Pemphigus Foliaceus — MCQs
A 70-year-old man comes to the emergency department because of a skin rash and severe itching. He appears ill; there is a generalized skin rash that is scaly, erythematous, and thickened. His palms, soles, and scalp are also involved. Which of the following is the most likely diagnosis?
Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
Which of the following drugs can lead to pemphigus?
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 the skin lesions shown in the image. While evaluating for possible blistering disorders, all of the following conditions could present with similar morphology EXCEPT:
A child presents with grouped vesicles on an erythematous base on the buttocks. What is the most likely diagnosis?
Which of the following is NOT associated with erythema nodosum?
A 50-year-old female presents with blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. What is the most likely diagnosis?
Which histological finding is most specific for pemphigus vulgaris?
A 26-year-old girl at 31 weeks' gestation complains of a 4-week history of a pustular eruption that initially developed on the periumbilical skin and subsequently spread to involve the breasts, back, flexures, and proximal limbs, accompanied by cutaneous pain, fever, and malaise. On examination, the lesions were found to be pustules arranged in concentric rings, while on the breasts, there was coalescence of pustules forming lakes of pus. What is the treatment of choice in this case?
Pemphigus Foliaceus Indian Medical PG Practice Questions and MCQs
Question 1: A 70-year-old man comes to the emergency department because of a skin rash and severe itching. He appears ill; there is a generalized skin rash that is scaly, erythematous, and thickened. His palms, soles, and scalp are also involved. Which of the following is the most likely diagnosis?
- A. erythroderma (exfoliative dermatitis) (Correct Answer)
- B. pemphigus vulgaris
- C. dermatitis herpetiformis
- D. rosacea
Explanation: ***erythroderma (exfoliative dermatitis)*** - Erythroderma is characterized by a **generalized erythematous (red), scaly, and thickened skin rash** covering more than 90% of the body surface, accompanied by **severe itching**. - This condition is often associated with a **systemic illness**, and the patient's description of "appears ill" further supports this diagnosis. *pemphigus vulgaris* - Pemphigus vulgaris typically presents with **flaccid blisters** and erosions, particularly affecting mucous membranes, which are not described here. - While it can be widespread, the primary lesion is a **blister** rather than diffuse erythema and scaling. *dermatitis herpetiformis* - Dermatitis herpetiformis is characterized by intensely **pruritic (itchy) papules and vesicles** typically found on the extensor surfaces (e.g., elbows, knees, buttocks). - It is strongly associated with **celiac disease** and does not present as a generalized scaly, erythematous thickening. *rosacea* - Rosacea primarily affects the **face**, causing **erythema**, flushing, papules, and pustules, often sparing the palms, soles, and scalp. - It is not characterized by generalized scaling, thickening, or severe itching over the entire body.
Question 2: Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
- A. Pemphigus vulgaris
- B. Pemphigus foliaceus
- C. Bullous pemphigoid
- D. Dermatitis herpetiformis (Correct Answer)
Explanation: ***Dermatitis herpetiformis*** - The immunofluorescence image shows **granular IgA deposits** at the **dermal papillae region**, which is characteristic of dermatitis herpetiformis. - This condition is strongly associated with **celiac disease** and presents with intensely pruritic papules and vesicles. *Pemphigus vulgaris* - Immunofluorescence in pemphigus vulgaris typically shows a **fishnet pattern** of IgG deposits throughout the **epidermis**, reflecting antibodies against desmoglein 3 and 1. - This pattern is an intercellular deposition, not granular at the dermal papillae. *Pemphigus foliaceus* - Similar to pemphigus vulgaris, pemphigus foliaceus also exhibits **intercellular IgG deposits** in the epidermis, but it is usually more superficial, targeting desmoglein 1. - The image does not show this intercellular epidermal staining. *Bullous pemphigoid* - Bullous pemphigoid is characterized by **linear IgG and C3 deposits along the dermal-epidermal junction** (basement membrane zone). - The image distinctly shows granular IgA, not linear IgG/C3, and specifically in the dermal papillae.
Question 3: Which of the following drugs can lead to pemphigus?
- A. Carbamazepine
- B. Penicillamine (Correct Answer)
- C. Isoniazid
- D. Furosemide
Explanation: ***Penicillamine*** - **Penicillamine** is a well-known drug that can induce **pemphigus**, often through mechanisms involving alterations in **desmosome structure** or function. - The drug's sulfhydryl groups are thought to interfere with the integrity of **desmoglein proteins**, leading to blister formation. *Isoniazid* - **Isoniazid** is a first-line antituberculosis drug primarily associated with **hepatotoxicity** and **peripheral neuropathy**. - It is not typically implicated in the development of **pemphigus**. *Carbamazepine* - **Carbamazepine** is an anticonvulsant that can cause various cutaneous reactions, most notably **Stevens-Johnson syndrome (SJS)** and **toxic epidermal necrolysis (TEN)**. - While it can cause severe skin reactions, **pemphigus** is not a common side effect of carbamazepine. *Furosemide* - **Furosemide** is a loop diuretic that can cause **photosensitivity**, rashes, and rarely, severe skin reactions like **erythema multiforme**. - It is not recognized as a drug that induces **pemphigus**.
