Autoimmune Skin Diseases — MCQs

A patient presents with a pruritic lesion over the left shoulder exhibiting cigarette paper atrophy and poikiloderma, along with generalized lymphadenopathy. Histopathological examination of the lesion reveals CD4-positive Sézary-Lutzner cells. What is the characteristic dermo-epidermal manifestation of this disease?

Q49Medium

These skin changes developed during treatment for end-stage renal failure. What is the diagnosis?

Q50Medium

A 23-year-old male complains of recurrent scaly lesions on the glans penis. The lesions always occurred at the same site and healed with slight hyperpigmentation. What is the most likely diagnosis?

Autoimmune Skin Diseases Indian Medical PG Practice Questions and MCQs

Question 41: Localized scleroderma is also known as?

A. Shagreen patch
B. Morphea (Correct Answer)
C. Heliotrope erythema
D. Gottron's papule

Explanation: **Explanation:** **Localized Scleroderma (Morphea)** is a chronic autoimmune connective tissue disorder characterized by excessive collagen deposition, leading to thickening and hardening of the skin. Unlike systemic sclerosis, morphea typically lacks internal organ involvement, Raynaud’s phenomenon, or sclerodactyly. It clinically presents as asymptomatic, circumscribed, ivory-colored sclerotic plaques with a characteristic "lilac border" during the active inflammatory phase. **Analysis of Incorrect Options:** * **A. Shagreen patch:** This is a connective tissue nevus (collagenoma) found on the lower back. It is a major diagnostic criterion for **Tuberous Sclerosis**, not scleroderma. * **C. Heliotrope erythema:** This refers to a violaceous eruption on the upper eyelids, often associated with periorbital edema. It is a pathognomonic feature of **Dermatomyositis**. * **D. Gottron’s papule:** These are lichenoid, violaceous papules found over the bony prominences of the hands (MCP, PIP, and DIP joints). They are also a hallmark sign of **Dermatomyositis**. **High-Yield Clinical Pearls for NEET-PG:** * **Histopathology:** Shows "squared-off" biopsy specimens due to dense collagen bundles replacing the subcutaneous fat and loss of skin appendages (eccrine glands/hair follicles). * **Linear Morphea:** A subtype that can follow Blaschko’s lines. When it occurs on the forehead/scalp, it is called **"En coup de sabre"** (resembling a sword cut). * **Parry-Romberg Syndrome:** Progressive hemifacial atrophy that can be associated with linear morphea. * **Treatment:** Potent topical steroids or calcineurin inhibitors for limited disease; UVA1 phototherapy or systemic methotrexate for generalized/disabling cases.

Question 42: Gottron's papules are characteristically found on which of the following locations?

A. Face
B. Trunk
C. Spine
D. Dorsal aspects of the interphalangeal joints of the fingers (Correct Answer)

Explanation: **Explanation:** **Gottron’s papules** are considered a pathognomonic cutaneous feature of **Dermatomyositis**. These are erythematous to violaceous, flat-topped, lichenoid papules and plaques. The underlying medical concept involves an autoimmune inflammatory process targeting the skin and muscles. They characteristically occur over **pressure points and bony prominences**, specifically the **dorsal aspects of the metacarpophalangeal (MCP) and interphalangeal (IP) joints**. This distribution is a key diagnostic criterion (Option D). **Analysis of Incorrect Options:** * **A. Face:** While Dermatomyositis presents with facial features like the **Heliotrope rash** (periorbital violaceous edema) and the **Shawl sign** (erythema on the upper back/neck), Gottron’s papules are specifically localized to the hands. * **B. Trunk:** Erythema on the trunk is seen in the **V-sign** (anterior chest) or **Shawl sign**, but these are confluent macules/patches, not the discrete papules described by Gottron. * **C. Spine:** This is not a characteristic site for Dermatomyositis lesions. **NEET-PG High-Yield Pearls:** * **Gottron’s Sign:** Symmetrical violaceous erythema (without papules) over the elbows, knees, or medial malleoli. * **Mechanic’s Hands:** Hyperkeratosis, scaling, and fissuring on the lateral and palmar aspects of the fingers (associated with **Anti-Jo-1** antibodies and interstitial lung disease). * **Holster Sign:** Poikiloderma on the lateral aspect of the thighs. * **Malignancy:** Dermatomyositis in adults is frequently associated with internal malignancies (e.g., ovarian, lung, breast).

Question 43: Which of the following is NOT true about Behçet's disease?

A. It shows presence of aphthous ulceration, genital ulceration, and uveitis.
B. Uveitis is bilateral, acute recurrent iridocyclitis with hypopyon.
C. It has a good visual prognosis. (Correct Answer)
D. Chlorambucil can be used to control the disease.

