrence with subsequent M pneumoniae or viral infections is possible [11,12]. In addition, most of the patients in the literature were children and adolescents that recovered from MIRM over the course of days [13][14][15][16][17]; however, the adult patient we described had persistent symptoms at 1 month follow up. It is possible that this longer duration is related to age because another patient in the literature at 42 years old also had persistent pain at 1 month follow up; however, further data are needed to make a conclusion [18]. ...
We describe a case of a 33-year-old-male with Mycoplasma pneumoniae-induced rash and mucositis and review the literature on this newly described syndrome.
... 3,4 Only in recent years have a few case reports of MIRM in adults been reported, describing presentations that vary in severity, involved sites, and prognosis. 5, 6 We present 2 adult cases of MIRM following pneumonia with mucositis with oral, ocular, and genital involvement and with sparse cutaneous lesions. We review the literature and discuss the knowledge gap regarding MIRM that warrants further studies with the aim of preventing MIRM, reducing its complications, and guiding its management. ...
... Moreover, severe mucosal involvement and mild skin rash induced from Mycoplasma pneumoniae infection, called Mycoplasma induced-rash and mucositis (MIRM), is also uncommon (Poddighe & Bruni, 2017). MIRM is characterized by non-specific skin rashes and remarkable mucosal lesions; these often involve the oral cavity and are very painful, steroid therapy in addition to macrolides may shorten the clinical course (Pereyre et al., 2016). ...
This is an unusual and rare case of plasma cell mucositis in Thai patient. The lesions were very difficult to treat from many medications. However, lobulated tongue lesion showed good response to potent topical steroid- fluocinolone acetonide 0.1% in solution. Challenge in diagnosis and treatment of this case are discussed.
Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a rare disease, which has not been reported in northern China previously. We retrospectively analyzed the clinical characteristics, diagnosis and treatment of 10 cases of MIRM in order to help clinicians to identify MIRM and to distinguish it from the similar mucositis and cutaneous characteristics of Stevens-Johnson syndrome. All 10 children included in the study had MIRM with skin and mucosal symptoms, but the characteristics of the skin and mucosal lesions differed by age. Most of the older children had sparse erythema and a vesicular rash, but the younger children had dense erythema without blisters but with purulent exudation. The mucositis was relatively mild in the younger children. The erythrocyte sedimentation rate, the levels of C-reactive protein, lactate dehydrogenase, and D-dimer were significantly elevated in most children with MIRM. Concomitant treatment of glucocorticoids and/or IVIG with macrolides may shorten the duration of fever and accelerate the clinical recovery. Additional case reports are needed to improve knowledge of the characteristics of MIRM and its response to therapy.
INTRODUCTION
Mycoplasma pneumoniae (MP) is a common respiratory pathogen that can result in community-acquired pneumonia (CAP). Approximately 25% of patients diagnosed with MP experience extrapulmonary manifestations. Mycoplasma-induced rash and mucositis (MIRM) was coined as a unique disease process in 2014. MIRM has prominent mucositis with or without a characteristic vesiculobullous and/or atypical targetoid eruption. Appropriate identification of this disease is important because it has a milder disease course with low rates of sequelae, and lower mortality compared to Stevens-Johnson syndrome, erythema multiforme, and toxic epidermal necrolysis. The objective of this systematic review was to examine the English literature on Mycoplasma Pneumonia-induced rash and mucositis since the establishment of its diagnosis in 2014.
METHODS
The following online databases were used to identify appropriate studies that met the established inclusion and exclusion criteria: Pubmed, Cochrane, MedLine, Health Evidence, EPPI center, Allied Health Evidence. The following MesH search terms were used to further identify articles; “Mycoplasma pneumoniae induced rash and mucositis,” “Mycoplasma pneumoniae rash and mucositis,” “Mycoplasma pneumoniae rash,” “Mycoplasma pneumoniae mucositis,” “MIRM,” “Mycoplasma induced rash and mucositis,” “Mycoplasma rash and mucositis,” “Mycoplasma rash,” and “Mycoplasma mucositis.” Data was extracted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
One hundred and seventy-five records were initially screened, and nineteen studies were included in the review, leading to a total of 27 patients. Patients had a mean age of 16 years old (Range 4 - 46 years old), with the majority being males (74%). Pulmonary symptoms tended to precede extrapulmonary symptoms on an average of 7.8 days. Extrapulmonary symptoms consisted of oral lesions (96.3%) followed by ocular lesions (92.6%) and genital lesions (59.3%). Female patients were more likely to have genital lesions (71.4%) when compared with male patients (55%). Cutaneous rashes occurred in approximately one-half of the patients, which supports the theory that MIRM is a separate clinical entity from SJS and other related skin disorders. Confirmatory testing for MIRM was performed using IgM/IgG Mycoplasma antibody testing or PCR in 19 (66.7%) and 6 (22.2%) patients respectively, although four cases reported the use of both serology and PCR, while five did not report confirmatory testing. Systemic antibiotics were used frequently in treatment 22 patients (77.8%) and 27 (100%) of the patients received various supportive care. Approximately 11 (37%) patients of reported cases used systemic steroids to reduce systemic inflammation. Other systemic treatments were used in six (21.4%) cases, and included intravenous immunoglobulins and cyclosporine A. Only eight patients (22.2%) reported having any lasting sequelae.
