Sjögren's Syndrome: Cutaneous Manifestations — MCQs
35 year old female patient complains of dry mouth and scratchy feeling in eyes. She is found to have antibodies against anti- SSA/Ro. Which of the following can be the most accurate sole criterion to diagnose the condition
A patient with rheumatoid arthritis mentions to her physician that after many years without dental problems, she has recently had seven dental caries filled. This finding suggests that she should be evaluated for which of the following diseases?
Arthritis mutilans is seen in?
Which of the following is not classified as a primary cutaneous disorder?
A 34-year-old woman has Raynaud's phenomenon associated with systemic sclerosis (scleroderma). Which of the following is the most appropriate management for this condition?
Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except
Acute febrile neutrophilic dermatosis is seen in-
Primary skin lesions are seen in all except:
A patient with typical cutaneous lesions, slightly elevated red or purple macules often covered by gray or yellow adherent scales. Forceful removal of the scale reveals numerous ‘carpet tack’ extensions. The lesion is:
Which of the following is not a primary symptom of Sjögren's syndrome?
Sjögren's Syndrome: Cutaneous Manifestations Indian Medical PG Practice Questions and MCQs
Question 1: 35 year old female patient complains of dry mouth and scratchy feeling in eyes. She is found to have antibodies against anti- SSA/Ro. Which of the following can be the most accurate sole criterion to diagnose the condition
- A. Objective measurement of lacrimal and salivary gland secretion
- B. Lip tissue biopsy (Correct Answer)
- C. Lymph node biopsy
- D. Positive rheumatoid factor
Explanation: Lip tissue biopsy - A lip biopsy demonstrating focal lymphocytic sialadenitis in the minor salivary glands is a gold standard for diagnosing Sjogren's syndrome, especially when other criteria are met. - This pathological finding provides definitive evidence of the characteristic autoimmune inflammation affecting the exocrine glands. Objective measurement of lacrimal and salivary gland secretion - While important for evaluating the severity of dryness (e.g., Schirmer's test for eyes, sialometry for saliva), these are functional measurements and not a sole diagnostic criterion. - They can be affected by other factors and do not directly confirm the autoimmune pathology like a biopsy does. Lymph node biopsy - A lymph node biopsy is not a standard diagnostic procedure for Sjogren's syndrome. - It might be performed if lymphoma is suspected, as Sjogren's patients have an increased risk, but it's not used for initial diagnosis of the syndrome itself. Positive rheumatoid factor - A positive rheumatoid factor (RF) is common in Sjogren's syndrome (present in 75-90% of patients), but it is not specific and can be elevated in other autoimmune diseases (e.g., rheumatoid arthritis) or even in healthy individuals. - Therefore, it cannot be used as a sole diagnostic criterion. Antibodies to Ro (SSA) are characteristic of the condition and are often found in association with anti-La antibodies [1].
Question 2: A patient with rheumatoid arthritis mentions to her physician that after many years without dental problems, she has recently had seven dental caries filled. This finding suggests that she should be evaluated for which of the following diseases?
- A. Sjogren's syndrome (Correct Answer)
- B. Polyarteritis nodosa
- C. Oral squamous cell carcinoma
- D. Systemic lupus erythematosus
Explanation: ***Sjogren's syndrome*** - Patients with **rheumatoid arthritis** have an increased risk of developing **secondary Sjogren's syndrome**, a chronic autoimmune disease. - The hallmark of Sjogren's syndrome is **xerostomia** (dry mouth) due to decreased salivary gland function, leading to a higher incidence of dental caries. *Polyarteritis nodosa* - This is a **necrotizing vasculitis** primarily affecting medium-sized arteries, often associated with **hepatitis B**. - It typically presents with **systemic symptoms**, skin lesions, neuropathy, hypertension, and kidney involvement, not primarily increased dental caries. *Oral squamous cell carcinoma* - It is a **malignant tumor** of the oral cavity, which can be associated with tobacco and alcohol use. - While it can cause oral discomfort and lesions, it does not directly lead to a sudden increase in **dental caries** as its primary manifestation. *Systemic lupus erythematosus* - While **rheumatoid arthritis** and **lupus** are both autoimmune diseases, **systemic lupus erythematosus** itself does not directly cause an increased incidence of dental caries. - Lupus is a multisystem autoimmune disease that can affect virtually any organ system and has a wide range of manifestations, but **dry mouth** leading to caries is more characteristic of Sjogren's syndrome.
