Relapsing Polychondritis — MCQs
A 12 year old girl was brought with fever, malaise, and migrating polyarthritis. She had a history of recurrent throat infections in the past. Elevated erythrocyte sedimentation rate is noted. Which among the following is NOT a major Jones criteria for diagnosis of acute rheumatic fever?
Osteoarthritis is associated with all of the following except -
A child presents with high grade fever, inspiratory stridor and develops swallowing difficulty with drooling of saliva since last 4-6 hours. Which of the following treatment is recommended apart from general airway management?
Which of the following conditions is least likely to present with polyarticular involvement in clinical practice?
The mode of inheritance of Incontinentia pigmenti is:
A study of persons developing skin lesions following sun exposure is conducted. The lesions are not found on skin protected from ultraviolet light. Biopsies of involved skin show immunoglobulin G deposition along the dermal-epidermal junction, along with vacuolization of the basal layer and a perivascular lymphocytic infiltrate. No other organ involvement is present. Which of the following diseases do these patients most likely have?
Morphea most commonly occurs in which location?
Gottron's papules are a characteristic clinical finding in which of the following conditions?
Which of the following is a common association with Celiac sprue?
Which of the following is NOT a feature of Netherton Syndrome?
Relapsing Polychondritis Indian Medical PG Practice Questions and MCQs
Question 1: A 12 year old girl was brought with fever, malaise, and migrating polyarthritis. She had a history of recurrent throat infections in the past. Elevated erythrocyte sedimentation rate is noted. Which among the following is NOT a major Jones criteria for diagnosis of acute rheumatic fever?
- A. Raised ESR (Correct Answer)
- B. Chorea
- C. Arthritis
- D. Carditis
Explanation: ***Raised ESR*** - **Elevated erythrocyte sedimentation rate (ESR)** is a **minor criterion** in the Jones Criteria for acute rheumatic fever, indicating inflammation but not specific enough to be a major criterion. - While it supports the diagnosis, it is a non-specific inflammatory marker rather than a distinct clinical manifestation of the disease. *Chorea* - **Sydenham's chorea** (St. Vitus' dance) is a **major manifestation** of acute rheumatic fever, characterized by involuntary, purposeless movements. - It results from central nervous system involvement and is a highly diagnostic sign, often appearing late in the disease course. *Arthritis* - **Migratory polyarthritis** is a **major criterion** for acute rheumatic fever, typically affecting large joints in a sequential pattern. - This symptom is often the presenting complaint and is highly responsive to anti-inflammatory treatment. *Carditis* - **Carditis**, involving inflammation of the heart muscle, pericardium, or endocardium, is a **major criterion** and the most serious manifestation of acute rheumatic fever. - It can lead to long-term valvular damage, particularly affecting the mitral and aortic valves.
Question 2: Osteoarthritis is associated with all of the following except -
- A. Subchondral sclerosis
- B. Ca++deposition in joint space (Correct Answer)
- C. Osteophyte formation
- D. Decreased joint space
Explanation: ***Ca++deposition in joint space*** - **Calcium pyrophosphate dihydrate (CPPD) crystal deposition** in the joint space is characteristic of **pseudogout**, not osteoarthritis [1]. - While some **calcification** may occur in osteophytes, direct **calcium crystal deposition** in the synovial fluid is not a primary feature of osteoarthritis [1]. *Subchondral sclerosis* - **Subchondral sclerosis** refers to the increased bone density that occurs beneath the cartilage in areas of stress in osteoarthritis. - This is a common radiological finding in osteoarthritis, reflecting the bone's response to increased mechanical load. *Osteophyte formation* - **Osteophytes** (bone spurs) are bony projections that form along the joint margins in osteoarthritis [2]. - They are a hallmark feature of the disease, resulting from the body's attempt to repair and stabilize the damaged joint [2]. *Decreased joint space* - **Decreased joint space** on radiographs is a classic sign of osteoarthritis, indicating loss of articular cartilage thickness [2]. - As the cartilage erodes, the distance between the bones within the joint decreases. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 683-684. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1210-1212.
Question 3: A child presents with high grade fever, inspiratory stridor and develops swallowing difficulty with drooling of saliva since last 4-6 hours. Which of the following treatment is recommended apart from general airway management?