Question 4: 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 5: A patient presents with the skin lesions shown in the image. While evaluating for possible blistering disorders, all of the following conditions could present with similar morphology EXCEPT:
- A. Pemphigus vulgaris
- B. Pemphigus erythematosus
- C. Bullous pemphigoid (Correct Answer)
- D. Pemphigus vegetans
Explanation: ***Bullous pemphigoid*** - Presents with **tense bullae** on an erythematous base, typically in elderly patients, unlike the **umbilicated papules** seen in this image. - Involves **subepidermal blistering** with **linear IgG deposition** at the basement membrane zone, not the viral inclusions of Molluscum contagiosum. *Pemphigus vegetans* - A rare variant of pemphigus vulgaris characterized by **vegetating plaques and pustules** in intertriginous areas, not discrete umbilicated lesions. - Shows **intraepidermal acantholysis** with **suprabasal clefting**, histologically distinct from the viral cytopathic changes in Molluscum contagiosum. *Pemphigus vulgaris* - Presents with **flaccid bullae** and painful **mucosal erosions** due to **autoantibodies against desmoglein 1 and 3**. - The **Nikolsky sign** is positive, and lesions are erosive rather than the solid, pearl-like papules characteristic of Molluscum contagiosum. *Pemphigus erythematosus* - Features **erythematous, scaly, crusted lesions** primarily on the **face and upper trunk** with a butterfly distribution. - Combines features of **lupus erythematosus** and pemphigus foliaceus, showing superficial blistering unlike the viral papules in this case.
Question 6: A child presents with grouped vesicles on an erythematous base on the buttocks. What is the most likely diagnosis?
- A. Bullous impetigo
- B. Dermatitis herpetiformis
- C. Pemphigus
- D. Herpes simplex (Correct Answer)
Explanation: ***Herpes simplex*** - Herpes simplex virus (HSV) classically presents with **grouped vesicles on an erythematous base**, which perfectly matches this clinical presentation. - In **children**, HSV commonly affects the **buttocks** through autoinoculation or direct contact, especially in the diaper area. - The lesions are typically **painful and pruritic**, and may be preceded by tingling or burning sensation. - Diagnosis is confirmed by **Tzanck smear** (multinucleated giant cells), **PCR**, or **viral culture**. - Treatment includes **acyclovir** or other antivirals, especially for severe or recurrent cases. *Dermatitis herpetiformis* - While DH does present with intensely pruritic, grouped vesicles on an erythematous base, it is **extremely rare in children** and typically presents in **adults (3rd-4th decade)**. - Classic sites include **extensor surfaces** (elbows, knees), scalp, and buttocks, but the pediatric presentation makes this diagnosis unlikely. - It is strongly associated with **celiac disease** and responds to **gluten-free diet** and **dapsone**. *Bullous impetigo* - Bullous impetigo presents with **flaccid bullae** that rupture to form **honey-colored crusts**, not grouped vesicles. - It is a **bacterial infection** caused by *Staphylococcus aureus* producing exfoliative toxin. - Common in **young children**, particularly in warm, humid conditions. *Pemphigus* - Pemphigus is **extremely rare in children** and causes **fragile bullae** that easily rupture, leading to erosions. - Typically affects **mucous membranes first** (oral cavity), then skin. - It is an **autoimmune blistering disease** with antibodies against desmoglein, causing intraepidermal acantholysis.
Question 7: Which of the following is NOT associated with erythema nodosum?
- A. Leprosy
- B. Tuberculosis
- C. Pemphigus vulgaris (Correct Answer)
- D. Sarcoidosis
Explanation: ***Pemphigus vulgaris*** - Pemphigus vulgaris is an **autoimmune blistering disease** affecting the skin and mucous membranes, characterized by **flaccid bullae** that easily rupture. - Its pathogenesis involves autoantibodies against **desmogleins 1 and 3**, components of desmosomes, and it does not typically manifest with subcutaneous nodules or inflammation seen in erythema nodosum. *Leprosy* - Leprosy, particularly its **lepromatous forms**, can cause immunologically mediated inflammatory reactions known as **erythema nodosum leprosum**. - This presentation involves multiple tender, inflamed subcutaneous nodules, clinically and histologically resembling typical erythema nodosum. *Tuberculosis* - Tuberculosis is a well-known infectious cause of **erythema nodosum**, especially in young adults and children. - The skin lesions often indicate a **hypersensitivity reaction** to the mycobacterial antigens. *Sarcoidosis* - Sarcoidosis is a systemic granulomatous disease, and **erythema nodosum** is a common **cutaneous manifestation**, particularly in acute sarcoidosis. - When associated with bilateral hilar lymphadenopathy and arthralgia, it forms **Löfgren's syndrome**, a specific presentation of sarcoidosis.
Question 8: A 50-year-old female presents with blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. What is the most likely diagnosis?