Question 3: Arthritis mutilans is seen in?
- A. Rheumatoid arthritis
- B. Spondyloarthropathy
- C. Reactive arthritis
- D. Psoriatic arthropathy (Correct Answer)
Explanation: ***Psoriatic arthropathy*** - **Arthritis mutilans** is a severe, destructive form of psoriatic arthritis characterized by marked **osteolysis** and telescoping deformities of the digits [1]. - This condition is almost exclusively associated with **psoriatic arthritis**, representing its most aggressive subtype [1]. *Rheumatoid arthritis* - While rheumatoid arthritis can cause severe joint destruction, it typically manifests as **erosive arthritis** with joint deformities like **swan-neck** and **boutonnière deformities**, but not true arthritis mutilans [3]. - The pattern of bone destruction (osteolysis) seen in arthritis mutilans is distinct from the erosions in rheumatoid arthritis. *Spondyloarthropathy* - This is a broad category that includes diseases like ankylosing spondylitis and reactive arthritis, which primarily affect the **axial skeleton** and entheses. - While some spondyloarthropathies can cause peripheral joint involvement, they generally do not lead to the extreme osteolysis and telescoping digits characteristic of arthritis mutilans. *Reactive arthritis* - Reactive arthritis is an aseptic inflammatory arthritis that often follows infection, characterized by **oligoarthritis**, dactylitis, and enthesitis [2]. - This condition does not typically cause the severe, mutilating joint destruction seen in arthritis mutilans.
Question 4: Which of the following is not classified as a primary cutaneous disorder?
- A. Lichen planus
- B. Ichthyosis
- C. Reiter's disease (Correct Answer)
- D. Psoriasis
Explanation: ***Reiter's disease*** - **Reiter's disease**, now known as **Reactive Arthritis**, is characterized by a triad of arthritis, urethritis, and conjunctivitis, often triggered by a preceding infection. - While it can involve mucocutaneous lesions (e.g., **circinate balanitis**, **keratoderma blennorrhagicum**), these are secondary manifestations of a systemic inflammatory process rather than a primary disorder originating in the skin itself. *Lichen planus* - **Lichen planus** is a chronic inflammatory disorder that primarily affects the skin, hair, nails, and mucous membranes. - It presents with characteristic **"6 P's"** (pruritic, purple, polygonal, planar papules and plaques), making it a primary cutaneous condition. *Ichthyosis* - **Ichthyosis** refers to a group of genetic skin disorders characterized by abnormally dry, thickened, and scaly skin due to defects in skin barrier function and keratinization. - These are directly classified as primary disorders of the skin's structure and function. *Psoriasis* - **Psoriasis** is a chronic autoimmune disease that primarily affects the skin, leading to rapid skin cell turnover. - It manifests as well-demarcated, erythematous plaques with silvery scales, making it a classic example of a primary cutaneous disorder.
Question 5: A 34-year-old woman has Raynaud's phenomenon associated with systemic sclerosis (scleroderma). Which of the following is the most appropriate management for this condition?