- A. IV ceftriaxone (Correct Answer)
- B. Anti-diphtheria toxin
- C. Corticosteroids
- D. Nebulized racemic epinephrine
Explanation: ***IV ceftriaxone*** - The symptoms (high-grade fever, inspiratory stridor, swallowing difficulty with drooling, rapid onset) are highly suggestive of **acute epiglottitis**, a life-threatening emergency. - **Empiric intravenous antibiotics** like ceftriaxone are crucial for treating the bacterial infection (commonly *Haemophilus influenzae* type b or *Streptococcus pneumoniae*) causing epiglottitis. *Anti-diphtheria toxin* - This treatment is specific for **diphtheria**, which causes a pseudomembrane and can lead to airway obstruction, but the clinical picture here is more consistent with epiglottitis due to its rapid and severe presentation without mention of a pseudomembrane. - Diphtheria typically has a more gradual onset and is characterized by a **grayish pseudomembrane** in the throat, unmentioned in this case. *Corticosteroids* - While corticosteroids are used in other forms of upper airway obstruction (like **croup**), their role in acute epiglottitis is controversial and not a primary life-saving measure; antibiotics and airway management are paramount. - Their primary benefit lies in reducing inflammation, but they do not address the acute bacterial cause of epiglottitis directly and are secondary to antibiotics. *Nebulized racemic epinephrine* - This treatment is primarily used for **laryngotracheobronchitis (croup)**, which presents with a barking cough and stridor, but typically lacks the high fever and severe drooling seen in epiglottitis. - Nebulized racemic epinephrine helps to reduce subglottic edema in croup but would not be effective against the severe supraglottic swelling of epiglottitis, nor would it treat the underlying bacterial infection.
Question 4: Which of the following conditions is least likely to present with polyarticular involvement in clinical practice?
- A. Psoriatic arthritis
- B. SLE
- C. Gout
- D. Ankylosing spondylitis (Correct Answer)
Explanation: ***SLE*** - Systemic lupus erythematosus (SLE) is a **systemic autoimmune disease** and while it can present with polyarthritis, other systemic features also occur, making it less common in isolation [1]. - It encompasses a broad spectrum of **clinical manifestations**, often leading to multi-organ involvement beyond joint symptoms [1]. *Gout* - Usually triggers **acute inflammatory arthritis**, characterized by sudden and severe pain in a single joint, particularly the big toe [2]. - It is significantly common and frequently treated in clinical practice. *Ankylosing spondylitis* - Primarily affects the spine and sacroiliac joints, causing stiffness and is quite an **identifiable form of inflammatory arthritis** [3]. - It has a notable association with **HLA-B27** and can present with back pain, making it relatively common among spondyloarthropathies [3]. *Psoriatic arthritis* - Associated with **psoriasis**, this form of arthritis can occur and is known for **asymmetrical polyarthritis** and dactylitis [4]. - It is a recognized inflammatory condition that leads to joint destruction and is increasingly prevalent among patients with skin involvement [4].
Question 5: The mode of inheritance of Incontinentia pigmenti is:
- A. Autosomal dominant
- B. Autosomal recessive
- C. X-linked dominant (Correct Answer)
- D. X-linked recessive
Explanation: **Explanation:** **Incontinentia Pigmenti (Bloch-Sulzberger syndrome)** is a rare multisystem neurocutaneous disorder caused by a mutation in the **IKBKG gene** (formerly NEMO). 1. **Why X-linked Dominant is correct:** The inheritance is **X-linked dominant**. The mutation is typically **lethal in males** in utero, which is why the clinical phenotype is seen almost exclusively in females. Affected females survive due to functional mosaicism resulting from **X-chromosome inactivation (Lyonization)**. 2. **Why other options are wrong:** * **Autosomal Dominant/Recessive:** The gene is located on the X chromosome (Xq28), ruling out autosomal inheritance. * **X-linked Recessive:** In recessive conditions, heterozygous females are usually asymptomatic carriers. In IP, a single mutated allele is sufficient to cause the disease phenotype in females. **Clinical Phases (High-Yield for NEET-PG):** The skin lesions characteristically follow the **Lines of Blaschko** and evolve through four distinct stages: 1. **Vesicular stage:** Linear vesicles (present at birth or shortly after). 2. **Verrucous stage:** Hyperkeratotic, wart-like plaques. 3. **Hyperpigmented stage:** "Swirl-like" or "Marble cake" pigmentation (due to melanin incontinence into the dermis). 4. **Hypopigmented/Atrophic stage:** Linear streaks of hypopigmentation and hair loss. **Clinical Pearls:** * **Associated findings:** Peg-shaped (conical) teeth, delayed dentition, seizures, and cicatricial alopecia. * **Histology:** Eosinophilic spongiosis is a characteristic feature of the first stage. * **Key differentiator:** Unlike other X-linked dominant conditions, the male-to-female ratio is heavily skewed due to male lethality.
Question 6: A study of persons developing skin lesions following sun exposure is conducted. The lesions are not found on skin protected from ultraviolet light. Biopsies of involved skin show immunoglobulin G deposition along the dermal-epidermal junction, along with vacuolization of the basal layer and a perivascular lymphocytic infiltrate. No other organ involvement is present. Which of the following diseases do these patients most likely have?
