Cicatricial Pemphigoid — MCQs
Which of the following is NOT a characteristic of pemphigus vulgaris?
Koebner's phenomenon is seen in all EXCEPT:
Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
Which of the following statements about mucous membrane pemphigoid is correct?
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 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?
The following lesion appears on the leg of a patient of ulcerative colitis. All are useful in management except:
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:
Pemphigus vulgaris is caused by what type of process?
Which of the following presents with a "string of pearls appearance"?
Cicatricial 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: Koebner's phenomenon is seen in all EXCEPT:
- A. Psoriasis
- B. Warts
- C. Lichen planus
- D. Pemphigoid (Correct Answer)
Explanation: ***Pemphigoid*** - **Pemphigoid** is an autoimmune blistering skin condition not typically associated with the Koebner phenomenon. - In pemphigoid, **bullae** arise from an autoimmune attack on **hemidesmosomes**, not from isomorphic response to trauma. *Psoriasis* - **Psoriasis** is a classic example of a condition exhibiting the Koebner phenomenon, where isomorphic lesions develop at sites of trauma. - This response is due to **keratinocyte proliferation** triggered by injury in genetically predisposed individuals. *Warts* - **Warts** (verrucae) can demonstrate the Koebner phenomenon, meaning that new warts can emerge along a line of trauma or scratching. - This is attributed to the local spread of the **human papillomavirus (HPV)** through minor excoriations. *Lichen planus* - **Lichen planus** frequently exhibits the Koebner phenomenon, where characteristic **purplish, polygonal papules** emerge at sites of skin injury. - This isomorphic response is a common diagnostic clue in active disease.
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: 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 5: 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 6: 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 7: The following lesion appears on the leg of a patient of ulcerative colitis. All are useful in management except:
- A. Steroids
- B. Sulfapyridine (Correct Answer)
- C. Procto-colectomy
- D. Infliximab
Explanation: ***Sulfapyridine*** - The image shows **pyoderma gangrenosum**, a painful ulcerative skin condition often associated with inflammatory bowel disease like ulcerative colitis. Among the given options, **sulfapyridine** has the **least established role** in pyoderma gangrenosum management. - **Sulfapyridine** is an inactive component of **sulfasalazine** and primarily acts as an **antibacterial agent**. While sulfasalazine has been reported in some PG cases, sulfapyridine alone is not a recognized treatment for the inflammatory, non-infectious nature of pyoderma gangrenosum. - Unlike the other options which have well-established roles, sulfapyridine lacks strong evidence for efficacy in PG. *Steroids* - **Corticosteroids** (oral or topical) are the **first-line treatment** for pyoderma gangrenosum due to their potent anti-inflammatory and immunosuppressive effects. - They help to reduce the inflammation and promote healing of the painful ulcers. *Procto-colectomy* - In cases of severe, refractory pyoderma gangrenosum associated with ulcerative colitis, **colectomy** can be a **definitive treatment** as it removes the underlying inflammatory trigger. - This surgical intervention is considered when medical therapies are unsuccessful or when the colonic disease itself necessitates surgery. *Infliximab* - **Infliximab**, a **TNF-alpha inhibitor**, is a biologic agent effective in treating both ulcerative colitis and pyoderma gangrenosum. - It is used in cases that are refractory to steroids or when patients cannot tolerate steroid therapy.
Question 8: 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 9: Pemphigus vulgaris is caused by what type of process?
- A. Bacterial infection
- B. Viral infection
- C. Autoimmune process (Correct Answer)
- D. Fungal infection
Explanation: **Explanation:** **Pemphigus vulgaris (PV)** is a chronic, life-threatening, intraepidermal blistering disease. The correct answer is **Autoimmune process** because PV is characterized by the production of IgG autoantibodies against **Desmogleins (Dsg1 and Dsg3)**. These are cadherin-type glycoproteins that form the "glue" of desmosomes, which hold epidermal keratinocytes together. When these antibodies bind, they cause a loss of cell-to-cell adhesion, a process known as **acantholysis**, leading to the formation of flaccid blisters. **Why other options are incorrect:** * **Bacterial, Viral, and Fungal infections:** While secondary infections (especially *Staphylococcus aureus*) are common complications due to denuded skin, the primary etiology of PV is not infectious. Blistering diseases caused by infections include Impetigo (bacterial) or Herpes Simplex (viral), which have distinct clinical and histological features. **High-Yield NEET-PG Clinical Pearls:** * **Target Antigens:** Dsg3 (primarily mucosal lesions) and Dsg1 (skin involvement). * **Clinical Presentation:** Flaccid bullae that rupture easily, leaving painful erosions. Oral mucosa is often the first site involved. * **Key Signs:** **Nikolsky sign** is positive (perilesional skin shears off with lateral pressure) and **Asboe-Hansen sign** is positive (bulla spreads laterally when pressed). * **Histopathology:** Shows a "row of tombstones" appearance (basal layer remains attached to the basement membrane) and acantholytic cells (Tzanck cells). * **Immunofluorescence:** Direct Immunofluorescence (DIF) shows a characteristic **"fishnet" or "lace-like" pattern** of IgG and C3 deposits.
Question 10: Which of the following presents with a "string of pearls appearance"?
- A. Pemphigus vulgaris
- B. Bullous pemphigoid
- C. Linear IgA disease (Correct Answer)
- D. Dermatitis herpetiformis
Explanation: **Explanation:** **Linear IgA Bullous Dermatosis (LABD)** is an autoimmune subepidermal blistering disease characterized by the linear deposition of IgA antibodies along the basement membrane zone. The "string of pearls" (or "cluster of jewels") appearance is a classic clinical descriptor for this condition, referring to the characteristic arrangement of new vesicles and bullae at the periphery of old, healing lesions. **Why the correct answer is right:** * **Linear IgA Disease:** The "string of pearls" appearance occurs because new blisters form in a ring-like (annular or polycyclic) pattern around a central crust or resolving lesion. This is a high-yield clinical sign often tested in NEET-PG. **Why the other options are incorrect:** * **Pemphigus Vulgaris:** Characterized by flaccid bullae and a positive **Nikolsky sign**. Histopathology shows "row of tombstones" appearance due to suprabasal acantholysis. * **Bullous Pemphigoid:** Presents with large, tense bullae in elderly patients. Direct Immunofluorescence (DIF) shows linear **IgG** and C3, not IgA. * **Dermatitis Herpetiformis:** Associated with Celiac disease. It presents with intensely pruritic, grouped (herpetiform) vesicles on extensor surfaces. DIF shows **granular IgA** deposits in dermal papillae. **Clinical Pearls for NEET-PG:** * **Drug-induced LABD:** Most commonly caused by **Vancomycin**. * **Target Antigen:** BP180 (collagen XVII) or LAD-1. * **Treatment of Choice:** **Dapsone** is the first-line treatment for Linear IgA disease. * **DIF Finding:** Continuous linear band of IgA along the dermo-epidermal junction.
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