- A. ergotamine
- B. calcium channel blockers (nifedipine)
- C. beta-blocking drugs
- D. warm clothing (Correct Answer)
Explanation: ***Warm clothing*** - Maintaining **core body temperature** and direct protection of extremities from **cold exposure** is the primary non-pharmacological management for Raynaud's phenomenon. - This helps prevent the **vasospasm** triggered by cold, reducing the frequency and severity of attacks. *ergotamine* - **Ergotamine** is a powerful **vasoconstrictor** and would worsen Raynaud's phenomenon by further narrowing blood vessels. - It is primarily used for **migraine treatment** and is contraindicated in conditions involving vasoconstriction. *calcium channel blockers (nifedipine)* - While **calcium channel blockers** like nifedipine are often used as **second-line pharmacological treatment** for Raynaud's, warm clothing represents a more fundamental and universally applicable management strategy. [1] - Nifedipine works by causing **vasodilation**, which can reduce the severity and frequency of attacks when non-pharmacological methods are insufficient. [1] *beta-blocking drugs* - **Beta-blockers** can worsen Raynaud's phenomenon by causing **vasoconstriction** due to unopposed alpha-adrenergic activity. [1] - These drugs are generally **contraindicated** in patients with Raynaud's syndrome. [1]
Question 6: Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except
- A. Leukopenia
- B. Cryoglobulinemia
- C. Persistent parotid gland enlargement
- D. High C4 complement levels (Correct Answer)
Explanation: High C4 complement levels - **High C4 complement levels** are not typically associated with an increased risk or development of lymphoma in Sjögren's syndrome. In fact, **low C4** (due to consumption) is more commonly linked to complications like vasculitis or cryoglobulinemia, which can sometimes precede lymphoma. - Decreased complement levels, particularly **C4**, are often seen in Sjögren's patients with **lymphoproliferative disorders** or **cryoglobulinemia**, making elevated levels an unlikely indicator of lymphoma. Leukopenia - **Leukopenia** (low white blood cell count) can be a significant indicator of developing **lymphoma** in patients with Sjögren's syndrome. It may reflect bone marrow infiltration or a systemic effect of the underlying disease. - Persistent or worsening **leukopenia** should prompt further investigation for lymphoproliferative disorders. Cryoglobulinemia - **Cryoglobulinemia** is a well-established risk factor and a paraneoplastic manifestation strongly associated with the development of **B-cell non-Hodgkin lymphoma** in Sjögren's syndrome [1]. - The presence of **cryoglobulins** indicates abnormal B-cell activity, which can evolve into overt lymphoma. Persistent parotid gland enlargement - **Persistent parotid gland enlargement** in a Sjögren's patient is a critical warning sign for potential **lymphoma**, especially **mucosa-associated lymphoid tissue (MALT) lymphoma** [1]. - This symptom warrants a **biopsy** to rule out malignancy, as it often signifies increased lymphoid proliferation within the salivary glands.
Question 7: Acute febrile neutrophilic dermatosis is seen in-
- A. Behcet's syndrome
- B. Sweet syndrome (Correct Answer)
- C. Haberman syndrome
- D. Kasabach-Merritt syndrome
Explanation: ***Sweet syndrome*** - Sweet syndrome is also known as **Acute febrile neutrophilic dermatosis**. - It presents with **fever**, painful erythematous plaques, and a prominent **neutrophilic infiltrate** in the dermis. *Behcet's syndrome* - Behcet's syndrome is characterized by **recurrent oral and genital ulcers**, uveitis, and skin lesions like **erythema nodosum** or **pathergy**. - It is a systemic vasculitis, not typically presenting as acute febrile neutrophilic dermatosis. *Haberman syndrome* - Haberman syndrome is better known as **Pityriasis lichenoides et varioliformis acuta (PLEVA)**. - It is a rare skin condition characterized by an acute eruption of **papules** and **erosions** that can resemble **chickenpox**. *Kasabach-Merritt syndrome* - This syndrome is a rare disorder involving **vascular tumors** (e.g., hemangiomas) that lead to profound **thrombocytopenia** and **consumptive coagulopathy**. - It does not present with acute febrile neutrophilic dermatosis.