- A. Bullous pemphigoid
- B. Celiac disease
- C. Discoid lupus erythematosus (Correct Answer)
- D. Dysplastic nevus syndrome
Explanation: ### Explanation The clinical presentation and histopathology described are classic for **Discoid Lupus Erythematosus (DLE)**, a form of Chronic Cutaneous Lupus Erythematosus (CCLE). **1. Why Discoid Lupus Erythematosus (DLE) is correct:** * **Photosensitivity:** DLE lesions are typically triggered or exacerbated by UV light and are confined to sun-exposed areas (face, scalp, ears). * **Histopathology:** The "vacuolization of the basal layer" (interface dermatitis) and "perivascular lymphocytic infiltrate" are hallmark features. * **Direct Immunofluorescence (DIF):** The "Lupus Band Test" shows a granular deposition of IgG and C3 along the dermal-epidermal junction (DEJ). In DLE, this is positive **only in involved (lesional) skin**, consistent with the question stating no other organ involvement (systemic lupus would often show deposition in uninvolved skin as well). **2. Why the other options are incorrect:** * **Bullous Pemphigoid:** While it shows IgG at the DEJ, it presents with subepidermal blisters and a linear (not granular) pattern. It is not typically induced by sun exposure. * **Celiac Disease:** This is associated with Dermatitis Herpetiformis, which presents with itchy vesicles on elbows/knees and shows **IgA** (not IgG) deposition in the dermal papillae. * **Dysplastic Nevus Syndrome:** This involves pigmented melanocytic lesions with architectural atypia, not an autoimmune inflammatory process with IgG deposition. **High-Yield Clinical Pearls for NEET-PG:** * **Lupus Band Test:** Positive in lesional skin in DLE; positive in both lesional and non-lesional skin in SLE. * **DLE Triad:** Erythema, adherent scales (carpet tack sign/follicular plugging), and atrophic scarring. * **Progression:** Only about 5-10% of patients with DLE progress to Systemic Lupus Erythematosus (SLE). * **Treatment:** Sun protection is the first step; topical corticosteroids or antimalarials (Hydroxychloroquine) are first-line medical therapies.
Question 7: Morphea most commonly occurs in which location?
- A. Forehead (Correct Answer)
- B. Sternum
- C. Limbs
- D. Back
Explanation: **Explanation:** **Morphea**, also known as localized scleroderma, is characterized by excessive collagen deposition leading to thickening and hardening of the skin. Unlike systemic sclerosis, it typically lacks internal organ involvement or Raynaud’s phenomenon. **Correct Option: A. Forehead** While morphea can occur anywhere on the body, the **forehead** is the most characteristic and classic site for the linear subtype of morphea, specifically known as **"En coup de sabre"** (resembling a stroke from a sword). This presentation involves a linear induration and atrophy on the forehead and scalp, often associated with alopecia and, occasionally, underlying hemi-facial atrophy (Parry-Romberg syndrome). In the context of standard medical examinations like NEET-PG, the forehead is recognized as the most frequently tested and clinically significant site for localized linear morphea. **Incorrect Options:** * **B. Sternum:** While morphea can occur on the trunk, the sternum is not the most common site. The trunk is more frequently involved in "Generalized Morphea," but the forehead remains the classic site for localized linear variants. * **C. Limbs:** Linear morphea can affect the extremities, potentially leading to joint contractures or limb-length discrepancies, but it is statistically less "classic" for board-style questions than the forehead presentation. * **D. Back:** Plaque-type morphea often appears on the trunk (including the back), but it is not the most common or characteristic site compared to the forehead in clinical vignettes. **Clinical Pearls for NEET-PG:** * **En coup de sabre:** Linear morphea on the forehead/scalp. * **Histology:** Shows "squared-off" biopsy specimens, thickened collagen bundles, and loss of adnexal structures (eccrine glands/hair follicles). * **Treatment:** First-line therapy typically involves topical corticosteroids or calcineurin inhibitors; methotrexate or UVA1 phototherapy is used for deeper or progressive disease.
Question 8: Gottron's papules are a characteristic clinical finding in which of the following conditions?
