Urogynecologic complications in Stevens-Johnson syndrome and toxic epidermal necrolysis: Presentation of a case and recommendations for management

Petukhova, Tatyana A.; Maverakis, Emanual; Ho, Bruce Kuo Ting; Sharon, Victoria R.

doi:10.1016/j.jdcr.2016.03.006

Cited by 8 publications

(14 citation statements)

References 6 publications

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“…28 Acute involvement includes vaginal erosions and ulcerations, dysuria, urinary retention, and vaginal discharge, pain, and bleeding. 47 There are limited data on effective interventions, but acute gynecologic care may help mitigate the chronic sequelae of vulvar adhesions and vaginal stenosis. These sequelae can occur in up to 25% of survivors and cause dyspareunia, chronic pain and bleeding, and difficulty conceiving.…”

Section: Gynecologic Involvementmentioning

confidence: 99%

Multidisciplinary care in Stevens-Johnson syndrome

Shanbhag

Chodosh

Fathy

et al. 2020

Therapeutic Advances in Chronic Disease

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Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are potentially fatal mucocutaneous diseases that can involve many organ systems. Manifestations of SJS/TEN outside of the skin, eyes, and oral mucosa are not well defined or well recognized, and, therefore, are often not addressed clinically. As supportive care improves and mortality from SJS/TEN decreases, chronic complications in affected organ systems are becoming more prevalent. Recognition of the manifestations of SJS/TEN in the acute phase is critical to optimal care. In this review, we review the organ systems that may be involved in SJS/TEN, provide an overview of their management, and propose a list of items that should be communicated to the patient and family upon discharge. The organ systems discussed include the pulmonary, gastrointestinal/hepatic, oral, otorhinolaryngologic, gynecologic, genitourinary, and renal systems. In addition, the significant psychosocial, nutritional, and pain consequences and management of SJS/TEN are discussed.

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Section: Gynecologic Involvementmentioning

confidence: 99%

Multidisciplinary care in Stevens-Johnson syndrome

Shanbhag

Chodosh

Fathy

et al. 2020

Therapeutic Advances in Chronic Disease

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“…While urogynecologic sequelae have been documented across many case reports and series, including vaginal synechiae and occlusion leading to hydrocolpos, hematocolpos, hematometra, dysuria, and dyspareunia, the literature on preventative techniques to guide acute phase management of vulvovaginal SJS/TEN is sparse. [3][4][5][7][8][9][10][11][12] Long-term sequelae of vulvovaginal SJS/TEN can be devastating for patients, and surgical intervention to reverse complications including vaginal occlusion and labial synechiae are typically only partially effective, especially for treating pain. 3,13 Thus, a preventative approach including a clear treatment regimen during the acute phase of vulvovaginal SJS/TEN should be employed.…”

Section: Discussionmentioning

confidence: 99%

“…Our management strategy is consistent with the findings published in a recent study in the Journal of the American Academy of Dermatology based on current available literature and expert opinion. 7,8 Special considerations should be given specifically to pediatric and pregnant patient populations. Despite these limitations, this study is the largest series to evaluate treatment and outcomes of vulvovaginal SJS/TEN.…”

Section: Discussionmentioning

confidence: 99%

“…In conclusion, early gynecologic consultation including pelvic examination and treatment with topical estrogen and ultrapotent topical corticosteroids, along with menstrual suppression, is recommended to decrease urogynecologic sequelae of vulvovaginal SJS/TEN. 7,8,26,27 Bimanual and speculum examination to assess for syncytia should be performed, however, full pelvic examination may at times be impractical due to patient discomfort and positioning difficulties in severely ill or intubated patients. If full pelvic examination with speculum and bimanual is not feasible or too uncomfortable, we recommend consideration of intravaginal treatment as almost half (42.1%) of patients in this study had intravaginal involvement.…”

Section: Discussionmentioning

confidence: 99%

“…Prospective studies are lacking, limited retrospective studies exist, and the manuscripts that provide recommendations for acute management of vulvovaginal involvement in SJS/TEN are based on literature reviews and expert opinions. 7,8 In this study, we aimed to characterize the frequency of vulvovaginal involvement in SJS/TEN at our institution and evaluate patient characteristics, treatment modalities, and outcomes. Additionally, we present a comprehensive treatment algorithm based on our institution's protocol for specific management of vulvovaginal involvement in SJS/TEN in the adult, pediatric, and pregnant patient populations.…”

