Scleroderma and Morphea — MCQs
A 35-year-old female presents with skin thickening and muscle weakness. Her peripheries became pale on exposure to cold. Her ANA is positive and creatine kinase is increased. Scl-70 is positive and perifascicular infiltration is noted in biopsy. What is the antibody associated with this condition?
What is the screening test for scleroderma?
The skin biopsy shown below is most consistent with which of the following conditions? 
C.R.E.S.T syndrome is?
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?
Identify the lesion:
A 28-year-old female presents with a 3-month history of violaceous, flat-topped papules on her wrists and lower back associated with intense pruritus. Examination reveals white lacy streaks on the buccal mucosa bilaterally. She was recently started on antihypertensive medication 4 months ago. Histopathology shows hyperkeratosis, irregular acanthosis, and band-like lymphocytic infiltrate at the dermoepidermal junction. What is the most appropriate next step in management?
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?
Scleroderma and Morphea Indian Medical PG Practice Questions and MCQs
Question 1: A 35-year-old female presents with skin thickening and muscle weakness. Her peripheries became pale on exposure to cold. Her ANA is positive and creatine kinase is increased. Scl-70 is positive and perifascicular infiltration is noted in biopsy. What is the antibody associated with this condition?
- A. Anti Jo1 antibody
- B. Anti centromere antibody
- C. Antinuclear antibody
- D. Anti PM scl antibody (Correct Answer)
Explanation: ***Anti PM scl antibody*** - The constellation of **skin thickening** [2], **muscle weakness** [1], **Raynaud's phenomenon** (pale peripheries on cold exposure) [3], **elevated creatine kinase**, and **perifascicular infiltration** on muscle biopsy indicates an overlap syndrome between **systemic sclerosis** and **polymyositis/dermatomyositis** [3]. - **Anti-PM/Scl antibodies** are highly specific markers for this **overlap syndrome**, explaining the mixed features of scleroderma and myositis. *Anti Jo1 antibody* - This antibody is primarily associated with **polymyositis** or **dermatomyositis**, especially with the **anti-synthetase syndrome**, which includes features like **interstitial lung disease**, **Raynaud's**, and **arthritis** [1]. - While muscle weakness is present, the prominent skin thickening and positive Scl-70 point away from isolated myositis. *Anti centromere antibody* - This antibody is characteristic of **limited cutaneous systemic sclerosis (CREST syndrome)**, which involves **calcinosis**, **Raynaud's phenomenon**, esophageal dysmotility, sclerodactyly, and telangiectasias. - While Raynaud's is present, the widespread skin thickening, muscle weakness, and perifascicular infiltration are not typical of limited cutaneous systemic sclerosis. *Antinuclear antibody* - A **positive antinuclear antibody (ANA)** is a general screening test for **autoimmune diseases** and is present in a wide range of conditions, including systemic sclerosis, lupus, and myositis [4]. - While ANA is positive in this patient, it is not specific enough to diagnose the exact overlap syndrome with its unique clinical and laboratory findings.
Question 2: What is the screening test for scleroderma?
- A. Anti-nuclear antibody (Correct Answer)
- B. U1-Ribonucleoprotein antibody
- C. Anti-LKM antibody
- D. Anti-topoisomerase antibody
Explanation: ***Anti-nuclear antibody*** - **ANA** is the most sensitive **screening test** for systemic autoimmune diseases, including scleroderma [1]. - A positive ANA test indicates the presence of autoantibodies, leading to further, more specific testing for scleroderma. *U1-Ribonucleoprotein antibody* - This antibody is primarily associated with **mixed connective tissue disease (MCTD)**, not scleroderma as a screening test [1]. - It can be positive in other autoimmune diseases, but it is not the primary screening test for scleroderma. *Anti-LKM antibody* - **Anti-liver kidney microsomal (LKM) antibody** is predominantly found in **autoimmune hepatitis type 2**. - It has no role in the screening or diagnosis of scleroderma. *Anti-topoisomerase antibody* - This antibody, also known as **anti-Scl-70**, is **highly specific for diffuse systemic sclerosis** (scleroderma). - While specific, it is used as a confirmatory test once scleroderma is suspected, rather than a general screening test.
Question 3: The skin biopsy shown below is most consistent with which of the following conditions? 
- A. Lichen planus (Correct Answer)
- B. Lichen nitidus
- C. Morphea
- D. Lupus erythematosus
Explanation: ***Lichen planus*** - The image shows **basal cell degeneration** (liquefaction degeneration), a **sawtooth rete ridge pattern**, and a band-like inflammatory infiltrate primarily composed of lymphocytes at the dermo-epidermal junction, which are classic histological features of **lichen planus**. - **Civatte bodies** (apoptotic keratinocytes forming colloid bodies) are typically present, resulting from keratinocyte damage at the basal layer. - These features make lichen planus the most consistent diagnosis. *Lichen nitidus* - Characterized by **"ball and claw" lesions**, which are small, localized epidermal invaginations enclosing a central infiltrate of lymphocytes and histiocytes. - The granulomatous infiltrate is more focal and circumscribed compared to the band-like pattern of lichen planus. - While both are interface dermatitides, the architectural pattern differs significantly. *Morphea* - This is a localized form of **scleroderma**, characterized by increased **collagen deposition**, thickening of the dermis, and loss of adnexal structures like hair follicles and sweat glands. - The inflammatory infiltrate is typically perivascular and interstitial, not band-like at the dermo-epidermal junction. - The image does not show features of dermal fibrosis or homogenization of collagen bundles expected in morphea. *Lupus erythematosus* - Also shows **interface dermatitis** with basal vacuolar changes and lymphocytic infiltrate. - However, lupus typically shows a **perivascular and periappendageal pattern** of infiltrate rather than the dense band-like pattern of lichen planus. - Additional features in lupus include dermal mucin deposition, thickened basement membrane (PAS-positive), and follicular plugging. - The dense, continuous band-like infiltrate and sawtooth rete ridges favor lichen planus over lupus.
