Stevens-Jonhson syndrome associated with cytomegalovirus infection in a child with ependymoma

Abstract

Background: Stevens-Johnson syndrome is an uncommon, acute life-threatening disease characterized by extensive epidermal sloughing and mucositis. In childhood, as in adulthood, this condition is mostly related to drugs, in particular antibiotics. Only a few cases reported were firmly attributed to infectious agents, mainly Mycoplasma pneumonia but the causative role of infectious microorganisms seems particularly relevant in pediatric patients. The seriousness of this condition imposes a prompt recognition and the early withdrawal of the potential causative drugs or the institution of directed measures against infectious agents (depending on the suspected etiology), as well as a supportive and more specific therapy. Some treatments claim to halt the progression of skin detachment, but remain of unproven benefit due to the lack of prospective, well controlled, randomized clinical trials.Main Observations: We report a case of a 2-year-old boy admitted in our hospital for the treatment of an ependymoma of the posterior fossa, who developed a Stevens-Johnson syndrome associated most probably with a cytomegalovirus infection. He was successfully treated with high dose intravenous immunoglobulin and gancyclovir.Conclusion: To the best of our knowledge, this is the first case of SJS associated with CMV infection.

Two pediatric cases of influenza B‐induced rash and mucositis: Stevens‐Johnson syndrome or expansion of the Mycoplasma pneumoniae‐induced rash with mucositis (MIRM) spectrum?

“…The vast majority of cases of SJS in pediatric patients are due to infection, most commonly mycoplasma or HSV infection . However, a handful of other viruses have been implicated, including Epstein‐Barr virus (EBV), cytomegalovirus (CMV), human herpesvirus 6 (HHV6), hepatitis A, varicella‐zoster virus, and Merkel cell polyomavirus . The mechanisms underlying the development of SJS are complex and poorly understood.…”

“…The most common cause of SJS in pediatric patients is infection, classically due to mycoplasma or herpes simplex (HSV) . Although rare, other viruses have been associated with SJS . More recently Mycoplasma pneumoniae– associated rash and mucositis (MIRM) has replaced the previous M. pneumoniae ‐induced SJS nomenclature, and other infectious etiologies have been described .…”

Viral Infections and Cutaneous Drug-Related Eruptions

“…Similar observations have been made in SJS and TEN. These syndromes are most commonly caused by DHR rather than viruses (such as EBV, CMV, HHV-6, HSV, Varicella zoster virus, hepatitis A virus and HIV) ( Stutman, 1987 ; Werblowsky-Constantini et al, 1989 ; Lam et al, 2004 ; Bay et al, 2005 ; Pereira et al, 2007 ; Cruz et al, 2010 ; Wetter and Camilleri, 2010 ; Khalaf et al, 2011 ; Kunimi et al, 2011 ; Kim et al, 2012 ; Sotelo-Cruz, 2012 ; Ferrandiz-Pulido and Garcia-Patos, 2013 ; Irungu et al, 2017 ). In about 30% of cases of SJS and TEN, no causative drug is identified, and in 15%, drug responsibility is deemed unlikely ( Duong et al, 2017 ).…”

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

“…34 Regarding the broader spectrum of RIME, infections clearly implicated as triggers include Chlamydophila (Chlamydia) pneumoniae 13,35 and influenza B. 36,37 Occurrence of what is described as SJS has been linked less convincingly with other infections 30,[40][41][42][43][44][45][46] (Table 4). [38][39][40][41][42][43][44][45] Clinical presentation…”