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.

Pediatric toxic epidermal necrolysis treated successfully with infliximab.

Successful management of toxic epidermal necrolysis (TEN) with tumor necrosis factor-α inhibitors has been described in adults. We present a case of a 7-year-old boy with infection-associated TEN, diagnosed by typical clinical and histopathological features, most likely caused by Mycoplasma pneumoniae. Treatment with a single dose of infliximab 5 mg/kg intravenously on day 5 after the onset of symptoms was followed by cessation of all blister formation over 3 days and complete resolution within a week. Sequelae were mild, consisting of postinflammatory hyperpigmentation and dry eyes.

[Drug-induced toxic epidermal necrolysis with secondary aspergillus fumigatus infection: a case report].

Among the various drug induced dermatological entities toxic epidermalnecrolysis (TEN) and Stevens-Johnson syndrome (SJS) occupy a primary place in terms of mortality. Toxic epidermal necrolysis also known as Lyell's syndrome was first described by Lyell in 1956. Drugs are by far the most common cause of toxic epidermal necrolysis, in which large sheets of skin are lost from the body surface making redundant the barrier function of the skin, with its resultant complications. Drug-induced toxic epidermal necrolysis are severe adverse cutaneous drug reactions to various precipitating agents that predominantly involve the skin and mucous membranes. Toxic epidermal necrolysis is rare but considered medical emergencies as they are potentially fatal. Drugs are the most common cause accounting for about 65%-80% of the cases. The most common offending agents are sulfonamides, NSAIDs, butazones and hydrantoins. An immune mechanism is implicated in the pathogenesis, but its nature is still unclear. There is a prodormal phase in which there is burning sensation all over the skin and conjunctivae, along with skin tenderness, fever, malaise and arthralgias. Early sites of cutaneous involvement are the presternal region of the trunk and the face, but also the palms and soles, rapidly spread to their maximum extent, the oral mucosa and conjunctiva being affected. Initial lesions are macular, followed by desquamateion, or may be from atypical targets with purpuriccenters that coalesce, from bullae, then slough. The earlier a causative agent is withdrawn the better is the prognosis. Several treatment modalities given in addition to supportive care are reported in the literature, such as systemicsteroids, high-dose intravenous immunoglobulins, ciclosporin, TNF antagonists. Recovery is slow over a period of 14-28 days and relapses are frequent. Mortality is 25%-50% and half the deaths occur due to secondary infection. Here we report a 50-year-old female of drug-induced toxic epidermal necrolysis. She was admitted to the dermatology ward with extensive peeling of skin over the trunk and limbs. She had taken alamotrigine for epilepsy. A week after taking the tablets, the patient developed a severe burning sensation all over the body and followed by a polymorphic erythematous dermatitis and widespread peeling of skin. We treated this patient with high dose corticosteroids, high-dose intravenous immunoglobulins and etanercept, but eventually she died of secondary aspergillus fumigatus infection.

Microbiological findings and antibacterial therapy in Stevens-Johnson syndrome/toxic epidermal necrolysis patients from a Swedish Burn Center.

BACKGROUND: Superimposed infections/sepsis are the major cause of morbidity/mortality in Stevens-Johnson syndrome/Toxic Epidermal Necrolysis (SJS/TEN). It is a delicate balance between avoiding new pharmaceuticals and prophylactically treat an incipient infection. The objective of this study was to investigate the rates and types of infection-microbials and antibiotics involved in SJS/TEN patients. MATERIALS AND METHODS: Microbiology and clinical data were collected for SJS/TEN patients admitted to our Burn Center from January 2010 through January 2016. RESULTS: A total of 24 patients were admitted over the study period. There were 303 bacterial cultures taken whereof 113 (37.3%) were positive (median of 4.4 per patient). Twenty-two (91.7%) patients had at least 1 positive sample recorded. Fifteen (62.5%) patients had a confirmed episode of sepsis with skin being the most common source of colonization (77.8%). Eleven (45.8%) patients received empiric antibiotic therapy at referral facility/prior to admission to our Center. Patients who grew a higher number of different species were significantly less likely to have received early empiric antimicrobial therapy (P < .001). CONCLUSION: Secondary bacterial infection and sepsis were a highly common finding in our patient population. Despite the risk of resistance and further immunological provocation, empirical antibiotic treatment might have a place in clinical management.

What's new with common, uncommon and rare rashes in childhood.

