Management of Autoimmune Bullous Diseases — MCQs
A 30-year-old woman presents with flaccid bullae on her skin that are easy to rupture. A biopsy of the lesion reveals a suprabasal split. What is the most likely diagnosis?
All of the following are used in systemic therapy of psoriasis except
A patient presents with itchy skin lesions with blistering along with gastrointestinal issues. Which of the following is the most specific serological test for this condition?
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 -
All of the following diseases cause intraepidermal bullae except:
A young female with asymptomatic macules and papules over trunk and reddish patch over palate with a flat, moist lesion on vulva. Patient has generalized lymphadenopathy. What is the line of management?
Which of the following HLA types is associated with dermatitis herpetiformis?
A 85-year-old woman has large blistering lesions on the abdomen and thighs that come and go without therapy, and Nikolsky sign is negative. Which of the following is the most likely diagnosis?
A skin biopsy shows acantholysis with intraepidermal blistering. Which immunofluorescence pattern would confirm pemphigus vulgaris?
Which of the following treatment options is not useful in the management of Guillain-Barré syndrome?
Management of Autoimmune Bullous Diseases Indian Medical PG Practice Questions and MCQs
Question 1: A 30-year-old woman presents with flaccid bullae on her skin that are easy to rupture. A biopsy of the lesion reveals a suprabasal split. What is the most likely diagnosis?
- A. Erythema multiforme
- B. Pemphigus vegetans
- C. Pemphigus vulgaris (Correct Answer)
- D. Pemphigus foliaceous
Explanation: ***Pemphigus vulgaris*** - Characterized by **flaccid bullae** that are easily ruptured, and a classic histological finding of a **suprabasal split** in the epidermis, indicating acantholysis just above the basal layer. - Mucosal involvement is common, and the positive **Nikolsky sign** (epidermal detachment with lateral pressure) is often present, which is typical for pemphigus vulgaris due to the superficial nature of the blistering. - The combination of **flaccid bullae + suprabasal split** is pathognomonic for pemphigus vulgaris. *Erythema multiforme* - Typically presents with **targetoid lesions** (concentric rings of erythema) and is often associated with infections, particularly herpes simplex virus (HSV). - Histologically, it shows **interface dermatitis** with vacuolar degeneration of basal cells and scattered necrotic keratinocytes, not a suprabasal split or acantholysis. *Pemphigus vegetans* - A rare variant of pemphigus vulgaris, it presents with **vegetating plaques** in intertriginous areas (axillae, groin), which are eroded but not primarily flaccid bullae covering wide areas. - While it also involves a suprabasal split at the same level as pemphigus vulgaris, the clinical presentation of vegetating plaques rather than widespread flaccid bullae helps differentiate it. *Pemphigus foliaceous* - This autoimmune blistering disease features very **superficial bullae** that rupture so easily they typically present as erosions, crusts, and scaling rather than intact blisters. - Histologically, it shows a **subcorneal or granular layer split** (more superficial than pemphigus vulgaris), not the deeper suprabasal split seen in this patient's biopsy. - Mucosal involvement is **rare** in pemphigus foliaceous, unlike pemphigus vulgaris.
Question 2: All of the following are used in systemic therapy of psoriasis except
- A. Methotrexate
- B. Cyclosporine
- C. Oral glucocorticoids (Correct Answer)
- D. Acitretin
Explanation: ***Oral glucocorticoids*** - **Oral glucocorticoids** are generally avoided in psoriasis because they can precipitate severe **rebound flares** upon discontinuation or during dose tapering. - While they can temporarily suppress inflammation, the risk of worsening psoriasis and other systemic side effects makes them unsuitable for long-term systemic therapy. *Methotrexate* - **Methotrexate** is a commonly used systemic agent for psoriasis due to its **immunosuppressive** and **anti-proliferative effects**, targeting rapidly dividing cells. - It works by inhibiting dihydrofolate reductase and is typically given once weekly for chronic plaque psoriasis. *Cyclosporine* - **Cyclosporine** is an effective systemic immunosuppressant used for severe, resistant psoriasis, particularly when rapid control is needed. - It primarily acts by inhibiting **T-cell activation** and proliferation, thereby reducing the inflammatory response in psoriasis. *Acitretin* - **Acitretin** is an oral retinoid derivative of vitamin A, used in severe forms of psoriasis, especially **pustular** and **erythrodermic** types. - It works by modulating **keratinocyte differentiation** and proliferation, helping to normalize skin cell growth.
Question 3: A patient presents with itchy skin lesions with blistering along with gastrointestinal issues. Which of the following is the most specific serological test for this condition?
