Stevens-Johnson Syndrome Clinical Presentation

Author: C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor: Hampton Roy Sr, MD more...

Updated: Sep 23, 2011

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History
Physical Examination
Complications
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History

Typically, Stevens-Johnson syndrome (SJS) begins with a nonspecific upper respiratory tract infection. This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present. Vomiting and diarrhea are occasionally noted as part of the prodrome.

Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions are typically nonpruritic.

A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.

Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink. Patients with genitourinary involvement may complain of dysuria or an inability to void.

A history of a previous outbreak of Stevens-Johnson syndrome or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.

Typical prodromal symptoms are as follows:

Cough productive of a thick purulent sputum
Headache
Malaise
Arthralgia

Patients may complain of a burning rash that begins symmetrically on the face and the upper part of the torso. This may be accompanied by ocular symptoms.

In addition to the skin, lesions in Stevens-Johnson syndrome may involve the following parts of the body:

Oral mucosa
Esophagus
Pharynx
Larynx
Anus
Trachea
Vagina
Urethra

Ocular symptoms include the following:

Red eye
Tearing
Dry eye
Pain
Blepharospasm
Itching
Grittiness
Heavy eyelid
Foreign body sensation
Decreased vision
Burn sensation
Photophobia
Diplopia

Delineation of a drug exposure timeline is essential, especially in the 1-3 weeks preceding the cutaneous eruption.

Physical Examination

The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema. The center of these lesions may be vesicular, purpuric, or necrotic.

The typical lesion has the appearance of a target; this is considered pathognomonic. However, in contrast to the typical lesions of erythema multiforme, these lesions have only two zones of color. The core may be vesicular, purpuric, or necrotic; that zone is surrounded by macular erythema. Some have called these targetoid lesions.

Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection. Extensive sloughing is shown in the image below.

Note extensive sloughing of epidermis from Stevens-Johnson syndrome. Courtesy of David F. Butler, MD.

Urticarial lesions typically are not pruritic. Infection may be responsible for the scarring associated with morbidity.

Although lesions may occur anywhere, the palms, soles, dorsum of the hands, and extensor surfaces are most commonly affected. Desquamation on the foot is shown in the image below.

Sheetlike desquamation on the foot in a patient with toxic epidermal necrolysis. Courtesy of Robert Schwartz, MD.

The rash may be confined to any one area of the body, most often the trunk.

Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis. An example of this type of involvement is shown in the image below.

Hemorrhagic crusting of the mucous membranes in toxic epidermal necrolysis. Similar lesions are seen in Stevens-Johnson syndrome. Courtesy of Robert Schwartz, MD.

For more information, see the Medscape Reference article Dermatologic Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Although some have suggested the possibility of Stevens-Johnson syndrome without skin lesions, most believe that mucosal lesions alone are not enough to establish the diagnosis. Cases without skin lesions have been termed "atypical" or "incomplete."^[11]These authors suggested that the combination of urethritis, conjunctivitis, and stomatitis established the diagnosis of Stevens-Johnson syndrome in a patient with Mycoplasma pneumonia– induced signs and symptoms.

The following signs may be noted on examination:

Fever
Orthostasis
Tachycardia
Hypotension
Altered level of consciousness
Epistaxis
Conjunctivitis
Corneal ulcerations
Erosive vulvovaginitis or balanitis
Seizures
Coma

The following signs may be noted on external examination:

Conjunctival hyperemia (ie, red eye)
Entropion
Skin lesions
Nasal lesions
Mouth lesions
Discharge (ie, catarrhal, mucous, membranous)

The following ocular signs may be noted on slit lamp examination (see the images below):

Eyelids: Trichiasis, distichiasis, meibomian gland dysfunction, blepharitis
Conjunctiva: Papillae, follicles, keratinization, subepithelial fibrosis, conjunctival shrinkage, foreshortening of fornices, symblepharon, ankyloblepharon
Cornea: Superficial punctate keratitis, epithelial defect, stromal ulcer, neovascularization, keratinization, limbitis, conjunctivalization, stromal opacity, perforation A patient with severe eye involvement associated with Stevens-Johnson syndrome. Note corneal neovascularization and conjunctivalization of the ocular surface. Epithelial defect of the cornea with neovascularization and surface conjunctivalization.

Complications

Of patients with Stevens-Johnson syndrome, 27-50% progress to severe ocular disease. Ocular complications of Stevens-Johnson syndrome include the following:

Chronic cicatrizing conjunctivitis
Corneal epithelial defects
Corneal stromal ulcers
Corneal perforation
Endophthalmitis

Other complications may include the following:

Gastroenterologic - Esophageal strictures
Genitourinary - Renal tubular necrosis, renal failure, penile scarring, vaginal stenosis
Pulmonary - Tracheobronchial shedding with resultant respiratory failure
Cutaneous - Scarring and cosmetic deformity, recurrences of infection through slow-healing ulcerations

Lesions may continue to erupt in crops for as long as 2-3 weeks. Mucosal pseudomembrane formation may lead to mucosal scarring and loss of function of the involved organ system. Esophageal strictures may occur when extensive involvement of the esophagus exists. Mucosal shedding in the tracheobronchial tree may lead to respiratory failure.

Blindness may develop secondary to severe keratitis or panophthalmitis in 3-10% of patients. Vaginal stenosis and penile scarring have been reported. Renal complications are rare.

Cutaneous lesions may resolve with a patchwork of hyperpigmentation and hypopigmentation. Fingernails and toenails may regrow abnormally. Lesions of the genitourinary system may lead to phimosis or vaginal synechiae.

Proceed to Differential Diagnoses

Contributor Information and Disclosures

Author

C Stephen Foster, MD, FACS, FACR, FAAO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Rola Ba-Abbad, MBBS Fellow in Vitreoretinal Division, Department of Ophthalmology, Lund University Hospital, Sweden

Rola Ba-Abbad, MBBS is a member of the following medical societies: American Academy of Ophthalmology and Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Erik Letko, MD Corneal Consultants of Colorado

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Daniel J Dire, MD, FACEP, FAAP, FAAEM Clinical Professor, Department of Emergency Medicine, University of Texas Medical School at Houston; Clinical Professor, Department of Pediatrics, School of Medicine, University of Texas Health Sciences Center San Antonio

Daniel J Dire, MD is a member of the following medical societies: American Academy of Clinical Toxicology, American Academy of Emergency Medicine, American Academy of Pediatrics, American College of Emergency Physicians, and Association of Military Surgeons of the US

Disclosure: Talecris Biotherapeutics Honoraria Speaking and teaching

Mark T Duffy, MD, PhD Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Kilbourn Gordon III, MD, FACEP Urgent Care Physician

Kilbourn Gordon III, MD, FACEP is a member of the following medical societies: American Academy of Ophthalmology and Wilderness Medical Society