Classification and etiology
Stevens-Johnson syndrome is classified among prognostically unfavorable forms of drug eruptions. This same group of severe skin and mucosal disorders includes toxic epidermal necrolysis (Lyell's syndrome), which is distinguished by massive epidermal detachment and an extremely high mortality rate (up to 50%).
The syndrome is most commonly provoked by medications. The main triggers include:
- Antimicrobial drugs (with sulfonamides, particularly co-trimoxazole, leading the list).
- Beta-lactam antibiotics.
- Anticonvulsants.
- Antiretroviral drugs (NNRTIs — non-nucleoside reverse transcriptase inhibitors).
Additionally, the syndrome can develop as a severe immunoallergic manifestation associated with vanishing bile duct syndrome (VBDS), a rare form of drug-induced cholestatic liver injury.
Clinical presentation
The disease is characterized by an acute onset and a severe course.
Systemic Manifestations:
- High fever (body temperature rising up to 40 °C).
- Pronounced general intoxication.
- Intense headache.
- Myalgia (muscle pain) and arthralgia.
- Possible onset of seizures.
Dermatological and mucosal symptoms: A generalized dark red macular rash appears on the skin. The pathological process is accompanied by the formation of erosions and blisters. Mucosal involvement is extremely severe: the patient develops erosive stomatitis. Due to severe pain and tissue damage in the oral cavity, speech and eating become severely impaired.
Complications and long-term consequences
The disease frequently leads to severe complications affecting internal organs and the eyes.
- Respiratory system: the course of the syndrome is frequently complicated by the development of bronchopneumonia.
- Ophthalmological disorders: eye involvement is one of the leading symptoms. The sequelae of the disease include cicatricial shortening of the conjunctiva and deformation of the eyelid cartilage, which becomes the main cause of cicatricial entropion.
- Corneal involvement: cicatricial entropion is frequently combined with trichiasis (misdirected eyelash growth caused by scarring at their roots). A pathogenetic vicious circle is established: constant friction of the eyelashes and eyelid margins against the cornea induces spastic contraction of the pretarsal part of the orbicularis oculi muscle, further exacerbating the entropion. Chronic trauma leads to keratopathy, corneal erosions and dystrophy, and ulcerative keratitis. The ultimate outcome of this process is the formation of opacity — leukoma.
Principles of treatment
The golden rule in the development of a severe cutaneous reaction with pronounced symptoms is the immediate discontinuation of the drug that provoked the toxicoderma. This is the fundamental principle for managing patients with both Stevens-Johnson syndrome and Lyell's syndrome, allowing to halt the further progression of a life-threatening condition.
To remember the classic triad of lesions in Stevens-Johnson syndrome, use the «SMM» rule: Skin (dark red macular rash with blisters), Mucous membranes (erosive stomatitis that interferes with eating), Mucous membranes of the eyes (conjunctival involvement with risk of cicatricial entropion).
Class and exam questions
What is the main rule in the development of Stevens-Johnson syndrome?
Immediate discontinuation of the medication that provoked the severe reaction is required. This is the basic treatment principle for any life-threatening toxicoderma.
Which drug classes most frequently act as triggers?
The reaction is most frequently provoked by sulfonamides (co-trimoxazole), beta-lactam antibiotics, anticonvulsants, and antiretrovirals (NNRTIs).
How does the disease affect the patient's ability to eat?
Due to the development of severe erosive stomatitis and oral mucosa involvement, eating and even speaking become severely impaired.
What is the mechanism of ophthalmological complications following the syndrome?
The outcome of the disease leads to cicatricial shortening of the conjunctiva and deformation of the eyelid cartilage. This causes cicatricial entropion, which is exacerbated by trichiasis and leads to chronic corneal trauma.
What else to study on this topic
- Toxic epidermal necrolysis (Lyell's syndrome) and its differentiators
- DRESS syndrome: systemic manifestations and laboratory diagnostics
- Vanishing bile duct syndrome (VBDS)
- Pathogenesis and complications of cicatricial entropion
- Drug eruption: typical course and timing of manifestation
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