stevens johnson syndrome...without a rash

STEVENS-JOHNSON SYNDROME:
--severe idiosyncratic reactions, most commonly triggered by medications, which are characterized by fever and mucocutaneous lesions leading to necrosis and sloughing


--less severe disease on the same spectrum as TEN (toxic epidermal necrolysis)


DIAGNOSIS: NOT FAIR
--apparently, you can have Stevens-Johnson syndrome without a rash


PMID:  22041607
The Stevens-Johnson syndrome (SJS) classically involves a rash, conjunctivitis and mucositis.

case report of isolated mucositis and conjunctivitis.

Previous rare reports of severe SJS like syndromes without a rash are confined to children, usually with mycoplasma pnemoniae infection


PMID:  22012144
The commonest infections associated with SJS have been HSV and Mycoplasma pneumoniae. 

Less than 10% cases of Mycoplasma pneumonia develop extra pulmonary complications like hemolytic anemia, hepatitis, arthritis, meningitis and SJS.  

unclear from existing literature whether antibiotic treatment of M. pneumoniae infection decreases the risk for SJS. 


PMID:  20678095
another case report of mucocutaneous involvement without skin lesions. 

Oral lesions are present in all cases with SJS associated with M. pneumoniae infection, ocular lesions only in two-thirds and genital lesions in three-fourths of all cases. 

That SJS in association with M. pneumoniae infection is predominantly mucosal is reflected by few reports of SJS presenting without skin lesions. Even when skin lesions are present, mucocutaneous lesions predominate.


BOTTOM LINE:
--stevens johnson syndrome: skin and mucosal involvement, can progress to sloughing (e.g. TEN)
--can present withOUT rash. Mycoplasma pneumonia is common source.


Submitted by J. Gullo.




Reference(s): uptodate.com: Stevens-Johnson syndrome and toxic epidermal necrolysis: Clinical manifestations; pathogenesis; and diagnosis; article 1, article 2, article 3; picture