- A. Anti-TTG antibody
- B. Anti-nuclear antibody
- C. Anti-endomysial antibody (Correct Answer)
- D. IgA deposits at the dermoepidermal junction
- E. Anti-desmoglein antibody
Explanation: ***Anti-endomysial antibody*** - The combination of **itchy, blistering skin lesions** and **gastrointestinal issues** is highly suggestive of **Dermatitis Herpetiformis**, which is the cutaneous manifestation of **celiac disease**. - **Anti-endomysial antibody (EMA)**, particularly IgA, is highly specific (nearly 100%) for **celiac disease** and thus for Dermatitis Herpetiformis, especially when tested on primate esophagus. *Anti-TTG antibody* - **Anti-tissue transglutaminase (tTG) antibody** (IgA) is a sensitive and specific serological marker for **celiac disease** and is often the first-line test. - While highly indicative, **EMA** is generally considered to have slightly higher specificity than tTG for celiac disease, particularly in predicting intestinal villous atrophy. *Anti-nuclear antibody* - **Anti-nuclear antibodies (ANA)** are primarily associated with **systemic autoimmune diseases** like Systemic Lupus Erythematosus. - They are not specific for **celiac disease** or **Dermatitis Herpetiformis**. *Anti-desmoglein antibody* - **Anti-desmoglein antibodies** (anti-Dsg1 and anti-Dsg3) are specific for **pemphigus vulgaris** and **pemphigus foliaceus**, which are autoimmune blistering disorders. - While these conditions present with blistering, they typically lack the gastrointestinal symptoms and the specific pruritic, grouped vesicular pattern seen in **Dermatitis Herpetiformis**. - This is not the appropriate serological test for DH/celiac disease. *IgA deposits at the dermoepidermal junction* - The presence of **granular IgA deposits at the dermoepidermal junction** (dermal papillae) is the **gold standard for diagnosing Dermatitis Herpetiformis** through **direct immunofluorescence** of a skin biopsy. - However, this is a **histopathological finding**, not a serological test, and therefore does not fit the question's criteria for a "serological test."
Question 4: 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 5: All of the following diseases cause intraepidermal bullae except:
- A. Miliaria rubra
- B. Herpes gestationalis (Correct Answer)
- C. Herpes zoster
- D. Pemphigus
Explanation: ***Herpes gestationalis.*** - Herpes gestationalis is characterized by **urticarial papules** and vesicles, typically occurs in pregnancy, and does not form **intraepidermal bullae**. - This condition is linked more with **dermatitis herpetiformis** rather than with the intraepidermal blistering seen in the other options [2]. *Herpes zoster* - Herpes zoster causes **vesicular lesions** that are often grouped, presenting as painful **erythematous vesicles** along a dermatome. - The lesions can form intraepidermal bullae due to the **varicella-zoster virus** affecting the skin [1]. *Miliaria rubra* - Miliaria rubra, or **heat rash**, results from occluded sweat glands leading to **superficial vesicles or papules** in the epidermis. - The lesions may resemble blisters but are not true intraepidermal bullae and are prominent in hot, humid conditions. *Pemphigus* - Pemphigus is an autoimmune disorder causing **flaccid bullae** due to **acantholysis** in the epidermis, leading to intraepidermal bulla formation [3]. - It is characterized by **painful, fragile blisters** that rupture easily, differentiating it from other conditions listed [3]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Infectious Diseases, p. 366. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Skin, pp. 1172-1174. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Skin, pp. 1170-1172.
Question 6: A young female with asymptomatic macules and papules over trunk and reddish patch over palate with a flat, moist lesion on vulva. Patient has generalized lymphadenopathy. What is the line of management?
- A. Fluconazole
- B. Ceftriaxone
- C. Benzathine penicillin (Correct Answer)
- D. Acyclovir
Explanation: ***Benzathine penicillin*** - This clinical presentation, including **asymptomatic macules and papules** on the trunk, a **reddish palatal patch**, a **flat, moist vulval lesion (condyloma lata)**, and **generalized lymphadenopathy**, is highly suggestive of **secondary syphilis** [1]. - **Benzathine penicillin G** is the *drug of choice* for treating all stages of syphilis, particularly effective for early syphilis like this manifestation. *Fluconazole* - **Fluconazole** is an **antifungal medication** primarily used to treat *candidiasis* and other fungal infections [1]. - The symptoms described are *not characteristic* of a fungal infection. *Ceftriaxone* - **Ceftriaxone** is a *beta-lactam antibiotic* used to treat a wide range of bacterial infections, especially *gonorrhea*, *meningitis*, and *respiratory tract infections*. - While a potent antibiotic, it is *not the primary treatment* for syphilis, which requires penicillin. *Acyclovir* - **Acyclovir** is an *antiviral drug* used to treat *herpes simplex virus* infections (e.g., genital herpes, cold sores) and *varicella-zoster virus* [1]. - The lesions described, particularly the *flat, moist condyloma lata* and *generalized maculopapular rash*, are *not typical manifestations of herpes* [1].
