Pemphigoid Gestationis — 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
Acantholytic cells in pemphigus are derived from:
Which of the following statements is true regarding pemphigus vulgaris?
Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
Which of the following statements about mucous membrane pemphigoid is correct?
A 5-month-old child presented to the dermatology OPD with dryness along with white, fine scales on most parts of the body with sparing of face. The child was born at 39 weeks gestation by spontaneous vaginal delivery outside the hospital. On examination, fine, white scales were observed predominantly on the extensor surfaces of the limbs along with characteristic hyperlinearity of palms and accentuation of skin markings. Which of the following genes is most likely defective in the above condition:
What is the primary clinical feature of Henoch-Schonlein purpura?
Fourteen weeks pregnancy with third degree prolapse. Best management will be:
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 Tzanck smear from a patient with bullous lesions shows which of the following findings?
Pemphigoid Gestationis 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: Acantholytic cells in pemphigus are derived from:
- A. Stratum spinosum (Correct Answer)
- B. Stratum basale
- C. Stratum granulosum
- D. Langerhans cells
Explanation: Acantholytic cells in pemphigus are keratinocytes that have lost their desmosomal attachments due to autoimmune destruction, primarily affecting the integrity of the stratum spinosum [1]. This layer is characterized by abundant desmosomes, and their disruption leads to the characteristic intraepidermal blistering seen in pemphigus [2]. While pemphigus can affect the basal layer in some forms (like pemphigus vegetans), the primary acantholytic process occurs above this layer, leading to suprabasal cleavage [1]. The stratum granulosum lies above the stratum spinosum and contains keratohyalin granules and lamellar granules, which are essential for skin barrier function. While cells from all epidermal layers can eventually slough off, acantholysis itself primarily originates from the more adhesive cells of the stratum spinosum. Langerhans cells are dendritic antigen-presenting cells found predominantly in the stratum spinosum but are not keratinocytes. While they play a role in immune surveillance, they are not the cells that undergo acantholysis or form the bulk of the epidermal layers [3]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Skin, pp. 1170-1172. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Disorders Involving Inflammatory And Haemopoietic Cells, pp. 645-646. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Skin, p. 1144.
Question 3: Which of the following statements is true regarding pemphigus vulgaris?
- A. It primarily affects the dermal-epidermal junction.
- B. It is a subepidermal blistering disease.
- C. It is an intraepidermal blistering disease. (Correct Answer)
- D. Antibodies are formed against basement membrane proteins.
Explanation: ***It is an intraepidermal blistering disease.*** - **Pemphigus vulgaris** is characterized by the formation of blisters *within the epidermis* due to the loss of cell-to-cell adhesion between **keratinocytes**. - This **acantholysis** results from autoantibodies targeting **desmosomes**, specifically **desmoglein 1 and 3**. *It is a subepidermal blistering disease.* - This statement is incorrect as it describes conditions like **bullous pemphigoid** or **dermatitis herpetiformis**, where blisters form *below* the epidermis. - In pemphigus vulgaris, the separation occurs *above* the basement membrane within the epidermal layer. *It primarily affects the dermal-epidermal junction.* - This is characteristic of **bullous pemphigoid** or **epidermolysis bullosa acquisita**, where the primary pathology involves the separation at the interface between the dermis and epidermis. - Pemphigus vulgaris directly affects the adhesion *within* the epidermis itself. *Antibodies are formed against basement membrane proteins.* - This is characteristic of **bullous pemphigoid**, where autoantibodies target components of the **basement membrane zone**, such as **BP180** and **BP230**. - In pemphigus vulgaris, the autoantibodies target **desmogleins** on the surface of keratinocytes, not basement membrane proteins.
Question 4: 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 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: A 5-month-old child presented to the dermatology OPD with dryness along with white, fine scales on most parts of the body with sparing of face. The child was born at 39 weeks gestation by spontaneous vaginal delivery outside the hospital. On examination, fine, white scales were observed predominantly on the extensor surfaces of the limbs along with characteristic hyperlinearity of palms and accentuation of skin markings. Which of the following genes is most likely defective in the above condition:
- A. Filaggrin (Correct Answer)
- B. Desmoglein
- C. Plakophilin
- D. Plakoglobin
Explanation: ***Filaggrin*** - The clinical description of **fine, white scales**, **facial sparing**, **extensor surface involvement**, and **palmar hyperlinearity** is classic for **ichthyosis vulgaris**, the most common inherited disorder of keratinization. - **Filaggrin (FLG)** gene mutations are the most common cause of ichthyosis vulgaris, leading to impaired formation of the skin barrier and loss of natural moisturizing factor. - Typically presents between 3-12 months of age with xerosis and fine scaling. - Key features include **facial sparing**, **flexural sparing**, and **prominent palmar/plantar markings**. *Desmoglein* - **Desmoglein 1** and **Desmoglein 3** are desmosomal cadherins associated with **pemphigus** (autoimmune blistering disorder). - Desmoglein mutations do not cause ichthyotic scaling disorders. - The clinical presentation of fine scaling with facial sparing is not consistent with pemphigus or other desmoglein-related conditions. *Plakophilin* - **Plakophilin-1** mutations cause **ectodermal dysplasia-skin fragility syndrome**, characterized by skin erosions, blistering, alopecia, and nail dystrophy. - Does not present with generalized dry scaling or the pattern described in this case. - Skin fragility and erosions are the hallmark, not ichthyosis. *Plakoglobin* - **Plakoglobin (JUP gene)** mutations cause **Naxos disease**, featuring the triad of arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. - While palmoplantar involvement occurs, it is a focal keratoderma, not generalized fine scaling with facial sparing. - Cardiac manifestations are the most serious feature of this condition.
