Immunofluorescence in Bullous Diseases — MCQs
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
Which immunofluorescence finding is characteristic of dermatitis herpetiformis?
Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
A skin biopsy shows 'crown of thorns' pattern on immunofluorescence. Which additional finding would confirm IgA vasculitis?
A skin biopsy shows suprabasilar acantholysis with 'row of tombstones' appearance. Which immunofluorescence pattern would confirm 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 60-year-old female presents with eczematous itching lesions. Biopsy revealed a subepidermal cleft with Direct Immunofluorescence showing Linear C3 & IgG deposition along the basement membrane zone. What is the likely diagnosis?
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?
Immunofluorescence in Bullous Diseases Indian Medical PG Practice Questions and MCQs
Question 1: 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 2: Which immunofluorescence finding is characteristic of dermatitis herpetiformis?
- A. IgG deposits
- B. Granulomas
- C. IgA deposits (Correct Answer)
- D. Suprabasal acantholysis
Explanation: ***IgA deposits*** - **Direct immunofluorescence** studies in dermatitis herpetiformis characteristically show **granular IgA deposits** in the dermal papillae. - These IgA deposits are pathognomonic for the condition and are strongly associated with **celiac disease** (gluten-sensitive enteropathy). - This finding is the **gold standard** for diagnosis and distinguishes DH from other bullous diseases. *IgG deposits* - **IgG deposits** are typically found in other bullous diseases like **pemphigus vulgaris** and **bullous pemphigoid**, not dermatitis herpetiformis. - Their presence would indicate an autoimmune response directed against **desmosomal** or **hemidesmosomal proteins**. *Granulomas* - **Granulomas** are aggregates of macrophages and are characteristic of conditions like **tuberculosis**, **sarcoidosis**, or **deep fungal infections**. - They are not a feature of immunofluorescence studies in blistering diseases. *Suprabasal acantholysis* - **Suprabasal acantholysis**, the loss of cohesion between keratinocytes above the basal layer, is the hallmark of **pemphigus vulgaris** seen on histology. - This histological finding leads to intraepidermal blistering, which is distinct from the subepidermal blisters seen in dermatitis herpetiformis.
Question 3: 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 4: A skin biopsy shows 'crown of thorns' pattern on immunofluorescence. Which additional finding would confirm IgA vasculitis?
- A. Leukocytoclastic vasculitis (Correct Answer)
- B. Granulomatous inflammation
- C. Interface dermatitis
- D. Eosinophilic spongiosis
Explanation: ***Leukocytoclastic vasculitis*** - **IgA vasculitis**, also known as Henoch-Schönlein purpura, is characterized by **leukocytoclastic vasculitis** on skin biopsy, which involves fragmentation of neutrophils and fibrinoid necrosis of vessel walls [1]. - The "crown of thorns" pattern on immunofluorescence refers to the **perivascular IgA deposition** seen in IgA vasculitis, which is pathognomonic when coupled with the histological finding of leukocytoclastic vasculitis. *Granulomatous inflammation* - This type of inflammation is characterized by aggregates of **macrophages** (histiocytes) and is typical of conditions like **tuberculosis**, sarcoidosis, or certain fungal infections, not IgA vasculitis. - **Granulomas** form as a defense mechanism to wall off foreign substances or pathogens that cannot be eliminated by other inflammatory responses. *Eosinophilic spongiosis* - **Eosinophilic spongiosis** is characterized by intercellular edema within the epidermis accompanied by **eosinophil infiltration**, typically seen in **allergic contact dermatitis**, drug reactions, or pemphigus. - This pattern reflects an allergic or hypersensitivity response rather than a vasculitic process, and is not a feature of IgA vasculitis. *Interface dermatitis* - **Interface dermatitis** is a pattern of inflammation at the dermo-epidermal junction, typically seen in conditions like **lichen planus**, lupus erythematosus, or erythema multiforme. - It is characterized by vacuolar degeneration of basal keratinocytes and a prominent lymphocytic infiltrate at the junction, which is fundamentally different from a vasculitic process. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 278-280.
Question 5: A skin biopsy shows suprabasilar acantholysis with 'row of tombstones' appearance. Which immunofluorescence pattern would confirm pemphigus vulgaris?
