When healing hurts: Understanding a rare but devastating reaction to common medications
SJS affects only 1-2 people per million annually, making it exceptionally rare but extraordinarily dangerous 5 .
Stevens-Johnson Syndrome (SJS) and its more severe form, toxic epidermal necrolysis (TEN), represent a spectrum of severe skin reactions characterized by widespread epidermal detachment and mucosal erosion. These conditions are classified based on the percentage of body surface area affected: SJS involves less than 10% skin detachment, TEN involves more than 30%, and cases between 10-30% are classified as SJS/TEN overlap 3 .
SJS typically occurs within 4-28 days after starting the culprit medication, though reactions can sometimes appear sooner 5 .
Azithromycin is a widely prescribed antibiotic belonging to the macrolide class, frequently used to treat respiratory infections like pneumonia. While generally considered safe, azithromycin has been implicated in rare cases of SJS, though it's not among the most common causative agents 2 .
The mechanism behind this severe reaction appears to be a type IV hypersensitivity reaction, specifically a subtype involving T-cell mediated immune responses that lead to widespread keratinocyte apoptosis (cell death) 5 .
A 6-year-old Chinese boy with no significant medical history except premature birth presented with fever and cough that had persisted for seven days. After initial treatment with cefotiam at a local hospital provided only partial improvement, he was admitted to a major hospital for further care 6 .
The treatment team initiated intravenous azithromycin (0.25g daily) combined with cefmetazole sodium for suspected pneumonia. The boy continued to experience high fever on the second day of treatment 6 .
On the second hospital day, the child developed copious purulent material from his oral mucosa and conjunctiva, followed by the appearance of red maculopapules and blisters scattered over his entire body 6 .
Consultation with a dermatologist led to an initial diagnosis of erythema multiforme and possible adverse drug reaction to azithromycin, which was immediately discontinued. However, the child's condition continued to worsen dramatically 6 .
| Parameter | Value | Normal Range |
|---|---|---|
| White blood cell count | 13.84 × 10⁹/L | 4.5-11.0 × 10⁹/L |
| Neutrophils | 84.4% | 40-60% |
| C-reactive protein | 7.60 mg/L | 0-3 mg/L |
| AST (Liver Enzyme) | 143 U/L | 10-40 U/L |
| Amylase (Pancreatic Enzyme) | 1718 U/L | 25-125 U/L |
These findings confirmed both the severe inflammatory response characteristic of SJS and suggested a possible underlying Mycoplasma pneumoniae infection that might have contributed to the pathogenesis 1 6 .
| Mucosal Area | Interventions | Frequency |
|---|---|---|
| Ocular | Ofloxacin eye drops | Every 2 hours |
| Ofloxacin eye ointment | Every 4 hours | |
| Oral | Special mouthwash solution | 3 times daily |
| Stomatitis spray | Twice daily | |
| General | b-FGF spray to promote healing | Twice daily |
Perhaps most remarkably, the nursing team provided essential psychological support to a child enduring what many adults would find unbearable pain. The nurses noted the boy showed "extremely strong adherence and will power both physiologically and psychologically" throughout his ordeal 6 . This emotional support, combined with effective pain management, was crucial to his recovery.
Recent pharmacovigilance studies have strengthened our understanding of the relationship between antibiotics and SJS/TEN. A comprehensive analysis of the FDA Adverse Event Reporting System (FAERS) database identified 26 antibiotics associated with SJS/TEN reports 4 .
| Antibiotic Class | Examples | Reported Odds Ratio | Risk Level |
|---|---|---|---|
| Sulfonamides | Sulfamethoxazole | 20.27 | High |
| Penicillins | Amoxicillin, Ampicillin | 5.17 | Moderate |
| Cephalosporins | Cefaclor, Cefixime | 4.69 | Moderate |
| Macrolides | Azithromycin, Clarithromycin | 3.28 | Moderate |
| Quinolones | Levofloxacin, Moxifloxacin | 2.91 | Moderate |
The relationship between infection and SJS is complex and bidirectional. Some infections themselves can trigger SJS, while in other cases, antibiotics used to treat infections appear to be the culprit. A Japanese pharmacoepidemiological study using the JADER database found significant associations between infection status and SJS/TEN development, with odds ratios of 2.04 for patients taking anti-infective drugs and 2.44 for those with concomitant infections 8 .
Research has identified specific genetic markers that increase susceptibility to SJS/TEN reactions. The HLA-B*1502 allele has been strongly associated with carbamazepine-induced SJS in Han Chinese populations, while HLA-B*5801 is linked to allopurinol-induced SJS 3 .
The cold agglutination test used in diagnosing our case study patient represents a simple but effective bedside diagnostic technique. The procedure involves:
The case of this young boy illustrates both the devastating potential of adverse drug reactions and the remarkable power of dedicated, skilled nursing care. His recovery after 33 days of hospitalization stands as a testament to the importance of multidisciplinary collaboration, meticulous supportive care, and early recognition of this life-threatening condition.
As research continues to unravel the genetic and immunological mysteries behind these severe reactions, there is hope that someday we might predict and prevent SJS before it occurs. Until then, the compassionate, competent care provided by nurses and other healthcare professionals remains the most vital resource for those caught in the silent storm of Stevens-Johnson Syndrome.
Fever and cough symptoms
Hospital admission
Azithromycin initiated
First SJS symptoms appear
SJS diagnosis confirmed
Intensive treatment and recovery