Blistering Diseases — MCQs

Blistering Diseases Indian Medical PG Practice Questions and MCQs

Question 211: Acantholysis is seen in which of the following conditions?

A. Darier's disease
B. Staphylococcal scalded skin syndrome
C. Bullous pemphigoid
D. Pemphigus vulgaris (Correct Answer)

Explanation: ***Pemphigus vulgaris*** - **Pemphigus vulgaris** is the **prototypical example** of acantholysis, characterized by **autoantibodies** against **desmoglein 1 and 3**, components of **desmosomes**, leading to the loss of cell-cell adhesion or **acantholysis** within the epidermis. - This **acantholysis** results in the formation of **intraepidermal blisters** that are typically flaccid and rupture easily, leading to erosions. - Histologically shows **tombstone pattern** with complete loss of intercellular adhesion throughout the epidermis. *Darier's disease* - **Darier's disease** is an autosomal dominant disorder due to mutations in the **ATP2A2 gene**. - While it does show **focal acantholytic dyskeratosis** histologically, the primary feature is **dyskeratosis** (premature/abnormal keratinization) with characteristic **corps ronds** and **grains**. - The acantholysis is **focal and suprabasal**, associated with dyskeratotic cells, unlike the widespread acantholysis of pemphigus. *Staphylococcal scalded skin syndrome* - This condition is caused by **exfoliative toxins** (ETA, ETB) produced by *Staphylococcus aureus*, which target **desmoglein 1** in the **superficial epidermis**. - While it does cause **acantholysis in the granular layer**, this is **toxin-mediated** rather than autoimmune. - The split occurs in the **superficial granular layer only**, with intact deeper epidermis, unlike the mid-epidermal split in pemphigus vulgaris. *Bullous pemphigoid* - **Bullous pemphigoid** is an autoimmune blistering disease where antibodies target components of the **hemidesmosomes** in the **basement membrane zone**, specifically BP180 and BP230. - This leads to a **subepidermal split**, meaning the epidermis separates from the dermis as an intact layer. - There is **no acantholysis** - the keratinocytes remain adherent to each other; the separation occurs at the dermal-epidermal junction.

Question 212: Fogo selvagem is a type of:

A. Pemphigus vulgaris
B. Pemphigus foliaceus (Correct Answer)
C. Bullous pemphigoid
D. Pemphigus vegetans

Explanation: ***Pemphigus foliaceus*** - **Fogo selvagem** is the endemic form of pemphigus foliaceus found in Brazil, characterized by **superficial blistering**. - It involves **autoantibodies** against **desmoglein 1**, leading to separation within the superficial epidermis. *Pemphigus vulgaris* - This autoimmune blistering disease is characterized by autoantibodies against **desmoglein 1 and 3**, leading to **deep epidermal blisters**. - Patients typically present with **mucosal lesions** and flaccid blisters that rupture easily, which differs from the superficial cutaneous blistering of fogo selvagem. *Pemphigus vegetans* - This is a rare variant of **Pemphigus vulgaris**, characterized by verrucous plaques and hypertrophic vegetations, particularly in intertriginous areas. - While it's a form of pemphigus, it has distinct clinical features that differentiate it from the superficial blistering seen in fogo selvagem. *Bullous pemphigoid* - This condition involves **subepidermal blistering** due to autoantibodies targeting hemidesmosomes, specifically BP180 and BP230. - Clinically, it presents with tense blisters and is distinct from the intraepidermal blistering of pemphigus types.

Practice by Chapter

Pemphigus Vulgaris

Practice Questions

Pemphigus Foliaceus

Practice Questions

Bullous Pemphigoid

Practice Questions

Cicatricial Pemphigoid

Practice Questions

Dermatitis Herpetiformis

Practice Questions

Epidermolysis Bullosa

Practice Questions

Linear IgA Bullous Dermatosis

Practice Questions

Pemphigoid Gestationis

Practice Questions

Drug-Induced Bullous Disorders

Practice Questions

Immunofluorescence in Bullous Diseases

Practice Questions

Management of Autoimmune Bullous Diseases

Practice Questions

Genetic Counseling in Inherited Blistering Diseases

Practice Questions

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