Stevens-Johnson Syndrome (SJS) is a rare but serious medical condition that primarily affects the skin and mucous membranes. While SJS can occur at any age, many cases commonly occur in children and younger adults. Although rare, the impact of SJS on a child’s health can be life-threatening, making awareness and early intervention critical.
Causes and Triggers of SJS in Children
One of the primary causes of SJS in children is an adverse reaction to medications. Studies have identified several drugs commonly linked to SJS, including:
● Antibiotics (e.g., penicillins, sulfonamides, cephalosporins)
● Anticonvulsants (e.g., carbamazepine, lamotrigine, phenytoin, phenobarbital, valproic acid)
● Nonsteroidal anti-inflammatory drugs (NSAIDs) (e.g., ibuprofen, naproxen)
In addition to medications, SJS can also be caused by infections, particularly Mycoplasma pneumoniae, a bacterial infection that affects the lungs. Viral infections such as herpes simplex and influenza have also been associated with cases of SJS in children.
Symptoms and Diagnosis
SJS typically presents with vague symptoms, making early diagnosis challenging. It often starts with flu-like symptoms and some early warning signs include:
● Fever and body aches
● Painful blisters in the mouth, nose, eyes, and genitals
● Red or purplish skin rashes that spread quickly
● Peeling or sloughing of the skin
● Eye redness and irritation, which can lead to long-term vision problems
Since these symptoms can be mistaken for other illnesses, doctors may use a combination of clinical evaluation and skin biopsy to confirm the diagnosis.
Treatment Options for Children with SJS
There is no single cure for SJS, so treatment focuses on stopping the progression, managing symptoms, and preventing complications. The key steps include:
Discontinuing the Trigger – If a medication is identified as the cause, it should be stopped immediately under medical supervision.
Hospitalization – Most children with SJS require admission to specialized units, such as burn centers or intensive care units, for close monitoring and treatment.
Supportive Care – Management includes pain control, wound care, intravenous fluids, and nutritional support to promote healing and prevent complications.
Medication Therapy – Cyclosporine has shown greater effectiveness in reducing disease severity compared to intravenous immunoglobulin (IVIG) and corticosteroids, which are not generally recommended.
Specialist Care – Given the multisystem involvement of SJS, early consultation with ophthalmologists, urologists, and gynecologists is essential to prevent complications such as lacrimal duct scarring, urethral and vaginal strictures, and long-term functional impairments.
Complications and Long-Term Effects
Even after recovery, many children experience long-term complications from SJS, including:
● Chronic eye problems (dryness, scarring, vision loss)
● Skin sensitivity and scarring
● Psychological distress, such as anxiety or post-traumatic stress disorder (PTSD)
Preventing SJS in Children
Since SJS is mostly caused by drug reactions, careful medication use is crucial. If a child has had SJS in the past, avoiding the suspected medication and its drug class with a similar chemical structure is essential. Some genetic tests may help predict SJS risk in certain populations.
Raising Awareness for a Rare but Serious Condition
Although SJS is rare, its effects can be devastating. Awareness among parents, doctors, and caregivers can lead to earlier diagnosis and better outcomes. Understanding the signs, causes, and treatments is essential. Additionally, HLA genetic testing for drugs with strong associations can help identify at-risk individuals, enabling preventive strategies and reducing the incidence of SJS in children.
References
Del Pozzo-Magana BR, Lazo-Langner A, Carleton B, Castro-Pastrana LI, Rieder MJ. A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. J Popul Ther Clin Pharmacol. 2011 Mar 21;18(1):121-33.
Ferrandiz-Pulido C, Garcia-Patos V. A review of causes of Stevens–Johnson syndrome and toxic epidermal necrolysis in children. Archives of disease in childhood. 2013 Dec 1;98(12):998-1003.
Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years’ experience. Drug safety. 2002 Nov;25:965-72.
Lucia L, Silvia C, Paolo B, Roberto B, Fabio C, Francesca S, Francesca M, Giuseppe C, Fabrizio F, Carlo C. Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis. Acta Bio Medica: Atenei Parmensis. 2019;90(Suppl 3):52.
White KD, Abe R, Ardern-Jones M, Beachkofsky T, Bouchard C, Carleton B, et al., SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation. J Allergy Clin Immunol Pract. 2018 Jan-Feb;6(1):38-69.
Chang WC, Abe R, Anderson P, Anderson W, Ardern-Jones MR, Beachkofsky TM, Bellón T, et al., SJS/TEN 2019: From science to translation. J Dermatol Sci. 2020 Apr;98(1):2-12.
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