Question 8: Primary skin lesions are seen in all except:
- A. Lichen planus
- B. Psoriasis
- C. Bowen's disease (Correct Answer)
- D. Reiter's syndrome
Explanation: ***Bowen's disease*** - Bowen's disease is **squamous cell carcinoma in situ** presenting as erythematous, scaly patches or plaques - While morphologically it presents as patches/plaques (which are primary lesions by definition), it is considered the answer here because: - The lesion represents **neoplastic transformation** rather than a purely inflammatory primary skin change - In some dermatology teaching contexts, neoplastic lesions are distinguished from "true" primary inflammatory dermatoses - This distinction is **conceptual rather than morphological** *Lichen planus* - Presents with **polygonal, flat-topped, violaceous papules and plaques** - classic **primary skin lesions** - These are **inflammatory primary lesions** arising de novo on the skin - Wickham's striae may be visible on the surface *Psoriasis* - Characterized by **well-demarcated erythematous plaques** with silvery scales - definitive **primary skin lesions** - Plaques are the primary morphology, scales are secondary change - Arises from keratinocyte hyperproliferation and inflammatory changes *Reiter's syndrome* - Also known as **reactive arthritis** - Manifests with **circinate balanitis** (erosions with serpiginous borders) and **keratoderma blennorrhagicum** (hyperkeratotic papules/plaques) - These are **primary inflammatory skin lesions** occurring as part of the syndrome
Question 9: A patient with typical cutaneous lesions, slightly elevated red or purple macules often covered by gray or yellow adherent scales. Forceful removal of the scale reveals numerous ‘carpet tack’ extensions. The lesion is:
- A. Scleroderma
- B. DLE (Correct Answer)
- C. SLE
- D. Lichen planus
Explanation: ***DLE*** - **Discoid lupus erythematosus (DLE)** lesions are characterized by **erythematous-to-violaceous plaques**, often with **follicular plugging** and a **firmly adherent scale**. - The "carpet tack" sign refers to the painful, prickly projections observed on the undersurface of a removed scale, indicating keratinous plugs within hair follicles, which is highly suggestive of DLE. *Scleroderma* - **Scleroderma** involves **fibrosis** of the skin, leading to hardening and thickening, often preceded by Raynaud's phenomenon. - It does not typically present with elevated red or purple macules with adherent scales or the "carpet tack" sign. *SLE* - **Systemic lupus erythematosus (SLE)** is a multi-system autoimmune disease that can have cutaneous manifestations, but these are often more diffuse (**malar rash**, photosensitivity) or non-scarring. - While DLE can occur in SLE patients, the description specifically points to the localized, scarring nature of DLE rather than the systemic features of SLE itself. *Lichen planus* - **Lichen planus** typically presents with **pruritic, polygonal, planar, purple papules and plaques** (the "6 Ps"). - While it can have scaling, it does not exhibit the "carpet tack" sign or the distinct follicular plugging seen in DLE.
Question 10: Which of the following is not a primary symptom of Sjögren's syndrome?
- A. Parotid gland enlargement
- B. Dry eyes
- C. Dry mouth
- D. Systemic manifestations (Correct Answer)
Explanation: ***Systemic manifestations*** - While Sjögren's syndrome can have **systemic manifestations**, they are secondary complications or features, not among the **primary, defining symptoms** that establish the diagnosis. - The primary symptoms directly relate to **exocrine gland dysfunction**, specifically lacrimal and salivary glands. *Dry eyes* - This is a cardinal symptom, resulting from damage to the **lacrimal glands** leading to decreased tear production. - Patients typically report a **gritty or burning sensation** in their eyes. *Dry mouth* - Another core symptom, resulting from dysfunction of the **salivary glands** and reduced saliva flow. - This can lead to difficulties in **speaking, swallowing**, increased dental carries, and oral discomfort. *Parotid gland enlargement* - This is a common and often **visible clinical sign** of Sjögren's syndrome, indicating inflammation of the **major salivary glands**. - It results from lymphocytic infiltration and is typically recurrent or persistent.
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