Bullous Pemphigoid — MCQs
Which of the following is NOT a characteristic of pemphigus vulgaris?
A 40 year old male reported with recurrent episodes of oral ulcers, large areas of denuded skin and flaccid vesiculo-bullous eruptions. Which is the most important bedside investigation helpful in establishing the diagnosis -
Treatment of choice for Pustular psoriasis is:
Nikolsky's sign is seen in all of the following, except:
Which of the following statements about mucous membrane pemphigoid is correct?
Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
A patient presents with skin lesions and erosions on the buccal mucosa. The immunofluorescence image is shown. What is the most likely diagnosis?
A skin biopsy shows acantholysis with intraepidermal blistering. Which immunofluorescence pattern would confirm pemphigus vulgaris?
A 85-year-old female developed multiple blisters on the trunk and thighs. Nikolsky's sign is negative. The lesions came on and off. The most probable diagnosis is
Acantholysis is seen in all except which of the following conditions?
Bullous Pemphigoid Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following is NOT a characteristic of pemphigus vulgaris?
- A. Oral erosions
- B. Tzanck smear showing acantholytic cells
- C. Positive Nikolsky’s sign
- D. Subepidermal bulla (Correct Answer)
Explanation: ***Subepidermal bulla*** - Pemphigus vulgaris is characterized by **intraepidermal bullae** resulting from acantholysis (loss of cohesion between keratinocytes), not subepidermal bullae. - **Subepidermal bullae** are characteristic of conditions like **bullous pemphigoid**, where the split occurs below the epidermis. *Positive Nikolsky’s sign* - The **Nikolsky's sign** is positive in pemphigus vulgaris, indicating the fragility of the skin where gentle lateral pressure causes epidermal shearing. - This sign is a direct result of the **intraepidermal blistering** due to weakened cell-to-cell adhesion. *Oral erosions* - **Oral erosions** are a very common and often the initial manifestation of pemphigus vulgaris, frequently preceding skin lesions. - These painful erosions are persistent and heal slowly, sometimes making eating difficult. *Tzanck smear showing acantholytic cells* - A **Tzanck smear** from a fresh blister in pemphigus vulgaris typically reveals **acantholytic cells**, which are detached, rounded keratinocytes with basophilic cytoplasm. - The presence of acantholytic cells confirms the **loss of intercellular adhesion** within the epidermis, a hallmark of pemphigus.
Question 2: A 40 year old male reported with recurrent episodes of oral ulcers, large areas of denuded skin and flaccid vesiculo-bullous eruptions. Which is the most important bedside investigation helpful in establishing the diagnosis -
- A. Tzanck smear from the floor of bulla (Correct Answer)
- B. Gram staining of blister fluid
- C. Culture and sensitivity of blister fluid
- D. Skin biopsy with immunofluorescence
Explanation: ***Tzanck smear from the floor of bulla*** - A Tzanck smear from the floor of a bulla will reveal **acantholytic cells** (rounded keratinocytes that have lost their intercellular connections), which are characteristic of pemphigus, consistent with recurrent oral ulcers, denuded skin, and flaccid vesiculobullous eruptions. - This **bedside test** provides a rapid diagnosis by demonstrating the cytological features of acantholysis, differentiating it from other blistering disorders. *Gram staining of blister fluid* - This test is primarily used to identify **bacterial infections** and would show the morphology and Gram-staining characteristics of any bacteria present. - It would not provide information about the **acantholysis** or autoimmune nature of the blistering condition described. *Culture and sensitivity of blister fluid* - This investigation identifies **specific bacterial pathogens** and their antibiotic susceptibilities, which is useful for treating bacterial infections. - It would not help in diagnosing **autoimmune blistering diseases** like pemphigus, where bacteria are not the primary cause of the lesions. *Skin biopsy with immunofluorescence* - While a **skin biopsy with direct immunofluorescence** is the gold standard for confirming pemphigus by detecting autoantibodies, it is an **invasive procedure** requiring laboratory processing and is not considered a rapid bedside investigation. - The question specifically asks for the "most important **bed-side investigation**" helpful in establishing the diagnosis rapidly.