CONCLUSION
Mycoplasma-induced rash and mucositis is a recently described extra-pulmonary
manifestation of Mycoplasma pneumoniae infections. To the best of the authors’
knowledge, this is the first systematic review of the MIRM literature since the
introduction of the diagnosis in 2014. The authors hope that this review can serve to better our current understanding and lead to improved identification, work-up, and treatment of this disease. One notable limitation of this study is the relatively small sample size, which is due to the recent introduction of the term.
M. pneumoniae‐induced rash and mucositis (MIRM), proposed as a unique entity apart from erythema multiforme (EM), Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) by Canavan et al. in 2015, follows a less severe course and is associated with less morbidity and mortality.1,2 In SJS and TEN, widespread, confluent skin lesions, positive Nikolsky sign, and significant epidermal detachment are present.3 In MIRM, mucositis, especially oral, is nearly universal (94%) and predominant over a less marked or absent cutaneous involvement.1,4 It is a major cause of morbidity, may lead to multiple confluent erosions, hemorrhagic crusting, and sparse vesiculobullous perilabial eruptions, and, rarely, to sequelae such as mucosal synechiae and pigmentary changes.
Clinical Vignette Poster
Mycoplasma pneumoniae is a common cause of respiratory infections in children, but sometimes extra-pulmonary diseases can be observed. The immunological mechanisms involved in these extra-respiratory complications are unknown. Here, we report a small case series of Mycoplasma-related diseases including 5 children who developed: i) aseptic meningitis; ii) urticarial rash and pericardial effusion; iii) pleural effusion with severe eosinophilia; iv) Stevens-Johnson syndrome; v) multiform erythema. Interestingly, all children were moderately to highly atopic, as a common immunologic feature.
Background:
the etiologic diagnosis of pediatric chronic urticaria is quite challenging, as a minority of cases can be associated to specific triggers. Thus, more than 50% of chronic urticaria in children are labeled as idiopathic. Several evidences supported an autoimmune pathogenesis in 30-40% of patients with idiopathic (or spontaneous) chronic urticaria in adults, where the diagnosis of Autoimmune Chronic Urticaria should include in vivo and in vitro tests, revealing the presence of autoantibodies against high-affinity IgE receptors mainly, as stated by the majority of guidelines.
Objective:
in this review, we aimed at collecting and analyzing all the available evidences on the diagnosis and treatiment of autoimmune chronic urticaria in children, including most recent development and patents.
Results and conclusion:
Most pediatric studies relied on autologous serum skin test only, in order to evidence autoimmune urticaria, which make such a diagnosis be incomplete. A complete diagnostic assessment of pediatric autoimmune chronic urticaria, demonstrating an antibody-mediated mechanism, could ameliorate the therapeutical management, supporting the use of omalizumab rather than other immuno-suppressive therapy in cases resistant to the first-line treatments.
Mycoplasma pneumoniae infection is associated with extrapulmonary complications, including mucocutaneous eruptions. These eruptions, which have been termed either "Stevens-Johnson syndrome" or "erythema multiforme" in the literature, may differ from drug-induced Stevens-Johnson syndrome or viral-associated erythema multiforme.
We sought to review the literature characterizing morphology and disease course of M pneumoniae-associated mucocutaneous disease.
A comprehensive literature search identified 95 articles with 202 cases.
Patients were often young (mean age: 11.9 years) and male (66%). Cutaneous involvement ranged from absent (34%), to sparse (47%), to moderate (19%). Oral, ocular, and urogenital mucositis was reported in 94%, 82%, and 63% of cases, respectively. Treatments included antibiotics (80%), systemic corticosteroids (35%), supportive care alone (8%), and/or intravenous immunoglobulin (8%). Complications included mucosal damage (10%), cutaneous scarring (5.6%), recurrence (8%), and mortality (3%).
Mild cases may not have been published; thus this review may have a bias toward more severe disease.
M pneumoniae-associated mucocutaneous disease has prominent mucositis and sparse cutaneous involvement, although cutaneous involvement varies. Because of the distinct morphology, mild disease course, and potentially important clinical implications regarding treatment, we propose a revision of the nomenclature system and suggest the term "Mycoplasma-induced rash and mucositis" for these cases.
Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.