PURPOSE OF REVIEW: Children with rashes account for many of the outpatient visits to a general pediatrician. As such, pediatricians are often the first to identify and treat these rashes. Establishing an approach to common, uncommon and rare pediatric rashes assists in accurate assessment. This review highlights newly identified clinical patterns and disease severity. RECENT FINDINGS: Group A ß-hemolytic streptococci (GABHS) have been shown to be an important cause of intertrigo and to cause more widespread disease in some instances. Superficial skin infections with GABHS have been associated with strains secreting exfoliating toxins, whereas deeper infections have been associated with superantigen toxins. Hand-foot-and-mouth disease (HFMD) outbreaks have occurred with more virulent strains, causing more widespread disease that may be confused with eczema herpeticum or varicella. Mycoplasma pneumoniae has been shown to be an important cause of common disorders such as urticaria, and less common disorders such as Stevens-Johnson syndrome and Mycoplasma-associated mucositis. Recurrent toxin-mediated erythema is a recently described entity that must be differentiated from Kawasaki disease. SUMMARY: The number of rashes acquired in childhood is vast, requiring the pediatrician to be able to identify worrisome rashes from those with a more benign course. Key clinical signs may assist in clinical diagnosis and treatment.

Mycoplasma pneumonia--associated mucocutaneous disease in children: dilemmas in classification.

There is controversy regarding precise definitions for Stevens-Johnson syndrome (SJS) and erythema multiforme (EM) major because of overlap in clinical presentations. SJS and EM major associated with Mycoplasma pneumoniae have been reported to occur in children, but Mycoplasma is more commonly reported with SJS. We sought to further characterize Mycoplasma-associated mucocutaneous disease. Through retrospective chart review over 10 years, six children hospitalized with a diagnosis of SJS who also tested positive for Mycoplasma infection were reviewed. Using documented physical examinations and photographs, diagnoses of SJS or EM major were retrospectively made based upon cutaneous lesional morphology employing the classification system proposed by Bastuji-Garin et al. The majority of patients were boys, with limited acral cutaneous lesions. All patients required prolonged hospitalization because of mucosal involvement and had good short-term outcomes. When the classification system was retrospectively applied, five of the six patients were reclassified with a diagnosis of EM major instead of SJS. Children with Mycoplasma-associated EM major and SJS in our small retrospective series appeared to have significant mucosal involvement but more limited cutaneous involvement with lesional morphology, which is more characteristic of EM major.

Mycoplasma-associated Stevens-Johnson syndrome in children: retrospective review of patients managed with or without intravenous immunoglobulin, systemic corticosteroids, or a combination of therapies.

Administration of intravenous immunoglobulin (IVIG) to patients with Stevens-Johnson syndrome (SJS) has been controversial. The objective of this study was to evaluate the effectiveness of IVIG, systemic corticosteroids, or both in treating Mycoplasma pneumoniae-associated SJS (mpSJS). Retrospective series of 10 pediatric mpSJS cases were stratified into four treatment groups: IVIG alone, IVIG and systemic corticosteroids together, systemic corticosteroids alone, and supportive care. The efficacy of therapy was evaluated on the basis of several proxies of disease severity, including hospital length of stay (LOSt ) and number of febrile days (Febt ) after initiation of therapy. Patients treated with IVIG alone had a longer LOSt and more Febt , despite different baseline characteristics, than patients treated with supportive therapy. Of patients who received IVIG, 50% were treated with corticosteroids concurrently and had similar characteristics of disease severity but showed a non-statistically significant trend toward shorter LOSt and fewer Febt than those who received IVIG alone. A patient treated with corticosteroids alone had the shortest LOSt in this series. Therefore treatment with IVIG alone was associated with a more severe disease course than supportive therapy, although causality cannot be inferred given possible confounding by indication. When systemic corticosteroids were used alone or in conjunction with IVIG, hospital LOSt and Febt trended lower than with the use of IVIG alone, although disease severity at baseline was similar between those treated with IVIG and corticosteroids concurrently and those treated with IVIG alone. It was thus concluded that treatment with systemic corticosteroids as monotherapy or in combination with IVIG may be preferable to IVIG alone. Further large-scale studies are warranted to evaluate this hypothesis.

Lid Margin Microbiome in Stevens-Johnson Syndrome Patients With Lid Margin Keratinization and Severe Dry Eye Disease.

