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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
Print
Larynx
Anus
Trachea
Vagina
History
Urethra
Physical Examination
OcularComplications
symptoms include the following:
Author:All
C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor:
Show
Red
eye Roy Sr, MD more...
Hampton
Tearing
Multimedia
Library
Dry eye
References
Pain
Overview
Blepharospasm
Presentation
Itching
DDx
Grittiness
Workup
Heavy eyelid
Treatment
Foreign body sensation
Medication
Decreased vision
Burn sensation
Updated: Nov 27, 2012
Photophobia
Diplopia

Stevens-Johnson Syndrome Clinical

Presentation

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."[17] 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.

cornea with neovascularization and surface conjunctivalization.

Epithelial defect of the

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

Read more about Stevens-Johnson Syndrome on Medscape

Related Reference Topics
Dermatologic Manifestations of Stevens-Johnson
Syndrome and Toxic Epidermal Necrolysis
Drug-Induced Bullous Disorders
Child Sexual Abuse

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