Stevens-Johnson Syndrome: Clinical Presentation and Management

Introduction to Stevens-Johnson Syndrome

The American Academy of Dermatology notes that Stevens-Johnson Syndrome presents with a characteristic triad of painful skin lesions, severe mucosal involvement, and systemic symptoms following a flu-like prodrome, typically 4-28 days after drug exposure 1
The British Association of Dermatologists recommends distinguishing Stevens-Johnson Syndrome from staphylococcal scalded skin syndrome (SSSS) based on mucosal involvement and subepidermal rather than intraepidermal cleavage 2

The European Academy of Dermatology and Venereology states that fever, malaise, and upper respiratory tract symptoms precede the cutaneous eruption by several days 1, 2
Ocular inflammation may develop before skin signs appear, according to the American Academy of Ophthalmology 2
The American College of Emergency Physicians notes that cutaneous pain is a prominent early feature and should alert clinicians to incipient epidermal necrolysis 2
Initial presentation includes atypical target lesions, purpuric macules, and blisters, as described by the British Association of Dermatologists 1

The American Academy of Dermatology states that purulent keratoconjunctivitis and eyelid edema occur early in the acute phase 1
Persistent ocular inflammation with involvement of eyelid skin develops, according to the American Academy of Ophthalmology 1
The European Academy of Dermatology and Venereology notes that ocular inflammation may precede skin manifestations 2
Erosive and hemorrhagic mucositis affecting the mouth is described by the British Association of Dermatologists 2
Mucosal erythema, blistering, and erosions are reported by the American College of Obstetricians and Gynecologists 3, 4

The American Thoracic Society states that dyspnea and increased respiratory rate occur in approximately 25% of patients 3, 4
Bronchial hypersecretion occurs in 70% of patients with early pulmonary manifestations, according to the European Respiratory Society 3, 4
Cough and hemoptysis may be present, as noted by the American College of Chest Physicians 1

The American College of Dermatology recommends immediately discontinuing all potential culprit drugs and transferring patients to a specialized burn unit or ICU with multidisciplinary expertise 5, 6
The British Association of Dermatologists suggests calculating SCORTEN on admission to predict mortality risk and guide intensity of care 5, 6
Transfer patients with >10% body surface area involvement to a burn center or ICU without delay, according to the American Burn Association 5, 6

The Society of Critical Care Medicine recommends careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 5, 6
The American College of Surgeons notes that leaving detached epidermis in situ to act as a biological dressing is beneficial 5, 6
The Wound, Ostomy, and Continence Nurses Society suggests applying bland emollients frequently to support barrier function 5, 6

The American Academy of Ophthalmology recommends ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout the acute phase 5, 6
The American Dental Association suggests daily oral review during acute illness 6
The American Urological Association notes that catheterizing all patients can help prevent urethral strictures 3, 4

The American College of Rheumatology states that ciclosporin has shown benefit with reduced mortality compared to predicted rates in multiple studies 5, 6
The American Academy of Dermatology recommends systemic corticosteroids, particularly IV methylprednisolone pulse therapy, if started within 72 hours of onset 5, 6, 7

The American College of Dermatology notes that essential team members include dermatology, intensive care, burn surgery, ophthalmology, and specialist skincare nursing 5, 6
The American Academy of Pediatrics suggests additional consultations based on involvement, such as otolaryngology, urology/gynecology, wound care, and infectious disease 5, 7

The American College of Dermatology recommends providing written information about culprit drug(s) to avoid and potentially cross-reactive medications 5, 6
The American Academy of Dermatology suggests encouraging a MedicAlert bracelet bearing the name of the culprit drug 5, 6
The American Medical Association notes that documenting drug allergy in medical records and informing all healthcare providers is crucial 5, 6

The American Academy of Ophthalmology states that ocular sequelae include chronic dry eye, symblepharon, corneal scarring, and vision loss 8, 2
The American College of Dermatology notes that cutaneous sequelae include pigmentation changes and scarring 8, 2
The American Urological Association reports that urogenital sequelae include urethral strictures, phimosis, vaginal synechiae, and sexual dysfunction 3, 4