Discussion in 'Spot Diagnosis' started by Egyptian Doctor, Mar 9, 2013.
What is your medical diagnosis ?
Patognomic vision for Steven johnson syndrome.
Classic Erythema multiforme lesions on the hands - only by itself or with mild mucosal involvement, goes in favor of --> Erythema multiforme
My bet is on SJS
Steven Johnson syndrome is similar to erythema multiforme with the following differences. Etiology is similar, save for the fact that drugs are more commonly implicated. Skin lesions are similar but target lesions ae less common, bullae are commoner and lesions tend to be concentrated over mucosae and mucocutaneous junctions leading to severe mucosal erosions and hemorrhagic crusting. Constitutional disturbance is severe and systemic complications are commoner.
Now SJS has 2 siblings, the youngest and cutest of the three is - erythema multiforme. The eldest ( nasty and antisocial ) is - Toxic Epidermal Necrolysis.
Just like all siblings they have similarities and differences. let's look at what those are....
Intro - the 3 entities probably represent morphological and distributional variants of the same pathologic process. Both drugs and infections can induce either of the three.
Drugs - Sulfonamides, NSAIDS, tetracycline, penicillin, phenytoin, quinolones, rifampicin, INH etc.
Infections - Viral ( Herpes simplex ), bacterial ( Streptococcal ), mycobacterial and mycoplasmal
Closer look at Erythema Multiforme - as implied in the name, the lesions are multiform ( varied morphology ). Initial lesions are erythematous macules and patches that rapidly become elevated to form papules and plaques which develops a dark red center. Within a few days, the lesions progress to typical target lesions ( also called bull's eyelesion or iris lesion ) that comprise 3 concentric zones, from outside in, erythema ( bright red), edema( pale ) and the dark red center that represents either purpura or vesiculation. Erosions surrounded by erythema charecterise the mucosal affection.
Distribution - mucocut. jxn ( lips, glans penis ), mucosae ( oral, conjunctival), acrae (palms, soles,, dorsa of hands and feet ) and extremities are preferentially involved. Trunk is affected commonly, though the density of the lesions is lesser.
Toxic Epidermal necrolysis - although etiopathologically related to reythema multiforme, toxic epidermal necrolysis is a life threatening disease due to necrosis of whole body epidermis and mucosal epithelia. Epidermis peels off in large sheets on a background of diffuse tender erythema and bullae all over the body. mucosal lesions resemble Stevens Johnson Syndrome. Nikolsky sign is positive. Systemic complications are fluid, electrolyte and temp. imbalance and propensity for developing infections. THerapy is similar to that of 100% superficial burns.
stevens johnson syndrome
Thanks Neo_Star, I was waiting for someone to teach and explain me this case for 3 hours :-D I learn one more experience today Regard from Olomouc of Cz Rep.
I am happy my post helped you..it's worth the effort, only if it helps someone and without a feedback, i wouldn't have known that.
Best Regards from,
neo and Faculty Of Medicine (Y)
Answer : Steven Johnson Syndrome "Thanks Neo "
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