Purpose: The current study evaluated the lid margin microbiome of keratinized lid margins of patients with chronic Stevens-Johnson syndrome (SJS) and compared it with healthy controls and historically reported lid margin microbiome of patients with meibomian gland dysfunction (MGD). Methods: Eyelid margin swabs of 20 asymptomatic adults (mean age = 29 ± 12 years) and 10 patients with chronic SJS (mean age = 31.2 ± 14 years) with lid margin keratinization were sequenced using next generation of 16S rDNA V3 to V4 variable region. Within SJS, the keratinized lid margin microbiome was compared with adjacent eyelid skin. Results: All patients had obstructive MGD, and mean Schirmer I value was 2.8 ± 1.9 mm. The phyla were similar in two groups, whereas at the genera level, an increase in the relative abundance of Corynebacterium, Haemophilus, Azotobacter, and Afipia and a decrease of Acinetobacter was noted in SJS compared to healthy lid margins. SJS-associated microbiota displayed lesser diversity and more heterogeneity than healthy controls. The Principal Components Analysis (PCA) plot revealed wide separation in the SJS and the control groups. Correlational network analysis revealed Corynebacterium and Sphingomonas forming a major hub of negative interactions with other bacterial genera in the SJS group. Significant differences exist in the prevalent genera between keratinized lid margins and historically reported meibum microbiome of patients with MGD. In addition, the eyelid skin of patients with SJS had predominant Staphylococcus, whereas Corynebacterium and Pseudomonas were more in the keratinized lid margins compared to the eyelid skin microbiome. Conclusions: Lid margin microbiome is significantly altered in the keratinized lid margins of patients with SJS compared to the eyelid skin of patients with SJS, normal lid margins, and patients with MGD.

Mycoplasma pneumoniae-induced recurrent Stevens-Johnson syndrome in children: a case report.

Mycoplasma pneumoniae, the major pathogen of primary atypical pneumonia, is reported as the most common infectious agent associated with Stevens-Johnson syndrome (SJS) in children. For that reason it is important to consider mycoplasma infection also in the absence of classical pulmonary symptoms. SJS is a rare and acute, self-limited disease, characterized by severe inflammation and necrosis of two or more mucous membranes. We report the case of a 12-year-old boy with a diagnosis of SJS induced by M. pneumoniae infection. The patient's SJS relapsed 8 months after discharge. When the condition is recurrent, it is important early on to identify the cause of a single episode to optimize care and therapeutic choices.

[Mycoplasma pneumoniae and Stevens-Johnson syndrome]. / Nuoren miehen Mycoplasma pneumoniae-infektio ja limakalvoille rajoittunut Stevens-Johnsonin oireyhtymä.

Mycoplasma pneumoniae causes up to 10-40 % of community-acquired pneumonias. The incidence of M. pneumoniae pneumonia is greatest among children and young adults. The symptoms of M. pneumoniae upper and lower respiratory infections are usually mild and often self-limited. The most frequent extrapulmonary complications present in CNS, heart and skin. The skin affiliations are usually transient erythematous maculopapular or vesicular rashes but may sometimes evolve into Stevens-Johnson syndrome. M. pneumoniae is one of the most common microbe behind the infectious causes of SJS. We present a patient who developed incomplete Stevens-Johnson syndrome concomitant of Mycoplasma pneumoniae pneumonia.

Severe Mycoplasma pneumoniae-associated mucositis treated with immunoglobulins.

UNLABELLED: Mycoplasma pneumoniae-associated mucositis (MPAM), previously labelled as atypical Stevens-Johnson syndrome (SJS), SJS with minimal or no skin manifestations, is a rare non-respiratory manifestation of Mycoplasma pneumoniae infection. The nineteen cases described so far in children and young adults were characterized by a high male gender prevalence (16/19) and a good response to appropriate antibiotic treatment and supportive care in the majority of patients. We describe a case of MPAM in a previously healthy girl, who improved after a 0.5 g/kg daily dose of intravenous immunoglobulins (IVIG) for four consecutive days, after traditional therapy had failed. CONCLUSION: The successful treatment with IVIG described in this report suggests that, where appropriate antibiotic and supportive therapy fails to improve the clinical course of severe MPAM, IVIG treatment is worth considering.

Statistical analysis of Stevens-Johnson syndrome caused by Mycoplasma pneumonia infection in Japan.