- A. Pemphigus vulgaris (Correct Answer)
- B. Dermatitis herpetiformis
- C. Bullous pemphigoid
- D. Erythema multiforme
Explanation: ***Pemphigus vulgaris*** - This condition is characterized by **flaccid blisters** that easily rupture, leading to painful erosions, often starting in the **oral mucosa** (50-70% of cases). - It is an **autoimmune disease** with antibodies against **desmoglein 3 (mucous membranes) and desmoglein 1 (skin)**, causing **intraepidermal blistering**. - **Nikolsky sign positive** - lateral pressure causes sloughing of skin. *Dermatitis herpetiformis* - This presents with intensely **pruritic (itchy) grouped vesicles** on extensor surfaces (elbows, knees, buttocks), not flaccid blisters with oral involvement. - It is strongly associated with **celiac disease** and characterized by **IgA deposition in the dermal papillae**. *Bullous pemphigoid* - Presents with **tense blisters** on an erythematous base, typically in **elderly patients (>60 years)**. - **Oral mucosa usually spared** or involved late in the disease course. - Involves autoantibodies against **hemidesmosomal proteins (BP180 and BP230)**, leading to **subepidermal blistering**. *Erythema multiforme* - Characterized by **target lesions** and can involve mucous membranes, but typically presents acutely with symmetrical distribution. - Often triggered by **infections (HSV) or medications**, not an autoimmune blistering disease. - Lacks the chronic, progressive oral erosions typical of pemphigus vulgaris.
Question 9: Which histological finding is most specific for pemphigus vulgaris?
- A. Basement membrane thickening
- B. Row of tombstones (Correct Answer)
- C. Granular IgA deposits
- D. IgG linear deposits
Explanation: ***Row of tombstones*** - This describes the appearance of a single layer of **basal keratinocytes** remaining attached to the basement membrane, resembling tombstones, while the overlying epidermal cells have detached due to **acantholysis**. - It is a highly characteristic histological feature of **pemphigus vulgaris**, resulting from the autoimmune attack on **desmogleins 1 and 3**, leading to loss of cell-to-cell adhesion. *Basement membrane thickening* - While seen in some chronic skin conditions, **basement membrane thickening** is not specific to pemphigus vulgaris and is more characteristic of diseases like **lupus erythematosus**. - Pemphigus vulgaris is primarily an intraepidermal blistering disease, with the primary pathology located within the epidermis, not at the dermal-epidermal junction. *Granular IgA deposits* - **Granular IgA deposits** at the dermal papillae are the hallmark of **dermatitis herpetiformis**, a pruritic blistering disease associated with celiac disease. - Pemphigus vulgaris involves **IgG autoantibodies** against desmogleins, not IgA, and these deposits are typically seen in a net-like or intercellular pattern. *IgG linear deposits* - **Linear IgG deposits** along the **dermal-epidermal junction** are characteristic of **bullous pemphigoid**, another autoimmune blistering disease. - In contrast, pemphigus vulgaris shows a **net-like deposition of IgG** throughout the epidermal intercellular spaces, reflecting the distribution of desmogleins.
Question 10: A 26-year-old girl at 31 weeks' gestation complains of a 4-week history of a pustular eruption that initially developed on the periumbilical skin and subsequently spread to involve the breasts, back, flexures, and proximal limbs, accompanied by cutaneous pain, fever, and malaise. On examination, the lesions were found to be pustules arranged in concentric rings, while on the breasts, there was coalescence of pustules forming lakes of pus. What is the treatment of choice in this case?
- A. Corticosteroids (Correct Answer)
- B. Methotrexate
- C. Topical itraconazole
- D. Third generation cephalosporins
Explanation: ***Corticosteroids*** - The constellation of **pustular eruption** with **concentric rings**, especially in pregnancy, along with systemic symptoms like fever and malaise, is highly suggestive of **Pustular Psoriasis of Pregnancy (PPP)**, also known as **Impetigo Herpetiformis**. - **Systemic corticosteroids** (prednisolone) are the **first-line treatment** for this rare but severe dermatosis in pregnancy, aimed at controlling inflammation and preventing maternal and fetal complications. *Methotrexate* - **Methotrexate** is an **anti-metabolite** and **immunosuppressant** that is **teratogenic** and absolutely **contraindicated in pregnancy** due to its potential to cause severe birth defects and miscarriages. - While it can be used for severe psoriasis in non-pregnant individuals, its use in this pregnant patient is inappropriate and dangerous. *Topical itraconazole* - **Itraconazole** is an **antifungal medication** used to treat fungal infections. While typically used systemically, topical formulations exist for localized fungal infections. - The clinical presentation of widespread pustules arranged in **concentric rings** with systemic symptoms and **"lakes of pus"** in a pregnant woman is pathognomonic for **Impetigo Herpetiformis**, not a fungal infection, making this treatment inappropriate. *Third generation cephalosporins* - **Third-generation cephalosporins** are **antibiotics** primarily used to treat bacterial infections. - The distinctive pattern of **pustules in concentric rings**, the periumbilical onset, and progression to form **lakes of pus** in a pregnant woman represents an **inflammatory dermatosis** (Impetigo Herpetiformis), not a primary bacterial infection requiring antibiotics.
Want unlimited practice?
Get full access to all questions, explanations, and performance tracking.
Start For Free