- A. Dermatomyositis (Correct Answer)
- B. Sarcoidosis
- C. Scleroderma
- D. Fungal infection
Explanation: **Explanation:** **Dermatomyositis** is the correct answer. Gottron’s papules are considered a **pathognomonic** cutaneous feature of this idiopathic inflammatory myopathy. They are characterized by erythematous to violaceous, flat-topped papules and plaques found symmetrically over the dorsal aspects of the interphalangeal (IP) and metacarpophalangeal (MCP) joints. Pathologically, they represent interface dermatitis similar to lupus but are specific to the bony prominences of the hands. **Why other options are incorrect:** * **Sarcoidosis:** Typically presents with "apple-jelly" nodules, lupus pernio (violaceous plaques on the nose/cheeks), or erythema nodosum. It does not feature Gottron’s papules. * **Scleroderma:** Characterized by skin tightening (sclerodactyly), Raynaud’s phenomenon, and "beaked nose" appearance. While it affects the hands, the pathology involves fibrosis rather than inflammatory papules over joints. * **Fungal infection:** Dermatophytosis (Tinea) usually presents as annular erythematous plaques with central clearing and peripheral scaling, not localized papules over small joints. **NEET-PG High-Yield Pearls:** 1. **Gottron’s Sign:** Symmetrical violaceous erythema (macular) over the knuckles, elbows, or knees (distinguish from *papules*). 2. **Heliotrope Rash:** Violaceous edema of the upper eyelids; another pathognomonic sign. 3. **Shawl Sign & V-Sign:** Photosensitive erythema over the upper back and chest, respectively. 4. **Mechanic’s Hands:** Hyperkeratosis and fissuring of the palms and lateral fingers (associated with **Anti-Jo-1** antibodies and interstitial lung disease). 5. **Malignancy:** Dermatomyositis in adults is frequently associated with internal malignancies (paraneoplastic syndrome).
Question 9: Which of the following is a common association with Celiac sprue?
- A. Herpes Gestationalis
- B. Dermatitis herpetiformis (Correct Answer)
- C. Pemphigus vulgaris
- D. Bullous pemphigoid
Explanation: **Explanation:** **Dermatitis Herpetiformis (DH)** is considered the cutaneous manifestation of gluten-sensitive enteropathy (**Celiac sprue**). The underlying pathophysiology involves IgA antibodies directed against **tissue transglutaminase (tTG)** in the gut, which cross-react with **epidermal transglutaminase (eTG)** in the skin. This leads to the characteristic subepidermal blisters and intense pruritus. Nearly 90% of patients with DH have associated gluten-sensitive enteropathy, though many remain asymptomatic for gastrointestinal symptoms. **Analysis of Incorrect Options:** * **A. Herpes Gestationalis (Pemphigoid Gestationis):** This is a pregnancy-associated autoimmune bullous disease caused by IgG antibodies against BP180. It has no association with gluten sensitivity. * **C. Pemphigus Vulgaris:** An intraepidermal blistering disease caused by antibodies against Desmoglein 1 and 3. It is associated with other autoimmune conditions (like Myasthenia Gravis) but not Celiac sprue. * **D. Bullous Pemphigoid:** A subepidermal blistering disease seen primarily in the elderly, targeting BP180 and BP230. It is not linked to gluten-sensitive enteropathy. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Presentation:** Symmetrical, extremely pruritic vesicles on extensors (elbows, knees, buttocks). * **Histopathology:** Neutrophilic microabscesses at the tips of dermal papillae. * **Direct Immunofluorescence (DIF):** **Granular IgA deposits** in the dermal papillae (Gold Standard). * **Treatment:** **Dapsone** is the drug of choice for skin lesions, while a **Gluten-free diet** is essential for long-term management and reducing the risk of intestinal lymphoma (EATL).
Question 10: Which of the following is NOT a feature of Netherton Syndrome?
- A. Autosomal dominant inheritance (Correct Answer)
- B. Ichthyosis
- C. Very short hair
- D. Erythroderma
Explanation: **Explanation:** Netherton Syndrome is a rare, severe multisystem disorder characterized by a classic triad of **ichthyosis linearis circumflexa**, **hair shaft abnormalities**, and **atopic features**. **1. Why Option A is the correct answer:** Netherton Syndrome is inherited in an **Autosomal Recessive** pattern, not autosomal dominant. It is caused by a mutation in the **SPINK5 gene** on chromosome 5q32, which encodes the serine protease inhibitor **LEKTI**. The deficiency of LEKTI leads to overactive epidermal proteases, resulting in premature desquamation and a severely compromised skin barrier. **2. Why the other options are incorrect:** * **B. Ichthyosis:** Patients typically present with **Ichthyosis Linearis Circumflexa (ILC)**, characterized by migratory, erythematous, polycyclic plaques with a unique "double-edged" scale. * **C. Very short hair:** This refers to **Trichorrhexis invaginata** (Bamboo hair), the pathognomonic hair defect. The hair is brittle and breaks easily, leading to short, sparse hair and eyebrows. * **D. Erythroderma:** Most neonates with Netherton Syndrome present at birth with generalized **congenital ichthyosiform erythroderma**, which can lead to life-threatening complications like dehydration and hypernatremia. **High-Yield Clinical Pearls for NEET-PG:** * **Pathognomonic Hair Sign:** "Bamboo hair" (intussusception of the distal hair shaft into the proximal portion). * **The Triad:** Ichthyosis + Trichorrhexis invaginata + Atopy (high IgE levels/asthma). * **Differential Diagnosis:** Often misdiagnosed as Atopic Dermatitis or Omenn Syndrome in infancy. * **Key Gene:** SPINK5 (LEKTI protein).
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