- A. Linear IgA deposits
- B. Fishnet pattern of IgG (Correct Answer)
- C. Granular IgG deposits
- D. Linear C3 deposits
Explanation: ***Fishnet pattern of IgG*** - Pemphigus vulgaris is characterized by **autoantibodies** (predominantly **IgG**) directed against **desmogleins 1 and 3**, components of desmosomes in the epidermis. - This binding leads to the **loss of cell adhesion** (acantholysis) and results in the characteristic **net-like or "fishnet" pattern** of IgG positivity on direct immunofluorescence (DIF), outlining keratinocyte cell surfaces. *Linear IgA deposits* - **Linear IgA bullous dermatosis** is characterized by the presence of continuous linear deposits of IgA along the **dermoepidermal junction** on direct immunofluorescence. - This condition is clinically and immunologically distinct from pemphigus vulgaris, which involves **intraepidermal deposition of IgG**. *Granular IgG deposits* - **Granular IgG deposits** at the dermoepidermal junction are typically seen in conditions like **lupus erythematosus**, particularly in the form of a **lupus band test**, rather than in blistering diseases like pemphigus. - Pemphigus involves **intercellular IgG deposition** within the epidermis, not granular deposits at the junction. *Linear C3 deposits* - **Linear C3 deposits** (often accompanied by IgG) along the **dermoepidermal junction** are characteristic of **bullous pemphigoid**, another autoimmune blistering disease. - Unlike pemphigus vulgaris, bullous pemphigoid involves autoantibodies targeting hemidesmosomes, leading to subepidermal blistering rather than intraepidermal acantholysis.
Question 6: 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 7: 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 8: A 60-year-old female presents with eczematous itching lesions. Biopsy revealed a subepidermal cleft with Direct Immunofluorescence showing Linear C3 & IgG deposition along the basement membrane zone. What is the likely diagnosis?
- A. Pemphigus foliaceus
- B. Pemphigus Vulgaris
- C. Dermatitis herpetiformis
- D. Bullous Pemphigoid (Correct Answer)
Explanation: ***Bullous Pemphigoid*** - The presence of **eczematous itching lesions**, a **subepidermal cleft**, and **linear C3 and IgG deposition along the basement membrane zone** on direct immunofluorescence (DIF) are classic diagnostic features of Bullous Pemphigoid. - This autoimmune blistering disease typically affects older individuals and is characterized by antibodies targeting components of the **hemidesmosomes**, specifically BP180 and BP230. *Pemphigus foliaceus* - This condition involves **intraepidermal blistering**, specifically within the granular layer, rather than a subepidermal cleft. - DIF in Pemphigus foliaceus shows **intercellular IgG deposition** in the epidermis, not linear deposition along the basement membrane zone. *Pemphigus Vulgaris* - Pemphigus Vulgaris is characterized by **intraepidermal blistering** above the basal cell layer (**suprabasal clefting**), leading to fragile bullae that rupture easily. - DIF typically reveals **intercellular IgG and C3 deposition** in a "chicken wire" pattern throughout the epidermis, which differs from the linear pattern seen in this case. *Dermatitis herpetiformis* - While Dermatitis herpetiformis is also an autoimmune blistering disease with itching lesions, its characteristic DIF finding is **granular IgA deposition** in the dermal papillae, not linear C3 and IgG at the basement membrane zone. - Histopathology in Dermatitis herpetiformis shows **subepidermal vesicles** with neutrophil infiltration in the dermal papillae, but the direct immunofluorescence pattern is distinct.
Question 9: 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 10: Which of the following is NOT associated with erythema nodosum?
- A. Pemphigus vulgaris (Correct Answer)
- B. Tuberculosis
- C. Sarcoidosis
- D. Leprosy
Explanation: ***Pemphigus vulgaris*** - **Pemphigus vulgaris** is an **autoimmune blistering disease** that affects the skin and mucous membranes, characterized by flaccid bullae, not subcutaneous nodules. - Its pathophysiology involves **autoantibodies** against **desmoglein 1 and 3**, leading to **acantholysis**, which is distinct from the inflammatory changes seen in erythema nodosum. *Tuberculosis* - **Tuberculosis (TB)** is a common infectious cause of **erythema nodosum**, especially in regions with high TB prevalence. - The development of erythema nodosum in TB is often considered a **hypersensitivity reaction** to mycobacterial antigens. *Sarcoidosis* - **Sarcoidosis** is a systemic granulomatous disease, and **erythema nodosum** can be a prominent cutaneous manifestation, particularly in **Löfgren's syndrome**. - Its presence with **bilateral hilar lymphadenopathy** and **arthralgia** is highly suggestive of acute sarcoidosis. *Leprosy* - **Leprosy**, caused by *Mycobacterium leprae*, can be associated with **erythema nodosum leprosum (ENL)**, which is a type 2 lepra reaction. - **ENL** involves the formation of painful, tender, inflamed nodules that resemble erythema nodosum and is linked to elevated immune complex deposition.
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