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Stevens-Johnson Syndrome

Author: Tappi, Posted on Tuesday, July 27 @ 08:49:46 IST by RxPG  

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Clinical Manifestations

1. Dusky plaques with necrosis often involve the trunk, face, and extremities.
2. These lesions may be painful.
3. Erosions or bullae cover <10% of the body surface area (BSA).
4. Bullae and erosions may be seen in the mouth, on the lips, on the genital mucosa, and on the anal mucosa.
5. The GI and respiratory tract may be involved as well.
6. Patients may be febrile.
7. Importantly, conjunctival involvement may lead to blindness.
8. Fever

Stevens-Johnson Syndrome: Differential Diagnosis

The clinical presentation of Stevens-Johnson syndrome is often easily recognizable. However, diagnostic consideration is often given to:
Vesicobullous diseases
Herpetic infections
Diseases with oral ulcerations

Vesicobullous Diseases in the Differential Diagnosis of SJS

1. Pemphigus vulgaris often presents with facial and trunk flaccid bullae and erosions. Mucosal lesions are often seen as in this image.

2. Paraneoplastic pemphigus is another variant of pemphigus. Interestingly, erythema-multiforme like lesions may also be seen in this condition.

3. Bullous Pemphigoid presents with urticarial lesions, tense bullae, and erosions. Mucosal lesions are rare but may sometimes be seen.

4. Bullous lupus erythematosus is an unusual manifestation of systemic lupus erythematosus that should be given diagnostic consideration in the appropriate clinical setting.

Herpesvirus Infections in the Differential Diagnosis of SJS

1. Neonatal herpesvirus infection often presents with widespread vesicles that may initially appear at sites of trauma: at scalp electrode sites, for example.

2. Disseminated herpes zoster may be seen in immunocompromised patients as in this patient with Hodgkin's Disease with vesicles and erosions on the trunk and leg.

Diseases with Oral Ulcerations in the Differential Diagnosis of SJS

Many dermatologic conditions are associated with cutaneous and oral mucosal findings: among these, Behcets Disease and oral erosive lichen planus may be considered in the differential diagnosis of Stevens-Johnson syndrome.

1. Behcets Disease is associated with large oral, perianal and genital ulcerations, as well as with erythema nodosum (painful nodules on the legs).

2. Lichen planus rarely presents with oral erosive lesions in the absence of the typical skin lesions.

Stevens-Johnson Syndrome: Etiology

In adults, approximately 50% of cases of Stevens-Johnson syndrome are drug induced; others may be associated with infection or be idiopathic. In children, SJS is usually induced by infectious agents.
For unknown reasons, there is an increased risk of SJS in HIV-positive patients.
The onset of symptoms typically begins from 1-3 weeks following initiation of drug therapy.
The onset may be more rapid if the patient is rechallenged with the causative medication.

Stevens-Johnson Syndrome: Treatment

The first step in the treatment of Stevens-Johnson syndrome is the withdrawal of all medications that are not absolutely necessary, especially if begun within 1 month of the onset of the eruption. Do not rechallenge the patient with any suspect medications.

Skin biopsy may help to confirm the diagnosis and rule out other blistering diseases. Hospital admission may be appropriate, for hydration, nutritional support and monitoring for signs of infection. Ophthalmologic care is essential to minimize ocular damage. Use of corticosteroids is very controversial: if to be used, then very early in the course or not at all. If the patient is to be monitored in an outpatient setting, careful follow-up is necessary as this syndrome may progress to toxic epidermal necrolysis. The mortality of Stevens-Johnson syndrome is approximately 5%.

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