Question 3: Treatment of choice for Pustular psoriasis is:
- A. Methotrexate (Correct Answer)
- B. Psoralen - UV therapy
- C. Systemic steroid
- D. Estrogen
Explanation: ***Methotrexate*** - **Methotrexate** is a systemic immunosuppressant often considered the first-line treatment for severe forms of **pustular psoriasis** due to its efficacy in reducing inflammation and hyperproliferation of skin cells. - It works by inhibiting **dihydrofolate reductase**, thereby interfering with DNA synthesis and cell division, which is crucial in rapidly dividing cells like those found in psoriasis. *Psoralen - UV therapy* - **Psoralen and ultraviolet A (PUVA)** therapy can be used for chronic plaque psoriasis, but it is generally **contraindicated or used with extreme caution** in pustular psoriasis due to the risk of exacerbating the disease or causing irritation. - **UV light therapy** can sometimes trigger or worsen pustular flares, especially in acute generalized pustular psoriasis. *Systemic steroid* - While systemic steroids can provide temporary relief by addressing inflammation, their use in pustular psoriasis is generally **not recommended for long-term management** due to the high risk of severe rebound flares upon withdrawal. - Withdrawal of **systemic corticosteroids** can precipitate or worsen generalized pustular psoriasis, making them a less desirable long-term treatment option. *Estrogen* - **Estrogen** has no direct role in the treatment of psoriasis. Psoriasis is an inflammatory skin condition, and its pathophysiology is not directly influenced by estrogen levels. - Hormonal therapies are not indicated for the management of psoriasis, including its pustular forms.
Question 4: Nikolsky's sign is seen in all of the following, except:
- A. Bullous pemphigoid (Correct Answer)
- B. Toxic epidermal necrolysis
- C. Scalded skin syndrome
- D. Mucous membrane pemphigoid
Explanation: ***Bullous pemphigoid*** - **Nikolsky's sign** is typically **negative** in bullous pemphigoid because the blistering occurs in the **subepidermal region**, leading to a strong dermo-epidermal adhesion that resists tangential pressure. - The blisters in bullous pemphigoid are generally **tense** and do not rupture easily, reflecting the deep separation plane. *Mucous membrane pemphigoid* - **Nikolsky's sign** is typically **negative** in mucous membrane pemphigoid (also known as cicatricial pemphigoid) because it is also a **subepidermal blistering disorder**. - Like bullous pemphigoid, the cleavage occurs below the epidermis, preserving the integrity of the epidermal layer and maintaining resistance to lateral shearing forces. - The blisters are typically tense rather than flaccid, reflecting the deeper plane of separation. *Toxic epidermal necrolysis* - **Nikolsky's sign** is **positive** in toxic epidermal necrolysis (TEN) due to the extensive **full-thickness epidermal necrosis** and detachment, which is the hallmark of the condition. - Gentle tangential pressure causes the epidermis to easily shear off, revealing large areas of denuded dermis. *Scalded skin syndrome* - **Nikolsky's sign** is **positive** in scalded skin syndrome (SSSS) because the **exfoliative toxins** produced by *Staphylococcus aureus* cleave **desmoglein 1** in the superficial epidermis. - This cleavage leads to rapid and widespread **intraepidermal detachment** and flaccid blistering, making the skin highly susceptible to shearing.
Question 5: Which of the following statements about mucous membrane pemphigoid is correct?
- A. It presents as multiple, painful ulcers preceded by bullae which form below the epithelium at the basement membrane.
- B. Oral lesions may be found in any region, especially in the attached gingiva; ocular lesions can lead to blindness if untreated. (Correct Answer)
- C. None of the options.
- D. It primarily affects young adults and children, with peak incidence in the 2nd to 3rd decade of life.
Explanation: ***Oral lesions may be found in any region, especially in the attached gingiva; ocular lesions can lead to blindness if untreated.*** - **Mucous membrane pemphigoid (MMP)** frequently manifests in the **oral cavity**, with the attached gingiva being the most common site, often presenting as **desquamative gingivitis**. - **Ocular involvement** occurs in 60-70% of cases and is a critical feature that can cause conjunctival scarring, symblepharon formation, ankyloblepharon, and eventually **blindness** if not recognized and managed early. - This statement captures the two most clinically significant features of MMP: the characteristic oral presentation and the sight-threatening ocular complications. *It presents as multiple, painful ulcers preceded by bullae which form below the epithelium at the basement membrane.* - While MMP does involve **subepithelial blister formation** at the basement membrane zone (confirmed by immunofluorescence showing linear IgG and C3 deposition), the clinical presentation is typically **chronic erosions and desquamation** rather than acute multiple painful ulcers. - The bullae in MMP are often **tense and intact initially** but rupture easily, leaving **slow-healing erosions** rather than the acute ulcerative picture this option suggests. - This description might be more characteristic of **pemphigus vulgaris** (which has flaccid, painful oral ulcers from intraepithelial bullae). *It primarily affects young adults and children, with peak incidence in the 2nd to 3rd decade of life.* - This is **incorrect**. MMP predominantly affects **middle-aged to elderly adults**, with peak incidence in the **6th to 7th decade of life** (ages 50-70 years). - The disease is rare in children and young adults, making this statement factually inaccurate.
