Skin Syndromes in Pediatrics

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Questions and Answers

What is the primary etiology associated with Erythema Multiforme?

Fungal infections

Autoimmune diseases

Herpes simplex virus (correct)

Bacterial infections

Which clinical feature distinguishes Toxic Epidermal Necrolysis (TEN) from Erythema Multiforme?

Less than 10% body surface area affected

Immune-mediated response

Mucosal involvement (correct)

Superficial skin involvement

What type of hypersensitivity reaction is primarily involved in Erythema Multiforme?

Type IV hypersensitivity (correct)

Type I hypersensitivity

Type II hypersensitivity

Type III hypersensitivity

In the context of Stevens-Johnson Syndrome (SJS), which of the following best describes skin involvement?

Epidermal necrosis involving less than 10% BSA (A) Signup and view all the answers

What is a common treatment approach for both Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis?

Aggressive supportive care (D) Signup and view all the answers

Which of the following can serve as a trigger for Erythema Multiforme?

Herpes simplex virus (A) Signup and view all the answers

What is a significant histopathological finding in Toxic Epidermal Necrolysis?

Full-thickness epidermal necrosis (A) Signup and view all the answers

Which of the following conditions is least likely to show a positive Nikolsky sign?

Erythema Multiforme (D) Signup and view all the answers

Which of the following statements correctly describes the pathophysiology of SSSS?

Exfoliative toxins target desmoglein-1, leading to cleavage within the epidermis (stratum granulosum). (B) Signup and view all the answers

Which of the following conditions typically involves widespread sloughing of the epidermis, covering more than 30% of the body surface area?

Toxic Epidermal Necrolysis (TEN) (C) Signup and view all the answers

Which of these conditions is primarily associated with a flu-like prodrome (fever, sore throat) as an initial symptom?

Both B and C (B) Signup and view all the answers

Which of the following is a common treatment approach shared by SSSS, SJS, and TEN?

Supportive care, including hydration and wound care. (C) Signup and view all the answers

What is the primary distinguishing feature between SJS and TEN?

The extent of epidermal detachment. (C) Signup and view all the answers

Which of the following is NOT a typical clinical feature of Staphylococcal Scalded Skin Syndrome (SSSS)?

Prominent mucosal involvement. (B) Signup and view all the answers

Which of these conditions is primarily caused by exotoxins produced by a bacteria?

SSSS (A) Signup and view all the answers

Which of the following statements accurately describes the histopathology of TEN?

Full-thickness epidermal necrosis. (B) Signup and view all the answers

What is a characteristic feature of target lesions seen in Erythema Multiforme?

Three distinct zones: central dusky, pale edematous, and erythematous halo (D) Signup and view all the answers

In terms of systemic symptoms, how does Erythema Multiforme differ from more severe conditions like SJS/TEN?

Systemic symptoms are often absent or mild in Erythema Multiforme (B) Signup and view all the answers

Which of the following statements about Erythema Multiforme Major is true?

It has mucosal involvement but is not life-threatening. (C) Signup and view all the answers

What type of lesions are commonly associated with Erythema Multiforme as opposed to SJS/TEN?

Target lesions (D) Signup and view all the answers

What is the typical mucosal involvement seen in Erythema Multiforme?

Mild oral ulcers and occasional ocular involvement (A) Signup and view all the answers

What is the primary mechanism for treatment in cases of Erythema Multiforme triggered by HSV?

Antiviral therapy to address the underlying cause (C) Signup and view all the answers

What distinguishes the triggering factors for Erythema Multiforme from those for SJS/TEN?

Infections like HSV are common triggers for Erythema Multiforme. (D) Signup and view all the answers

Which feature is characteristic of Erythema Multiforme in comparison to SJS/TEN?

Lesions that are self-limiting and resolve without intervention (B) Signup and view all the answers

Study Notes

Staphylococcal Scalded Skin Syndrome (SSSS)

Cause: Exotoxins (exfoliative toxins A and B) produced by Staphylococcus aureus.
Affected group: Primarily neonates and young children.
Pathophysiology: Toxins target desmoglein-1, causing epidermal cleavage in the stratum granulosum.
Symptoms: Fever, irritability, skin tenderness, widespread erythema, superficial flaccid blisters, large sheets of epidermal peeling, positive Nikolsky sign (epidermis detaches with pressure), no mucosal involvement.
Histology: Cleavage at the granular layer of the epidermis.
Treatment: Antistaphylococcal antibiotics (oxacillin, clindamycin), supportive care (hydration, wound care).

Stevens-Johnson Syndrome (SJS)

Cause: Severe immune-mediated hypersensitivity reaction, often triggered by drugs (e.g., sulfonamides, anticonvulsants) or infections (e.g., Mycoplasma pneumoniae).
Affected group: All ages.
Pathophysiology: Cytotoxic T cells induce keratinocyte apoptosis via Fas-Fas ligand interaction.
Symptoms: Flu-like prodrome (fever, sore throat), erythematous macules progressing to targetoid lesions and epidermal detachment (less than 10% body surface area), prominent mucosal involvement (oral, ocular, genital), positive Nikolsky sign.
Histology: Full-thickness epidermal necrosis.
Treatment: Stop offending agent, supportive care in ICU/burn unit, consider immunomodulation (IVIG, corticosteroids) in select cases.

Toxic Epidermal Necrolysis (TEN)

Cause: Similar to SJS (drug-induced hypersensitivity).
Affected group: All ages, more severe in adults.
Pathophysiology: Extensive keratinocyte apoptosis due to cytotoxic T-cell activity.
Symptoms: Similar to SJS but involves greater than 30% body surface area, severe skin and mucosal involvement, widespread sloughing of epidermis, positive Nikolsky sign, systemic complications (dehydration, infection, multi-organ failure).
Histology: Full-thickness epidermal necrosis with subepidermal detachment.
Treatment: Stop causative drug, aggressive supportive care (fluid replacement, wound care, temp control), IVIG or immunosuppressive therapy (controversial).

Erythema Multiforme (EM)

Cause: Immune-mediated hypersensitivity reaction, most commonly triggered by infections (especially Herpes simplex virus (HSV)), less frequently by drugs (e.g., sulfonamides, NSAIDs, anticonvulsants).
Pathophysiology: Type IV hypersensitivity reaction, immune response leading to immune complex deposition in small dermal blood vessels and inflammation.
Symptoms: Target lesions (central dusky/blistered area, pale edematous ring, erythematous halo), symmetrical involvement (palms, soles, extensor surfaces, mucocutaneous junctions), lesions can coalesce but usually discrete, mild mucosal involvement (oral ulcers, conjunctivitis, genital erosions) in EM Major, mild systemic symptoms (fever, malaise).
Classification: EM Minor (skin only, minimal mucosal involvement) and EM Major (mucosal involvement).
Differences from SJS/TEN: Less severe, usually self-limiting, target lesions, negative Nikolsky sign and less severe mucosal involvement, infections are frequent triggers, drugs less frequent. EM lesions usually remain discrete.
Treatment: Identify and treat underlying cause (e.g., antivirals for HSV), supportive care (hydration, analgesics, topical steroids). EM Major may require systemic corticosteroids or hospitalization, recurrent EM may need prophylactic antivirals if HSV is a trigger.

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Description

This quiz covers key details about Staphylococcal Scalded Skin Syndrome (SSSS) and Stevens-Johnson Syndrome (SJS), including their causes, affected demographics, pathophysiology, symptoms, and treatments. Test your knowledge on these serious skin conditions that primarily affect children and individuals of all ages.