Stevens-Johnson Syndrome (SJS)
So this condition hits home for me, because during my NESH Homeopathy training course, Dr. Paul Herscu saw a patient who had SJS, and I remember her distinctly. The patient complained of entropion (her eyelids and eyelashes turning inward) and her difficultly seeing, as her cornea and sclera were scarred and dry. She complained of blurred vision and a particular distinctive cough, as well as massive phlegm buildup whenever she came down with a cold. It was a very interesting case, and we ended up giving her a homeopathic, but here is some more information about SJS in general:
- Also classified as Erythema Multiforme Major (EMM) in 1950, but later this designation was limited to only target lesions
- Other scholars think that SJS and Toxic Epidermal Necrolysis (TEN) are the same disease of varying severities along the same continuum (identical clinical s/sxs, identical tx, and identical prognosis)
Etiology
- Rare condition: 0.1/0.7 per 100,000 individuals (or 2.6-7.1 cases per 1 million people)
- Male > Female 2:1; More common in Chinese populations
- Mortality rate: 1-3%
- Often a drug hypersensitivity reaction (anticonvulsants – pheytoin, Phenobarbital, carbamezepine, valproic acid; sulfonimides; penicillins, NSAIDs; anti-rheumatic drugs)
- Vaccination
- Infection
- Systemic diseases
- Physical agents
Contributory factors
o HIV/AIDS or other immunocompromised individual (chemotherapy, elderly)
o Autoimmunity (SLE)
o Slow acetylators (incapable of achieving complete detoxificaiton of reactive drug metabolites)
o Brain tumors undergoing radiotherapy with concomitant antiepileptiscs
- Diagnostic criteria
2 or more mucosal surfaces that are dry
• Eyes
• Oral cavity
• Upper airway
• Esophagus
• GI tract
• Anogenital mucosa
Pathology
- SJS has been defined as mucosal erosions and epidermal detachment affecting less than 10% of the body surface area
- Antigen presentation and TNFα production of epithelial dendritic cells recruit T cell proliferation of Th1, Th2, and CTL cells
o The problem is with CD8+ CTLs, which induce epidermal cell apoptosis
• Release granzyme B
• Release perforin
- Apoptosis of keratinocytes can trigger caspase system to lead to DNA disorganization and cellular death
DDx:
- Erythema multiforme
- Pemphigus
- Pemphigoid
- Serum sickness
- Scalded skin syndrome (Staphylococcus infection)
- Bechet’s syndrome
- Kawasaki’s Disease
- Toxic schock syndrome
- Toxic epidermal necrolysis
- Chemical burns
- Allergic, Bacterial, Viral Conjunctivitis
- Atopic dermatitis
- Distichiasis (abnormal growth of eyelashes from the orifices of meiboian glands)
- Entropion (eyelashes curling inwards)
- Atopic or Epidemic Keratoconjunctivitis
- Ocular Rosacea
- Sarcoidosis
- Sjogren’s syndrome
- Conjunctival squamous cell carcinoma
- Trachoma (Chlamydia trachomatis)
- Trichiasis
Treatment (Usually in ICU or burn centers)
- Corticosteroids, but its controversial
- Intravenous immunoglobulin, hemodialysis, plasmapheresis may be applicable (not standard of treatment)
- Fluid replacement + parenteral nutrition
- Prevention of secondary complications or infections
References:
http://emedicine.medscape.com/article/1197450-overview
http://www.mdconsult.com/das/pdxmd/body/276406972-3/0?type=med&eid=9-u1.0-_1_mt_1010382
Stevens-Johnson Syndrome (SJS)
So this condition hits home for me, because during my NESH Homeopathy training course, Dr. Paul Herscu saw a patient who had SJS, and I remember her distinctly. The patient complained of entropion (her eyelids and eyelashes turning inward) and her difficultly seeing, as her cornea and sclera were scarred and dry. She complained of blurred vision and a particular distinctive cough, as well as massive phlegm buildup whenever she came down with a cold. It was a very interesting case, and we ended up giving her a homeopathic, but here is some more information about SJS in general:
- Also classified as Erythema Multiforme Major (EMM) in 1950, but later this designation was limited to only target lesions
- Other scholars think that SJS and Toxic Epidermal Necrolysis (TEN) are the same disease of varying severities along the same continuum (identical clinical s/sxs, identical tx, and identical prognosis)
Etiology
- Rare condition: 0.1/0.7 per 100,000 individuals (or 2.6-7.1 cases per 1 million people)
- Male > Female 2:1; More common in Chinese populations
- Mortality rate: 1-3%
- Often a drug hypersensitivity reaction (anticonvulsants – pheytoin, Phenobarbital, carbamezepine, valproic acid; sulfonimides; penicillins, NSAIDs; anti-rheumatic drugs)
- Vaccination
- Infection
- Systemic diseases
- Physical agents
Contributory factors o HIV/AIDS or other immunocompromised individual (chemotherapy, elderly)
o Autoimmunity (SLE)
o Slow acetylators (incapable of achieving complete detoxificaiton of reactive drug metabolites)
o Brain tumors undergoing radiotherapy with concomitant antiepileptiscs
- Diagnostic criteria 2 or more mucosal surfaces that are dry
• Eyes
• Oral cavity
• Upper airway
• Esophagus
• GI tract
• Anogenital mucosa
Pathology
- SJS has been defined as mucosal erosions and epidermal detachment affecting less than 10% of the body surface area
- Antigen presentation and TNFα production of epithelial dendritic cells recruit T cell proliferation of Th1, Th2, and CTL cells
o The problem is with CD8+ CTLs, which induce epidermal cell apoptosis
• Release granzyme B
• Release perforin
- Apoptosis of keratinocytes can trigger caspase system to lead to DNA disorganization and cellular death
DDx:
- Erythema multiforme
- Pemphigus
- Pemphigoid
- Serum sickness
- Scalded skin syndrome (Staphylococcus infection)
- Bechet’s syndrome
- Kawasaki’s Disease
- Toxic schock syndrome
- Toxic epidermal necrolysis
- Chemical burns
- Allergic, Bacterial, Viral Conjunctivitis
- Atopic dermatitis
- Distichiasis (abnormal growth of eyelashes from the orifices of meiboian glands)
- Entropion (eyelashes curling inwards)
- Atopic or Epidemic Keratoconjunctivitis
- Ocular Rosacea
- Sarcoidosis
- Sjogren’s syndrome
- Conjunctival squamous cell carcinoma
- Trachoma (Chlamydia trachomatis)
- Trichiasis
Treatment (Usually in ICU or burn centers)
- Corticosteroids, but its controversial
- Intravenous immunoglobulin, hemodialysis, plasmapheresis may be applicable (not standard of treatment)
- Fluid replacement + parenteral nutrition
- Prevention of secondary complications or infections
References:
http://emedicine.medscape.com/article/1197450-overview
http://www.mdconsult.com/das/pdxmd/body/276406972-3/0?type=med&eid=9-u1.0-_1_mt_1010382