Question 4: C.R.E.S.T syndrome is?
- A. Localised scleroderma
- B. Generalised scleroderma
- C. Limited scleroderma (Correct Answer)
- D. Systemic sclerosis
Explanation: ***Limited scleroderma*** - CREST syndrome is an acronym for **Calcinosis**, **Raynaud's phenomenon**, **Esophageal dysmotility**, **Sclerodactyly**, and **Telangiectasias**, which are the hallmark features of **limited cutaneous systemic sclerosis**. - Its clinical presentation is distinguished by skin thickening that affects only the **distal extremities** (below the elbows and knees) and the face. - This is the **most specific answer** as CREST syndrome is synonymous with limited scleroderma. *Localised scleroderma* - This typically presents as **morphea** or **linear scleroderma**, which are benign forms of scleroderma largely confined to the skin without systemic involvement. - It does not involve the characteristic internal organ manifestations or the full spectrum of features seen in CREST syndrome. *Generalised scleroderma* - This term is often synonymous with **diffuse cutaneous systemic sclerosis**, a more severe form where skin thickening extends to the trunk and proximal extremities. - It involves a higher risk of early and severe **internal organ involvement**, differentiating it from the more benign course of limited scleroderma. *Systemic sclerosis* - While CREST syndrome is technically a subtype of systemic sclerosis (specifically the limited cutaneous form), this option is **too broad** and does not specifically define what CREST syndrome represents. - Systemic sclerosis encompasses both **limited (CREST)** and **diffuse forms**, which have different prognoses and patterns of organ involvement. - "Limited scleroderma" is the more **precise and preferred answer** for CREST syndrome.
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: Identify the lesion:
- A. Psoriasis
- B. Dermatitis herpetiformis
- C. Erythema marginatum
- D. Dermatomyositis (Correct Answer)
Explanation: ***Dermatomyositis*** - The image shows **Gottron's papules** over the extensor surfaces of the elbows, which are characteristic of dermatomyositis. These are violaceous, erythematous, flat-topped papules. - While typically found on the **dorsum of the hands** over the MCP and IP joints, they can also occur on elbows, knees, and ankles. *Psoriasis* - Psoriasis typically presents with **well-demarcated erythematous plaques** covered with silvery scales, especially on extensor surfaces. - The lesions in the image lack the characteristic **silvery scaling** of psoriasis. *Dermatitis herpetiformis* - This condition presents with intensely **itchy, polymorphic lesions**, including vesicles, bullae, and excoriations, arranged in a symmetrical fashion, often on extensor surfaces. - The lesions in the image are papular and nodular, not exhibiting the characteristic **vesicular or bullous eruption** of dermatitis herpetiformis. *Erythema marginatum* - Erythema marginatum is a **transient, non-pruritic erythematous rash** with serpiginous borders and central clearing, typically seen in **acute rheumatic fever**. - The lesions in the image are fixed papules/nodules without the characteristic migrating or rapidly changing appearance of erythema marginatum.
Question 7: A 28-year-old female presents with a 3-month history of violaceous, flat-topped papules on her wrists and lower back associated with intense pruritus. Examination reveals white lacy streaks on the buccal mucosa bilaterally. She was recently started on antihypertensive medication 4 months ago. Histopathology shows hyperkeratosis, irregular acanthosis, and band-like lymphocytic infiltrate at the dermoepidermal junction. What is the most appropriate next step in management?
- A. Start oral prednisolone 1 mg/kg and continue current antihypertensive
- B. Discontinue current antihypertensive and start topical corticosteroids (Correct Answer)
- C. Start oral methotrexate and continue current antihypertensive
- D. Perform direct immunofluorescence and continue current medication
Explanation: ***Discontinue current antihypertensive and start topical corticosteroids*** - The patient presents with **Lichen Planus (LP)**, which in this case is likely a **drug-induced lichenoid eruption** given the temporal link to starting **antihypertensive medication** (e.g., ACE inhibitors, beta-blockers, or diuretics). - The first-line management involves removing the offending agent and using **topical corticosteroids** to reduce inflammation and pruritus. *Start oral prednisolone 1 mg/kg and continue current antihypertensive* - **Systemic steroids** are generally reserved for severe or generalized cases and are not the first-line treatment if a causative drug is still present. - Continuing the offending **antihypertensive** would likely cause the eruption to persist or recur despite steroid therapy. *Start oral methotrexate and continue current antihypertensive* - **Methotrexate** is a second-line immunosuppressant used only for **recalcitrant or hypertrophic LP**, not for initial management of a suspected drug-induced case. - Using such a potent systemic drug without first addressing the **causative medication** would be inappropriate and increase the risk of toxicity. *Perform direct immunofluorescence and continue current medication* - **Direct immunofluorescence (DIF)** may show **cytoid bodies** and granular IgM deposits, but the diagnosis of LP is already confirmed by the characteristic **classic histology** (band-like infiltrate, saw-tooth rete ridges). - Delaying the withdrawal of the suspected drug to perform an unnecessary diagnostic test would delay resolution of the **itchy violaceous papules**.
Question 8: 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 9: 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 10: 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.
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