Question 6: Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
- A. Statements 1 & 2 are correct, 2 is not explaining 1 (Correct Answer)
- B. Statements 1 and 2 are correct and 2 is the correct explanation for 1
- C. Statements 1 and 2 are incorrect
- D. Statement 1 is incorrect
Explanation: ***Correct: Statements 1 & 2 are correct, 2 is not explaining 1*** **Analysis of Statement 1:** - A 59-year-old patient with **flaccid bullae** and **suprabasal acantholytic split** on histopathology is the classic presentation of **Pemphigus vulgaris** - The flaccid (easily ruptured) nature of bullae distinguishes it from tense bullae seen in bullous pemphigoid - The suprabasal location of the split (just above the basal layer) with acantholysis (loss of cell-to-cell adhesion) is pathognomonic - **Statement 1 is CORRECT** ✓ **Analysis of Statement 2:** - The **"row of tombstones" or "tombstone appearance"** is indeed a diagnostic histopathological feature of Pemphigus vulgaris - This appearance results from basal keratinocytes remaining attached to the basement membrane while suprabasal cells separate due to acantholysis - The intact basal cells standing upright resemble a row of tombstones - **Statement 2 is CORRECT** ✓ **Does Statement 2 explain Statement 1?** - Statement 2 describes a **histopathological appearance** (tombstone pattern) that is a **consequence** of the suprabasal split - However, it does NOT explain the **underlying cause** of the flaccid bullae or the suprabasal split - The true explanation involves **IgG autoantibodies against desmoglein 3 (and desmoglein 1)**, which attack intercellular adhesion structures (desmosomes), causing **acantholysis** - Therefore, **Statement 2 does NOT explain Statement 1** ✗ *Incorrect: Statement 2 is the correct explanation for Statement 1* - While both statements describe features of Pemphigus vulgaris, the tombstone appearance is a descriptive finding, not an explanatory mechanism *Incorrect: Statements 1 and 2 are incorrect* - Both statements are medically accurate descriptions of Pemphigus vulgaris features *Incorrect: Statement 1 is incorrect* - Statement 1 correctly describes the cardinal clinical and histopathological features of Pemphigus vulgaris
Question 7: A patient presents with skin lesions and erosions on the buccal mucosa. The immunofluorescence image is shown. What is the most likely diagnosis?
- A. Bullous pemphigoid
- B. Pemphigus vulgaris (Correct Answer)
- C. Linear IgA disease
- D. Dermatitis herpetiformis
Explanation: ***Pemphigus vulgaris*** - The combination of **flaccid blisters/erosions** on the skin and **buccal mucosal lesions** is characteristic of pemphigus vulgaris. The image showing **intercellular IgG deposits** (a "chicken wire" pattern) in the epidermis confirms the diagnosis on immunofluorescence. - Pemphigus vulgaris is an **autoimmune blistering disease** caused by autoantibodies against **desmoglein 1 and 3**, leading to acantholysis (loss of cell adhesion) within the epidermis. *Bullous pemphigoid* - This condition typically presents with **tense bullae** that are less prone to rupture, and **mucosal involvement is rare**. - Immunofluorescence in bullous pemphigoid shows **linear IgG and C3 deposits at the dermoepidermal junction**, not an intercellular epidermal pattern. *Linear IgA disease* - Characterized by **linear IgA deposition along the basement membrane zone** on direct immunofluorescence. - Clinically, it presents with **blisters** that can be variable in appearance, but the pathognomonic immunofluorescence pattern is distinct. *Dermatitis herpetiformis* - Presents with very **pruritic vesicles and papules**, primarily on extensor surfaces, and is strongly associated with **celiac disease**. - Direct immunofluorescence reveals **granular IgA deposits in the dermal papillae**, which is distinct from the intercellular IgG pattern seen here.