Question 7: What is the primary clinical feature of Henoch-Schonlein purpura?
- A. Abdominal pain due to vasculitis
- B. Joint pain associated with the condition
- C. Kidney involvement in the disease
- D. Skin rash characterized by palpable purpura (Correct Answer)
Explanation: ***Skin rash characterized by palpable purpura*** - **Palpable purpura** is the hallmark cutaneous manifestation of **Henoch-Schonlein purpura (HSP)**, a small-vessel vasculitis [1]. - This rash typically appears on the **lower extremities and buttocks**, reflecting the deposition of IgA in vessel walls [1]. *Abdominal pain due to vasculitis* - While **abdominal pain** is a common feature of HSP due to gastrointestinal vasculitis, it is not considered the primary clinical feature [1]. - Gastrointestinal involvement can manifest with pain, bleeding, and intussusception, but the **skin rash** is more consistently present and diagnostic. *Joint pain associated with the condition* - **Arthralgia** or **arthritis** (joint pain) is seen in a significant number of HSP patients, particularly in the knees and ankles. - However, it is a secondary manifestation, and not the **defining primary sign** of the disease. *Kidney involvement in the disease* - **Renal involvement**, presenting as hematuria and proteinuria, occurs in about one-third of HSP cases and can lead to serious long-term complications. - Despite its significance for prognosis, **kidney disease** is a later and not universally present feature, making the rash the most critical initial diagnostic clue.
Question 8: Fourteen weeks pregnancy with third degree prolapse. Best management will be:
- A. No treatment
- B. Foot end elevation
- C. Ring pessary (Correct Answer)
- D. Sling surgery
Explanation: ***Ring pessary*** - A **ring pessary** is the most appropriate management for a **third-degree uterine prolapse** during pregnancy, especially in the second trimester (14 weeks). - It provides **mechanical support** to the uterus, relieving symptoms and potentially allowing the pregnancy to progress without surgical intervention. *No treatment* - Leaving a **third-degree prolapse untreated** during pregnancy can lead to complications such as cervical erosion, infection, miscarriage, or preterm labor, making it an unsuitable option. - The patient would experience significant discomfort and potential obstruction, which needs active management. *Foot end elevation* - While **foot end elevation** can temporarily relieve some pelvic pressure, it is not an effective or sufficient treatment for a **third-degree uterine prolapse**, which requires direct mechanical support. - It does not address the underlying anatomical displacement and would not sustainedly reduce the prolapse. *Sling surgery* - **Sling surgery** is a surgical procedure typically indicated for severe, symptomatic uterine prolapse in non-pregnant individuals or after childbirth, not during pregnancy. - Performing surgery during the **second trimester** carries significant risks to both the mother and the fetus, including potential for miscarriage or preterm labor.
Question 9: 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 10: A Tzanck smear from a patient with bullous lesions shows which of the following findings?
- A. Langerhans cells are seen
- B. Acantholysis (Correct Answer)
- C. Leucocytosis
- D. Absence of melanin pigment
Explanation: **Explanation:** The **Tzanck smear** is a rapid bedside diagnostic test used in dermatology to examine cells from the base of a blister. The hallmark finding in autoimmune bullous diseases like **Pemphigus Vulgaris** is **Acantholysis**. **Why Acantholysis is correct:** Acantholysis refers to the loss of intercellular connections (desmosomes) between keratinocytes. In Pemphigus, autoantibodies attack desmogleins, causing the epidermal cells to detach from one another and become rounded. On a Tzanck smear, these are seen as **Tzanck cells**: large, round, nucleated keratinocytes with a peripheral rim of condensed cytoplasm (mourning-edged appearance). **Why other options are incorrect:** * **Langerhans cells:** These are antigen-presenting cells found in the stratum spinosum. While present in the skin, they are not the diagnostic feature of a Tzanck smear for bullous lesions. * **Leucocytosis:** This refers to an elevated white blood cell count in the blood, which is a systemic finding and cannot be determined via a localized skin smear. * **Absence of melanin pigment:** This is characteristic of vitiligo or albinism, not primary blistering disorders. **NEET-PG High-Yield Pearls:** 1. **Tzanck Smear Indications:** Used primarily for **Herpes Simplex/Varicella** (shows multinucleated giant cells) and **Pemphigus Vulgaris** (shows acantholytic cells). 2. **Stains used:** Giemsa, Wright’s, or Leishman stain. 3. **Bullous Pemphigoid:** Unlike Pemphigus, the Tzanck smear in Bullous Pemphigoid typically shows numerous **eosinophils** but *no* acantholysis, as the split is subepidermal. 4. **Hailey-Hailey Disease:** Also shows significant acantholysis on Tzanck smear (often described as a "dilapidated brick wall" on histology).
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