Acute Generalized Exanthematous Pustulosis (AGEP) Overview

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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?

T-cell-mediated disorder 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)?

Cytotoxic CD8+ T cells Signup and view all the answers

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

ABCD-10 Signup and view all the answers

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

T-cell-mediated drug reaction Signup and view all the answers

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

Corticosteroids Signup and view all the answers

What is the approximate mortality rate associated with AGEP?

5% Signup and view all the answers

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

Blistering and purpura Signup and view all the answers

What histological feature is characteristic of AGEP?

Neutrophil and eosinophil infiltration Signup and view all the answers

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

20-25% Signup and view all the answers

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

Necroptosis pathway Signup and view all the answers

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

Mediating apoptosis without direct cell-to-cell contact Signup and view all the answers

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

Expression of Fas on the surface of keratinocytes Signup and view all the answers

What is a key feature that distinguishes apoptosis from necroptosis?

Inflammatory response Signup and view all the answers

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

Via the perforin/granzyme pathway Signup and view all the answers

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

Positive Nikolsky sign Signup and view all the answers

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 Signup and view all the answers

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

Multidisciplinary supportive care Signup and view all the answers

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

Systemic corticosteroid therapy Signup and view all the answers

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

Acute phase Signup and view all the answers

What skin manifestation is characteristic of DIHS/DRESS syndrome?

Morbilliform eruption Signup and view all the answers

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

They are all aromatic anticonvulsants Signup and view all the answers

What is the most common cause of Erythema Multiforme?

Mycoplasma pneumoniae infection 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?

IL-15, galectin-7, and RIP3 Signup and view all the answers

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

Presence of full thickness epidermal necrosis in SJS/TEN Signup and view all the answers

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

Oropharynx and conjunctivae Signup and view all the answers

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

Keratinocyte death 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?

Prompt withdrawal of non-essential agents Signup and view all the answers

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

Periorbital facial edema with erythema Signup and view all the answers

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

Liver Signup and view all the answers

What is a rare manifestation of DIHS/DRESS?

Interstitial pneumonitis Signup and view all the answers

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

Cytomegalovirus (CMV) Signup and view all the answers

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

Hepatitis Signup and view all the answers

What are frequently encountered blood abnormalities in DIHS/DRESS?

Prominent eosinophilia Signup and view all the answers

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

>4 weeks Signup and view all the answers

What is the mortality rate associated with DRESS syndrome?

~10% Signup and view all the answers

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

Prompt recognition and discontinuation of the offending drug Signup and view all the answers

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

Flow diagram based on a severity scoring system Signup and view all the answers