Abstract:Key Clinical MessageFuchs syndrome is a milder form of the Stevens–Johnson syndrome (SJS) spectrum with only mucosal involvement which can be triggered by Mycoplasma pneumonia (MP) infection. Treatment should be directed toward supportive care including ocular and mucous membrane care, fluids and nutritional support, and pain control. In addition, antibiotic and immunomodulatory treatments are discussed for this entity.
“…El Mp es una causa común de neumonía extrahospitalaria, con manifestaciones mucocutáneas infrecuentes (25%) que tienden a afectar en su mayoría al sexo masculino, niños y adultos jóvenes [4][5][6][7][8][9] .…”
Section: Discussionunclassified
“…La afectación cutánea, a diferencia del EM mayor y SSJ, suele ser escasa, y de localización acral y está ausente en un tercio de los casos, lo que sugiere MIRM, ya que en la actual clasificación de EM-SSJ/NET no existe una categoría que contemple esta situación 2,6,11 . Las lesiones cutáneas más frecuentes son las vesículas o ampollas (77%) seguidas de las lesiones en diana (48%).…”
RESUMEN Una de las manifestaciones dermatológicas relacionadas a la infección por Mycoplasma pneumoniae es el síndrome de Stevens-Johnson (SSJ) donde predomina la mucositis sobre la afectación cutánea, por este motivo ha sido considerado como una nueva entidad separada del espectro del SSJ denominada "Exantema mucocutáneo inducido por Mycoplasma" (MIRM), por sus características clínicas y pronósticas más benignas. A pesar de ello, aún existe controversia si el MIRM es una entidad independiente del SSJ o es una forma más leve dentro del espectro de esta enfermedad. Se describe un caso clínico con el cual se discute esta nueva clasificación. PALABRAS CLAVE: Mycoplasma pneumoniae, mucositis, síndrome de Stevens-Johnson (DeCS) SUMMARY One of the dermatological manifestations related to the infection by Mycoplasma pneumoniae is Stevens-Johnson syndrome (SJS) where mucositis predominates over skin involvement, for this reason, it has been considered as a new entity, separate from the spectrum of SJS, called "Mucocutaneous Rash induced by Mycoplasma "(MIRM), due to its more benign clinical and prognostic characteristics. Despite this, there is still controversy whether MIRM is an independent entity from SJS or is a milder form within the spectrum of this disease. A clinical case is described with which this new classification is discussed.
“…El Mp es una causa común de neumonía extrahospitalaria, con manifestaciones mucocutáneas infrecuentes (25%) que tienden a afectar en su mayoría al sexo masculino, niños y adultos jóvenes [4][5][6][7][8][9] .…”
Section: Discussionunclassified
“…La afectación cutánea, a diferencia del EM mayor y SSJ, suele ser escasa, y de localización acral y está ausente en un tercio de los casos, lo que sugiere MIRM, ya que en la actual clasificación de EM-SSJ/NET no existe una categoría que contemple esta situación 2,6,11 . Las lesiones cutáneas más frecuentes son las vesículas o ampollas (77%) seguidas de las lesiones en diana (48%).…”
RESUMEN Una de las manifestaciones dermatológicas relacionadas a la infección por Mycoplasma pneumoniae es el síndrome de Stevens-Johnson (SSJ) donde predomina la mucositis sobre la afectación cutánea, por este motivo ha sido considerado como una nueva entidad separada del espectro del SSJ denominada "Exantema mucocutáneo inducido por Mycoplasma" (MIRM), por sus características clínicas y pronósticas más benignas. A pesar de ello, aún existe controversia si el MIRM es una entidad independiente del SSJ o es una forma más leve dentro del espectro de esta enfermedad. Se describe un caso clínico con el cual se discute esta nueva clasificación. PALABRAS CLAVE: Mycoplasma pneumoniae, mucositis, síndrome de Stevens-Johnson (DeCS) SUMMARY One of the dermatological manifestations related to the infection by Mycoplasma pneumoniae is Stevens-Johnson syndrome (SJS) where mucositis predominates over skin involvement, for this reason, it has been considered as a new entity, separate from the spectrum of SJS, called "Mucocutaneous Rash induced by Mycoplasma "(MIRM), due to its more benign clinical and prognostic characteristics. Despite this, there is still controversy whether MIRM is an independent entity from SJS or is a milder form within the spectrum of this disease. A clinical case is described with which this new classification is discussed.
“…Previously, this clinical condition was classified within the epidermolytic dermopathy spectrum as "atypical SJS, " "incomplete SJS, " or "Fuchs syndrome" (5,10,11,14,15). Recently, a systematic review concluded that this condition was a distinct entity called Mycoplasma pneumoniae-induced rash and mucositis (MIRM) (11,(14)(15)(16)(17). However, no patient with MIRM has been reported in China until now, indicating that many clinicians had no knowledge of this disease and that such patients could be misdiagnosed.…”
Section: Discussionmentioning
confidence: 99%
“…Mycoplasma -induced rash and mucositis is rare. Previously, this clinical condition was classified within the epidermolytic dermopathy spectrum as “atypical SJS,” “incomplete SJS,” or “Fuchs syndrome” ( 5 , 10 , 11 , 14 , 15 ). Recently, a systematic review concluded that this condition was a distinct entity called Mycoplasma pneumoniae -induced rash and mucositis (MIRM) ( 11 , 14 – 17 ).…”
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.
“…SJS is thought to fall within a spectrum of diseases that affect the skin and mucous membranes, including erythema multiforme minor, erythema multiforme major (or SJS), and toxic epidermal necrolysis [6, 10]. Mycoplasma pneumonia can be associated with isolated mucous membrane disease or in combination with skin involvement [11]. Many authors believe that M. pneumoniae -associated mucositis with the minimal or absence of skin lesions is a separate entity from SJS, labelled as atypical SJS or M. pneumoniae -induced rash and mucositis [3, 12].…”
Stevens–Johnson syndrome is a medical emergency which is characterized by skin and mucosal reaction to the use of certain drugs. Atypical Steven–Johnson syndrome can occur due to various microorganisms and Mycoplasma pneumoniae being one of them. We present a clinical course, diagnosis, and successful management of Steven–Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap due to Mycoplasma pneumoniae in a 17-year-old Nepalese female. In the resource-limiting country and hospitals where serology and PCR for M. pneumoniae is not easily accessible, a simple bedside cold agglutination test can be done to increase the suspicion of infectious cause (most common M. pneumoniae ) of SJS-TEN overlap. M. pneumoniae infection should be considered in all cases of mucositis, especially in patients having preceding respiratory tract infections (tracheobronchitis).
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