BACKGROUND: Stevens-Johnson syndrome (SJS) associated with Mycoplasma pneumoniae (M. pneumoniae) infection is mainly observed in children. In adults, drugs are a major cause of SJS, but some adult patients with SJS are infected with M. pneumoniae. We analyzed patients with SJS associated with M. pneumoniae infection to elucidate the differences between drug-induced SJS and M. pneumoniae-associated SJS and also to study differences between M. pneumoniae-associated SJS in children and adults. METHODS: This is a retrospective review of Japanese patients who have been reported as M. pneumoniae-associated SJS in medical Journals published from 1981 to 2009, compared with data of Japanese patients with drug-induced SJS reported from 2000 to 2009. RESULTS: Thirty-eight cases of M. pneumoniae-associated SJS and 78 cases of drug-induced SJS were analyzed in this study. Ocular lesions were observed more frequently in M. pneumoniae-associated SJS than in drug-induced SJS (p < 0.01), and adult patients showed a higher ratio of sequelae in their eyes than did patients under 20 years of age (p < 0.01). Sixty-six percent of adult patients with M. pneumoniae-associated SJS developed fever/respiratory symptoms and mucocutaneous lesions on the same day. In contrast, most of the patients under 20 years of age developed fever/respiratory symptoms before mucocutaneous involvement. This means that these adult patients were infected and immunized previously and developed allergic reactions to M. pneumoniae soon after the later infection. CONCLUSIONS: In order to prevent ocular sequelae in adult patients when M. pneumoniae infection is suspected, more intensive treatment may be needed in adult patients than in younger patients.

Diphtheroids as Corneal Pathogens in Chronic Ocular Surface Disease in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

PURPOSE: To characterize diphtheroid corneal infections in eyes in the chronic phase of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). METHODS: Observational case series. RESULTS: Four eyes of 3 patients were included in this review. Each eye presented with persistent corneal epithelial defect with corneal thinning in the chronic phase of SJS/TEN. None of the epithelial defects were associated with stromal infiltration. The corneas were cultured at the time of workup of persistent epithelial defect (3 eyes) or at time of tectonic penetrating keratoplasty after perforation (1 eye). Cultures yielded abundant growth of Corynebacterium spp., including Corynebacterium jeikeium (n = 2), Corynebacterium glucuronolyticum (n = 1), and a multidrug-resistant Corynebacterium striatum isolate (n = 1). The ocular surface was stabilized with surgical intervention (1 eye) or with introduction of fortified topical antibiotic based on laboratory identification and susceptibility testing of the isolated organisms (3 eyes). Numerous risk factors for microbial keratitis were present in all 4 eyes. CONCLUSIONS: In eyes with a persistent corneal epithelial defect in the chronic phase of SJS/TEN, even in the absence of an infiltrate, corneal culture should be undertaken. Recognition and treatment of Corynebacterium spp. as opportunistic pathogens may lead to favorable outcomes in cases of clinically sterile ulceration during the chronic phase of SJS/TEN.

Diff-Quik cytologic recognition of Chlamydophila psittaci in orolabial lesions of Stevens-Johnson Syndrome.

BACKGROUND: Chlamydophila psittaci causes psittacosis, an ornithosis acquired usually from infected birds. The disease is often focal and pneumonic but on rare instances can be protean and fatal. Diagnosis is by Chlamydophila serology, which may take as long as 21 days or more. The recovery of the organisms from mice, eggs or tissue culture inoculated with the patient's blood or sputum is tedious and dangerous for laboratory personnel. On occasion, C psittaci inclusion bodies have also been detected in infected cells by fluorescent antibody, Giemsa or Gimenez staining. This report describes heretofore not previously reported recognition of the causative organisms in Diff-Quik-stained clinical cytologic materials. CASE: A 17-year-old man presented with fever and sore throat, associated with Steven-Johnson syndrome, of 6 days' duration. In the touch and scrape smears of the orolabial mucosal lesions, C psittaci inclusion bodies were recognizable in Diff-Quik-stained but not with Papanicolaou-stained smears and Gram stain. There were few to numerous organisms per macrophage, which were enlarged or bloated and usually collared by polymorphonuclear leukocytes. The diagnosis was supported by a therapeutic trial with doxycycline and confirmed by a positive third serological tests for C psittaci 3 weeks after discharge. CONCLUSION: In a suspected or probable case of ornithosis, a rapid diagnosis of C psittaci inclusion bodies is possible in clinical cytology materials using Diff-Quik.

