40 Questions
What is the main differentiating factor between Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?
Extent of epidermal detachment
Which factor contributes mainly to mortality in both Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?
Respiratory complications and sepsis
What is the SCORTEN used for in the context of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?
To predict mortality
What type of disorder is Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) considered to be?
T-cell-mediated disorder
Which type of cells infiltrate mainly in the blister fluid and epidermis in the early stages of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?
Cytotoxic CD8+ T cells
What newly proposed prognosis score has been introduced for Toxic Epidermal Necrolysis (TEN)?
ABCD-10
What is the mechanism of Acute Generalized Exanthematous Pustulosis (AGEP)?
T-cell-mediated drug reaction
Which of the following is NOT typically reported in association with AGEP?
Corticosteroids
What is the approximate mortality rate associated with AGEP?
5%
What clinical characteristic is NOT typical of Acute Generalized Exanthematous Pustulosis?
Blistering and purpura
What histological feature is characteristic of AGEP?
Neutrophil and eosinophil infiltration
What percentage of patients with AGEP experience mild and nonerosive mucosal involvement?
20-25%
Which pathway is NOT involved in inducing apoptosis of keratinocytes in SJS/TEN?
Necroptosis pathway
What is the main role of granulysin in the cytotoxic effect of SJS/TEN?
Mediating apoptosis without direct cell-to-cell contact
What distinguishes the skin lesions of TEN patients from those with maculopapular drug reactions?
Expression of Fas on the surface of keratinocytes
What is a key feature that distinguishes apoptosis from necroptosis?
Inflammatory response
How do cytotoxic CD8+ T cells induce apoptosis in SJS/TEN?
Via the perforin/granzyme pathway
What is the significant clinical diagnostic clue in both SJS and TEN?
Positive Nikolsky sign
What is the main reason to avoid the sulfonamide derivatives in the treatment of skin conditions?
They can be absorbed significantly and induce severe reactions
According to the UK guidelines for the management of SJS/TEN, what is prioritized over systemic treatment?
Multidisciplinary supportive care
What is recommended as a first-line treatment in the Japanese guidelines for SJS/TEN?
Systemic corticosteroid therapy
Which phase of DIHS/DRESS syndrome may immunosuppressive treatments be considered suitable?
Acute phase
What skin manifestation is characteristic of DIHS/DRESS syndrome?
Morbilliform eruption
What is a common feature of the drugs known to cause DIHS/DRESS syndrome?
They are all aromatic anticonvulsants
What is the most common cause of Erythema Multiforme?
Mycoplasma pneumoniae infection
Which biomarkers have been established but are not yet used in the clinical setting for predicting severe forms of SJS/TEN?
IL-15, galectin-7, and RIP3
What is a distinguishing feature that helps differentiate Erythema Multiforme from SJS/TEN?
Presence of full thickness epidermal necrosis in SJS/TEN
Which area is most commonly affected by mucosal involvement in SJS/TEN?
Oropharynx and conjunctivae
What is typically seen in Erythema Multiforme lesions but only slightly present in SJS/TEN lesions?
Keratinocyte death
What is recommended as part of the treatment for SJS and TEN to decrease mortality rate despite some mucocutaneous progression?
Prompt withdrawal of non-essential agents
What is a hallmark of Drug-induced hypersensitivity syndrome (DIHS)/Drug reaction with eosinophilia and systemic symptoms (DRESS)?
Periorbital facial edema with erythema
Which internal organ is the most frequently involved in Drug-induced hypersensitivity syndrome (DIHS)/DRESS?
Liver
What is a rare manifestation of DIHS/DRESS?
Interstitial pneumonitis
Which virus reactivation has been demonstrated to occur during DIHS/DRESS?
Cytomegalovirus (CMV)
What is a potential fatal complication of CMV reactivation in DIHS/DRESS?
Hepatitis
What are frequently encountered blood abnormalities in DIHS/DRESS?
Prominent eosinophilia
How soon after onset can reactivation of CMV occur in DIHS/DRESS?
>4 weeks
What is the mortality rate associated with DRESS syndrome?
~10%
What is crucial for the treatment of DIHS/DRESS according to the text?
Prompt recognition and discontinuation of the offending drug
What did a Japanese group propose in 2019 for the treatment of DIHS/DRESS?
Flow diagram based on a severity scoring system
Learn about the rare skin condition AGEP, its low incidence rate, potential mortality factors, T-cell-mediated mechanism, common culprit medications, and typical presentation timeline.
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