Acute Generalized Exanthematous Pustulosis (AGEP) Overview
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Questions and Answers

What is the main differentiating factor between Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?

  • Presence of pustules
  • Severity of skin discoloration
  • Extent of epidermal detachment (correct)
  • Involvement of hair follicles

Which factor contributes mainly to mortality in both Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?

  • Liver dysfunction
  • Respiratory complications and sepsis (correct)
  • Neurological damage
  • Renal failure

What is the SCORTEN used for in the context of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)?

  • To predict mortality (correct)
  • To diagnose the condition
  • To select appropriate antibiotics
  • To determine the cause

What type of disorder is Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) considered to be?

<p>T-cell-mediated disorder (A)</p> Signup and view all the answers

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)?

<p>Cytotoxic CD8+ T cells (C)</p> Signup and view all the answers

What newly proposed prognosis score has been introduced for Toxic Epidermal Necrolysis (TEN)?

<p>ABCD-10 (A)</p> Signup and view all the answers

What is the mechanism of Acute Generalized Exanthematous Pustulosis (AGEP)?

<p>T-cell-mediated drug reaction (A)</p> Signup and view all the answers

Which of the following is NOT typically reported in association with AGEP?

<p>Corticosteroids (C)</p> Signup and view all the answers

What is the approximate mortality rate associated with AGEP?

<p>5% (B)</p> Signup and view all the answers

What clinical characteristic is NOT typical of Acute Generalized Exanthematous Pustulosis?

<p>Blistering and purpura (C)</p> Signup and view all the answers

What histological feature is characteristic of AGEP?

<p>Neutrophil and eosinophil infiltration (C)</p> Signup and view all the answers

What percentage of patients with AGEP experience mild and nonerosive mucosal involvement?

<p>20-25% (B)</p> Signup and view all the answers

Which pathway is NOT involved in inducing apoptosis of keratinocytes in SJS/TEN?

<p>Necroptosis pathway (A)</p> Signup and view all the answers

What is the main role of granulysin in the cytotoxic effect of SJS/TEN?

<p>Mediating apoptosis without direct cell-to-cell contact (D)</p> Signup and view all the answers

What distinguishes the skin lesions of TEN patients from those with maculopapular drug reactions?

<p>Expression of Fas on the surface of keratinocytes (C)</p> Signup and view all the answers

What is a key feature that distinguishes apoptosis from necroptosis?

<p>Inflammatory response (A)</p> Signup and view all the answers

How do cytotoxic CD8+ T cells induce apoptosis in SJS/TEN?

<p>Via the perforin/granzyme pathway (D)</p> Signup and view all the answers

What is the significant clinical diagnostic clue in both SJS and TEN?

<p>Positive Nikolsky sign (C)</p> Signup and view all the answers

What is the main reason to avoid the sulfonamide derivatives in the treatment of skin conditions?

<p>They can be absorbed significantly and induce severe reactions (B)</p> Signup and view all the answers

According to the UK guidelines for the management of SJS/TEN, what is prioritized over systemic treatment?

<p>Multidisciplinary supportive care (C)</p> Signup and view all the answers

What is recommended as a first-line treatment in the Japanese guidelines for SJS/TEN?

<p>Systemic corticosteroid therapy (C)</p> Signup and view all the answers

Which phase of DIHS/DRESS syndrome may immunosuppressive treatments be considered suitable?

<p>Acute phase (B)</p> Signup and view all the answers

What skin manifestation is characteristic of DIHS/DRESS syndrome?

<p>Morbilliform eruption (B)</p> Signup and view all the answers

What is a common feature of the drugs known to cause DIHS/DRESS syndrome?

<p>They are all aromatic anticonvulsants (D)</p> Signup and view all the answers

What is the most common cause of Erythema Multiforme?

<p>Mycoplasma pneumoniae infection (C)</p> Signup and view all the answers

Which biomarkers have been established but are not yet used in the clinical setting for predicting severe forms of SJS/TEN?

<p>IL-15, galectin-7, and RIP3 (B)</p> Signup and view all the answers

What is a distinguishing feature that helps differentiate Erythema Multiforme from SJS/TEN?

<p>Presence of full thickness epidermal necrosis in SJS/TEN (C)</p> Signup and view all the answers

Which area is most commonly affected by mucosal involvement in SJS/TEN?

<p>Oropharynx and conjunctivae (D)</p> Signup and view all the answers

What is typically seen in Erythema Multiforme lesions but only slightly present in SJS/TEN lesions?

<p>Keratinocyte death (A)</p> Signup and view all the answers

What is recommended as part of the treatment for SJS and TEN to decrease mortality rate despite some mucocutaneous progression?

<p>Prompt withdrawal of non-essential agents (A)</p> Signup and view all the answers

What is a hallmark of Drug-induced hypersensitivity syndrome (DIHS)/Drug reaction with eosinophilia and systemic symptoms (DRESS)?

<p>Periorbital facial edema with erythema (D)</p> Signup and view all the answers

Which internal organ is the most frequently involved in Drug-induced hypersensitivity syndrome (DIHS)/DRESS?

<p>Liver (B)</p> Signup and view all the answers

What is a rare manifestation of DIHS/DRESS?

<p>Interstitial pneumonitis (D)</p> Signup and view all the answers

Which virus reactivation has been demonstrated to occur during DIHS/DRESS?

<p>Cytomegalovirus (CMV) (C)</p> Signup and view all the answers

What is a potential fatal complication of CMV reactivation in DIHS/DRESS?

<p>Hepatitis (D)</p> Signup and view all the answers

What are frequently encountered blood abnormalities in DIHS/DRESS?

<p>Prominent eosinophilia (D)</p> Signup and view all the answers

How soon after onset can reactivation of CMV occur in DIHS/DRESS?

<p>&gt;4 weeks (D)</p> Signup and view all the answers

What is the mortality rate associated with DRESS syndrome?

<p>~10% (B)</p> Signup and view all the answers

What is crucial for the treatment of DIHS/DRESS according to the text?

<p>Prompt recognition and discontinuation of the offending drug (D)</p> Signup and view all the answers

What did a Japanese group propose in 2019 for the treatment of DIHS/DRESS?

<p>Flow diagram based on a severity scoring system (B)</p> Signup and view all the answers

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