A retrospective analysis of infections and antibiotic treatment in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare drug-related life-threatening acute conditions. Infection is a major cause of morbidity and mortality in these patients. The aim of this study was to analyze the infective characteristics and antimicrobial strategies in patients with SJS and TEN.Methods: A total of 125 patients who were diagnosed with SJS/TEN in West China Hospital from 2010 to 2017 were retrospectively analyzed.Results: There were 75 patients with coinfections (75/125, 60%), of whom 44 had SJS (44/90, 48.9%) and 31 had TEN (31/35, 88.6%). The most common infections were skin infections and pulmonary infections. Staphylococcus aureus (S. aureus) and Escherichia coli (E. coli) were the most frequently identified pathogenic organisms. The most common antibiotics used in patients with infections were vancomycin, carbapenems, quinolones, macrolides, and lincomycin.Conclusions: Antimicrobial therapy should be administered promptly if there are clinical signs of an infection. Empiric antibiotic selection is based on knowledge of the local microbiota, the different infected sites, the pathogens involved, and the severity of disease.

Toxic dermatolysis, tissue necrosis and impaired wound healing due to sunitinib treatment leading to forefoot amputation.

Innovative treatment strategies in urologic oncology confront the treating physician with a new spectrum of adverse events. With growing understanding of underlying pathomechanisms, we need to identify contraindications against the use of certain antiproliferative drugs. The management of toxicities involves a multidisciplinary approach and thus, the exchange of experience across medical specialties is mandatory. We report a case of fulminant toxic dermatolysis, tissue necrosis and impaired wound healing resulting in the amputation of one forefoot after 6 days of treatment with sunitinib.

Extended septic thrombophlebitis in a patient with duplicated inferior vena cava: case report and review of literature.

Duplication is one of the congenital abnormalities of the inferior vena cava (IVC) and is reported to be associated with deep venous thrombosis (DVT). We report a case who was admitted for carbamazepine-induced toxic epidermal necrolysis. The patient had persistent fever caused by septic thrombophlebitis extending from the left femoral vein to the duplicated left IVC. The fever and thrombosis resolved under combined treatment with antibiotics and anticoagulants, without further complication of symptomatic pulmonary embolism. This is the first case in patient with IVC duplication complicated by DVT induced by septic thrombophlebitis, which was not seen in the nine cases of IVC duplication reported previously.

Microbial Keratitis in Stevens-Johnson Syndrome: A Prospective Study.

PURPOSE: To evaluate the microbiological profile and outcome in cases with infective keratitis in Stevens-Johnson syndrome (SJS). METHODS: Eighty-three eyes of 68 patients with SJS presenting with microbial keratitis were recruited and managed with standard antimicrobial therapy. RESULTS: Microbial keratitis developed in 34% of patients with SJS (83 eyes, 68 patients) over a period of 5 years. Four eyes (4.8%) had a history of concurrent topical steroid use at the onset of keratitis. Mean baseline best-corrected visual acuity was 1.8 ± 0.9 logMAR units. The site of corneal ulceration was central in 52 eyes (62.6%), paracentral in 17 eyes (20.5%), and peripheral in 14 eyes (16.8%). The mean ulcer area was 3.9 ± 2.7 mm. Approximately 15 of 24 (62.5%) culture-positive eyes had bacterial infection, most of which (80%) were caused by Gram-positive bacteria. Polymicrobial infection was noted in 7 of 24 eyes (29.1%). Although 57 of 83 (68.6%) eyes healed with medical therapy, 26 of 83 (31.3%) eyes had corneal perforation and were managed with cyanoacrylate glue application (30.7%) or therapeutic keratoplasty (69.3%). Systemic infection as an inciting factor of SJS and an early presentation for keratitis were the major risk factors associated with corneal perforation. Large mean ulcer size, paracentral ulcers, and punctal involvement were associated with a good visual outcome. CONCLUSIONS: Infective keratitis in SJS is common, and unlike routine cases, surgical intervention is often required. However, the antibiotic sensitivity pattern suggests that resistance is not that high.

Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome in a hematopoietic stem cell transplant recipient.

Stevens-Johnson syndrome (SJS) is not typically reported following hematopoietic stem cell transplant (HSCT). The most severe form of SJS, which is toxic epidermal necrolysis (TEN) has been reported following HSCT, albeit very rarely. We describe a case of Mycoplasma-associated SJS following HSCT. While this association is commonly reported in previously healthy children, it has not been reported in patients following HSCT.