Question 8: A skin biopsy shows acantholysis with intraepidermal blistering. Which immunofluorescence pattern would confirm pemphigus vulgaris?
- A. Fishnet pattern of IgG (Correct Answer)
- B. Linear IgA deposits
- C. Granular IgG deposits
- D. Linear C3 deposits
Explanation: ***Fishnet pattern of IgG*** - A **fishnet or reticular pattern** of **IgG deposition** on direct immunofluorescence (DIF) is characteristic of **pemphigus vulgaris**, indicating antibodies targeting **desmoglein 1 and 3** in the intracellular spaces of the epidermis. - This pattern corresponds to the **acantholysis** observed on biopsy, where loss of cell adhesion leads to intraepidermal blistering. *Linear IgA deposits* - **Linear IgA deposits** at the **dermal-epidermal junction** are characteristic of **linear IgA bullous dermatosis**, a blistering disorder distinct from pemphigus. - This pattern signifies **antibodies targeting components of the basement membrane zone**, not intraepidermal desmogleins. *Granular IgG deposits* - **Granular IgG deposits** in the skin are typically seen in conditions like **lupus erythematosus** or **dermatitis herpetiformis** when IgA is targeted, signifying immune complex deposition or specific antigen targeting. - This pattern is not associated with the pathogenesis of pemphigus vulgaris, which involves antibodies against desmosomal proteins. *Linear C3 deposits* - **Linear C3 deposits**, particularly at the **dermal-epidermal junction**, are a hallmark of **bullous pemphigoid**, often accompanied by linear IgG or IgA. - This indicates **complement activation** at the basement membrane zone, leading to subepidermal blistering, not the intraepidermal blistering seen in pemphigus vulgaris.
Question 9: A 85-year-old female developed multiple blisters on the trunk and thighs. Nikolsky's sign is negative. The lesions came on and off. The most probable diagnosis is
- A. Pemphigus vulgaris
- B. Bullous pemphigoid (Correct Answer)
- C. Lepra reaction
- D. Lichen planus
Explanation: ***Bullous pemphigoid*** - The presence of **multiple tense blisters** on the trunk and thighs in an 85-year-old female, coupled with a **negative Nikolsky's sign**, is highly characteristic of bullous pemphigoid. - This condition tends to wax and wane, causing the lesions to "come on and off," and is more common in the **elderly**. *Lichen planus* - This condition presents with **pruritic, polygonal, purple, planar papules and plaques**, not blisters. - It does not typically involve the formation of **blisters** as the primary lesion nor does it involve a negative Nikolsky's sign. *Pemphigus vulgaris* - Characterized by **flaccid blisters** that rupture easily, leading to erosions, and a **positive Nikolsky's sign**. - This is in contrast to the **tense blisters** and **negative Nikolsky's sign** described in the patient. *Lepra reaction* - Refers to **acute inflammatory episodes** occurring in patients with leprosy, often presenting as **erythematous nodules** or plaques. - It does not typically involve the formation of **blisters** on the trunk and thighs in an elderly patient without a prior diagnosis of leprosy.
Question 10: Acantholysis is seen in all except which of the following conditions?
- A. Pemphigus vulgaris
- B. Darier's disease
- C. Bullous pemphigoid (Correct Answer)
- D. SSSS
Explanation: ***Bullous pemphigoid*** - This condition involves **subepidermal blistering**, meaning the separation of the epidermis from the dermis, which occurs *below* the **basal cell layer**. - **Acantholysis**, the loss of cohesion between keratinocytes *within* the epidermis, does not occur in bullous pemphigoid, making it the correct answer. *Pemphigus vulgaris* - This is an **autoimmune blistering disease** characterized by the presence of autoantibodies against **desmoglein 3** (and often desmoglein 1). - This leads to intraepidermal blistering caused by **acantholysis**, the primary pathophysiological event. *Darier's disease* - This is an **autosomal dominant genodermatosis** characterized by abnormal keratinization and acantholysis. - Due to defects in **ATP2A2** (encoding SERCA2), there is impaired calcium handling in keratinocytes, leading to premature desmosomal degradation and **acantholysis**. *SSSS (Staphylococcal Scalded Skin Syndrome)* - Caused by **exfoliative toxins** (ETA and ETB) produced by *Staphylococcus aureus* that target **desmoglein 1**. - The cleavage of desmoglein 1 results in superficial **intraepidermal blistering** due to **acantholysis** in the granular layer of the epidermis.
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