Section: Introductionmentioning

confidence: 99%

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Vulvovaginal involvement in Stevens‐Johnson syndrome and toxic epidermal necrolysis: management and techniques used to reduce gynecologic sequelae

et al. 2021

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Background Vulvovaginal involvement in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is common, likely underdiagnosed, and can result in severe sequelae if not managed acutely. There are few studies on acute management of vulvovaginal SJS/TEN. Current recommendations are predominantly based on expert opinion. We aimed to determine the frequency of vulvovaginal involvement in SJS/TEN at a single institution, identify treatment modalities, and assess outcomes at a tertiary care burn center.Methods This is a retrospective review of vulvovaginal SJS/TEN cases between 2009 and 2019. Demographic and clinical data including exam findings, treatment regimens, and outpatient follow-up were collected from the electronic medical record.Results Vulvovaginal involvement was observed in 12.7% (19/149) of cases of female patients with SJS/TEN. The mean age was 38.7 years (SD 23.6), and 21% (4/19) of patients were pediatric (age 9-18). Vulvar involvement was seen in 47.3% (9/19), and vulvar plus vaginal involvement was reported in 42.1% (8/19). Treatment regimens were variable until 2017, at which time institutional guidelines were implemented including application of ultrapotent topical steroid, vaginal estrogen, and menstrual suppression.Gynecology follow-up occurred in 15.7% (3/19) of cases. One complication of superficial vaginal agglutination was noted and was successfully treated in the office with blunt dissection. ConclusionThe most common treatment modalities employed at our institution included application of ultrapotent topical steroid, vaginal estrogen, and menstrual suppression.While follow-up was limited for our patient population, we propose an algorithm to prevent long-term sequalae of vulvovaginal SJS/TEN. Gynecologic surveillance is recommended to reduce urogynecologic sequelae.

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Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Lerch

Mainetti

Beretta‐Piccoli

et al. 2017

Clinic Rev Allerg Immunol

256

230

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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a delayed-type hypersensitivity reaction to drugs. They represent true medical emergencies and an early recognition and appropriate management is decisive for the survival. SJS/TEN manifest with an "influenza-like" prodromal phase (malaise, fever), followed by painful cutaneous and mucous membrane (ocular, oral, and genital) lesions, and other systemic symptoms. The difference between SJS, SJS/TEN overlap, and TEN is defined by the degree of skin detachment: SJS is defined as skin involvement of < 10%, TEN is defined as skin involvement of > 30%, and SJS/TEN overlap as 10-30% skin involvement. The diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology. The mortality rates for SJS and TEN have decreased in the last decades. Today, the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is available for SJS/TEN severity assessment. Drugs with a high risk of causing SJS/TEN are anti-infective sulfonamides, anti-epileptic drugs, non-steroidal anti-inflammatory drugs of the oxicam type, allopurinol, nevirapine, and chlormezanone. Besides conventional drugs, herbal remedies and new biologicals should be considered as causative agents. The increased risk of hypersensitivity reactions to certain drugs may be linked to specific HLA antigens. Our understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytotoxicity mechanisms were clarified. However, many factors contributing to epidermal necrolysis still have to be identified, especially in virus-induced and autoimmune forms of epidermal necrolysis not related to drugs. In SJS/TEN, the most common complications are ocular, cutaneous, or renal. Nasopharyngeal, esophageal, and genital mucosal involvement with blisters, erosions as well as secondary development of strictures also play a role. However, in the acute phase, septicemia is a leading cause of morbidity and fatality. Pulmonary and hepatic involvement is frequent. The acute management of SJS/TEN requires a multidisciplinary approach. Immediate withdrawal of potentially causative drugs is mandatory. Prompt referral to an appropriate medical center for specific supportive treatment is of utmost importance. The most frequently used treatments for SJS/TEN are systemic corticosteroids, immunoglobulins, and cyclosporine A.

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Urogynecologic complications in Stevens-Johnson syndrome and toxic epidermal necrolysis: Presentation of a case and recommendations for management

Cited by 8 publications

References 6 publications

Multidisciplinary care in Stevens-Johnson syndrome

Multidisciplinary care in Stevens-Johnson syndrome

Vulvovaginal involvement in Stevens‐Johnson syndrome and toxic epidermal necrolysis: management and techniques used to reduce gynecologic sequelae

Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

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