Explanation: **Explanation:** **Behçet’s Disease** is a chronic, multisystem, relapsing inflammatory perivasculitis. The correct answer is **C** because Behçet’s disease typically carries a **poor visual prognosis**. Ocular involvement occurs in about 70% of patients and is a leading cause of morbidity; persistent inflammation leads to complications like secondary glaucoma, cataract, and retinal vasculitis, often resulting in blindness if not treated aggressively. **Analysis of Options:** * **Option A:** This describes the classic **"Triple Symptom Complex"** (Oral ulcers, Genital ulcers, and Uveitis). Recurrent aphthous stomatitis is the most common and usually the first presenting sign. * **Option B:** Ocular involvement is classically a **bilateral panuveitis**. A hallmark finding is **acute recurrent iridocyclitis with a "shifting" hypopyon** (sterile pus in the anterior chamber that moves with head position). * **Option D:** Immunosuppressants are the mainstay for systemic or sight-threatening disease. **Chlorambucil**, Cyclosporine, and Azathioprine are used to control severe ocular and neurological manifestations. **High-Yield Clinical Pearls for NEET-PG:** * **Pathergy Test:** A highly specific diagnostic test where a sterile needle prick results in a papule or pustule within 24–48 hours. * **HLA Association:** Strongly associated with **HLA-B51**. * **Major Criteria:** Recurrent oral ulceration (mandatory for diagnosis) plus any two of: recurrent genital ulcers, eye lesions, skin lesions (erythema nodosum/pseudofolliculitis), or a positive pathergy test. * **Most common cause of death:** Rupture of large vessel aneurysms (e.g., pulmonary artery).

Question 44: A butterfly rash characteristically spares which of the following facial areas?

A. Cheeks
B. Nasolabial fold (Correct Answer)
C. Lower eyelids
D. Bridge of nose

Explanation: **Explanation:** The **butterfly rash** (malar rash) is the classic cutaneous manifestation of **Systemic Lupus Erythematosus (SLE)**. It is characterized by an erythematous, sometimes edematous, eruption over the malar eminence. **1. Why Nasolabial Folds are Spared:** The hallmark feature that distinguishes the butterfly rash of SLE from other facial rashes (like seborrheic dermatitis or rosacea) is the **sparing of the nasolabial folds**. This occurs because the rash is primarily **photosensitive**; the deep anatomical contour of the nasolabial fold is relatively shielded from direct UV radiation, preventing the inflammatory cascade in that specific area. **2. Analysis of Incorrect Options:** * **Cheeks (A):** These are the primary site of the rash (malar distribution). * **Lower eyelids (C):** While the rash can approach the eyes, it typically involves the malar area just below the lids; however, the nasolabial fold is the *characteristic* spared site mentioned in textbooks. * **Bridge of nose (D):** The rash characteristically connects across the bridge of the nose, completing the "butterfly" shape. **High-Yield Clinical Pearls for NEET-PG:** * **Differential Diagnosis:** In **Dermatomyositis**, the rash (Heliotrope rash) involves the eyelids and **does not** spare the nasolabial folds. In **Rosacea**, there are often associated pustules and telangiectasia, which are absent in SLE. * **Systemic Association:** A malar rash is one of the 11 ACR criteria for diagnosing SLE. * **Histopathology:** SLE shows vacuolar degeneration of the basal layer and a "lupus band" (IgG/C3 deposits) at the dermo-epidermal junction.

Question 45: "En coup de sabre" is seen in:

A. Syphilis
B. Leprosy
C. Scleroderma (Correct Answer)
D. Scabies

Explanation: **Explanation:** **En coup de sabre** is a classic clinical presentation of **Linear Scleroderma**, a subtype of localized scleroderma (morphea). The term is French for "cut of the sword," describing a linear, indented scar-like lesion that typically occurs on the forehead or scalp. 1. **Why Scleroderma is correct:** In linear scleroderma, there is localized inflammation followed by fibrosis and atrophy of the skin, subcutaneous tissue, and sometimes the underlying muscle and bone. When this occurs on the frontoparietal region, it creates a sunken, ivory-colored furrow that mimics a sword wound. It can be associated with hemi-facial atrophy (Parry-Romberg Syndrome). 2. **Why other options are incorrect:** * **Syphilis:** Presents with features like chancre (primary), condyloma lata, or maculopapular rashes (secondary), and gummas (tertiary), but not linear atrophy. * **Leprosy:** Characterized by hypopigmented patches with loss of sensation or thickened nerves, not linear fibrotic furrows. * **Scabies:** A parasitic infestation presenting with intense nocturnal pruritus and "burrows" (short, wavy lines), primarily in finger webs and flexures. **High-Yield Clinical Pearls for NEET-PG:** * **Morphea:** Localized scleroderma that lacks systemic involvement (no sclerodactyly or Raynaud’s). * **Parry-Romberg Syndrome:** Progressive facial hemi-atrophy often seen in conjunction with *en coup de sabre*. * **Histology:** Look for "squared-off" biopsy specimens due to dense collagen deposition and loss of periappecular fat. * **Treatment:** Potent topical steroids or calcineurin inhibitors for limited disease; methotrexate for deep or progressive lesions.

Question 46: What is true about Dermatomyositis?