Question 7: Which of the following HLA types is associated with dermatitis herpetiformis?
- A. HLA B8 (Correct Answer)
- B. HLA A5
- C. HLA A28
- D. HLA B27
Explanation: ***HLA B8*** - **HLA B8** is associated with **dermatitis herpetiformis**, an autoimmune blistering skin condition characterized by intensely pruritic vesicles. - HLA B8 is part of the extended haplotype (HLA-A1, B8, DR3, DQ2) commonly found in patients with dermatitis herpetiformis. - The **strongest association** is actually with **HLA-DQ2** and **HLA-DQ8** (found in ~95% of DH patients), as DH is closely linked with **celiac disease** and shares the same HLA associations. - Among the options listed, **HLA B8** is the one with a recognized association. *HLA A5* - **HLA A5** has no established association with **dermatitis herpetiformis**. - It is not considered a genetic risk factor for this condition. *HLA A28* - **HLA A28** has no significant association with **dermatitis herpetiformis**. - It does not confer increased susceptibility to this autoimmune blistering disorder. *HLA B27* - **HLA B27** is strongly associated with **seronegative spondyloarthropathies** including **ankylosing spondylitis**, **reactive arthritis**, and **psoriatic arthritis**. - It is not associated with dermatitis herpetiformis or other autoimmune blistering diseases.
Question 8: A 85-year-old woman has large blistering lesions on the abdomen and thighs that come and go without therapy, and Nikolsky sign is negative. Which of the following is the most likely diagnosis?
- A. pemphigus vulgaris
- B. dermatitis herpetiformis
- C. bullous pemphigoid (Correct Answer)
- D. herpes gestationis
Explanation: ***Bullous pemphigoid*** - This condition typically affects **elderly individuals** with large, tense bullae that often resolve spontaneously and a negative **Nikolsky sign**. - **Immunofluorescence** shows IgG and C3 deposits along the **dermal-epidermal junction**. *Pemphigus vulgaris* - Characterized by **flaccid bullae** that rupture easily and a **positive Nikolsky sign**, which is not seen here. - Patients are usually younger and involvement of **mucous membranes** is common. *Dermatitis herpetiformis* - This condition presents with **pruritic papulovesicular lesions** arranged in a herpetiform pattern. - It is associated with **celiac disease** and responds to a gluten-free diet. *Herpes gestationis* - Also known as **pemphigoid gestationis**, this rare autoimmune blistering disease occurs during **pregnancy or postpartum**. - It presents with **urticarial plaques and bullae**, primarily on the abdomen and extremities, but is not relevant to an 85-year-old woman.
Question 9: 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 10: Which of the following treatment options is not useful in the management of Guillain-Barré syndrome?
- A. Plasmapheresis
- B. Intra-venous immunoglobulin
- C. Physical medical rehabilitation
- D. Intra-venous methotrexate (Correct Answer)
Explanation: ***Intra-venous methotrexate*** - **Methotrexate** is an **immunosuppressant** used in conditions like **rheumatoid arthritis** or certain cancers, but it has no established role in treating the acute phase of **Guillain-Barré Syndrome (GBS)**. - Its mechanism of action and side effect profile are not suitable for the rapid-onset, immune-mediated demyelination characteristic of GBS, and it could potentially worsen the condition or delay recovery. *Intra-venous immunoglobulin* - **Intravenous immunoglobulin (IVIg)** is a standard treatment for GBS that works by providing a concentrated dose of pooled human antibodies to neutralize pathogenic autoantibodies and modulate the immune response. - It is effective in reducing the severity and duration of GBS symptoms, improving recovery rates and decreasing the need for mechanical ventilation. *Plasmapheresis* - **Plasmapheresis (plasma exchange)** is another effective first-line treatment for GBS, involving the removal of plasma from the blood to filter out harmful antibodies and inflammatory mediators that contribute to nerve damage. - It helps to reduce the autoimmune attack on the peripheral nerves, leading to faster recovery and reduced neurological deficits. *Physical medical rehabilitation* - **Physical medical rehabilitation** is crucial for long-term recovery in GBS, focusing on regaining muscle strength, movement, and functional independence once the acute phase has passed. - It includes **physical therapy**, **occupational therapy**, and **speech therapy** to address residual weakness, fatigue, and other neurological deficits, helping patients adapt and improve their quality of life.
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