A. Associated with HLA B27
B. Bimodal age distribution (Correct Answer)
C. No involvement of joints
D. Myopathy precedes skin lesions in most cases

Explanation: **Explanation:** **Dermatomyositis (DM)** is an idiopathic inflammatory myopathy characterized by distinctive cutaneous findings and proximal muscle weakness. **1. Why Option B is Correct:** Dermatomyositis exhibits a classic **bimodal age distribution**. It primarily affects two distinct groups: * **Juvenile DM:** Typically occurs between ages 5 and 15 years. * **Adult DM:** Typically occurs between ages 40 and 60 years. **2. Why Other Options are Incorrect:** * **Option A:** DM is associated with **HLA-DR3, DR52, and DRw53**, not HLA-B27 (which is linked to Seronegative Spondyloarthropathies like Ankylosing Spondylitis). * **Option C:** Joint involvement is common. Patients often present with **non-erosive polyarthritis or arthralgia**, typically affecting small joints of the hands in a symmetrical pattern similar to Rheumatoid Arthritis. * **Option D:** In approximately **60% of cases, skin lesions precede or occur simultaneously** with muscle weakness. When skin lesions occur without muscle involvement for $\geq$ 6 months, it is termed "Amyopathic Dermatomyositis." **3. NEET-PG High-Yield Pearls:** * **Pathognomonic Signs:** **Gottron papules** (violaceous papules over bony prominences/knuckles) and **Heliotrope rash** (periorbital violaceous erythema with edema). * **Other Signs:** Shawl sign (V-neck distribution), Holster sign (lateral thigh), and Mechanic’s hands. * **Malignancy:** Adult-onset DM is strongly associated with internal malignancies (Ovarian, Lung, Gastric). Screening is mandatory. * **Antibodies:** **Anti-Mi2** (specific for DM, good prognosis), **Anti-Jo1** (associated with Antisynthetase syndrome/interstitial lung disease). * **Histopathology:** Interface dermatitis (similar to SLE) and perifascicular atrophy on muscle biopsy.

Question 47: Exposure to sunlight can precipitate which of the following conditions?

A. Chloasma
B. Discoid lupus erythematosus (Correct Answer)
C. Dermatitis herpetiformis
D. Lupus vulgaris

Explanation: **Explanation:** **Discoid Lupus Erythematosus (DLE)** is a chronic cutaneous form of Lupus Erythematosus characterized by photosensitivity. Exposure to **Ultraviolet (UV) radiation** (specifically UVB) triggers keratinocyte apoptosis and the release of nuclear antigens. In genetically predisposed individuals, this leads to an inflammatory response, resulting in the classic erythematous, scaly plaques with follicular plugging and scarring alopecia. Photosensitivity is a hallmark feature of most Lupus variants. **Analysis of Incorrect Options:** * **Chloasma (Melasma):** While sunlight *exacerbates* or darkens existing melasma due to melanocyte stimulation, it is primarily a hormonal condition (pregnancy, OCPs) rather than a disease "precipitated" or caused by sunlight in the same pathological sense as DLE. * **Dermatitis Herpetiformis (DH):** This is an autoimmune blistering disease associated with **Gluten-sensitive enteropathy**. It is triggered by gluten ingestion, not UV light. Lesions typically appear on extensor surfaces. * **Lupus Vulgaris:** Despite the name "Lupus," this is a chronic progressive form of **Cutaneous Tuberculosis**. It is caused by *Mycobacterium tuberculosis* in individuals with moderate to high immunity and is unrelated to sunlight exposure. **High-Yield Clinical Pearls for NEET-PG:** * **DLE Triad:** Erythema, Adherent Scaling (Carpet tack sign/Tin tack sign), and Atrophy. * **Photosensitive Dermatoses (The "S" Rule):** **S**LE/DLE, **S**olar urticaria, **S**unburn, **S**teven-Johnson Syndrome (rarely), and **S**un-induced exacerbation of Darier’s disease. * **Drug-induced Photosensitivity:** Common culprits include Thiazides, Tetracyclines (Doxycycline), and Sulfonamides.

Question 48: A patient presents with a pruritic lesion over the left shoulder exhibiting cigarette paper atrophy and poikiloderma, along with generalized lymphadenopathy. Histopathological examination of the lesion reveals CD4-positive Sézary-Lutzner cells. What is the characteristic dermo-epidermal manifestation of this disease?

A. Pautrier's microabscess (Correct Answer)
B. Pinpoint ulcers
C. Discharging sinus
D. Miliaria

Explanation: ### Explanation The clinical presentation of pruritic lesions with **cigarette paper atrophy**, **poikiloderma**, and generalized lymphadenopathy, combined with the presence of **CD4-positive Sézary-Lutzner cells** (cerebriform nuclei), is diagnostic of **Mycosis Fungoides (MF)**, the most common type of Cutaneous T-Cell Lymphoma (CTCL). #### Why Option A is Correct The hallmark histopathological feature of Mycosis Fungoides is **epidermotropism**—the migration of atypical T-lymphocytes into the epidermis without significant spongiosis. When these malignant T-cells (Sézary-Lutzner cells) aggregate into small clusters within the epidermis, they form **Pautrier’s microabscesses**. This is a highly specific diagnostic finding for MF. #### Why Other Options are Incorrect * **B. Pinpoint ulcers:** These are more characteristic of conditions like Ecthyma or certain vasculitides, not a primary feature of CTCL. * **C. Discharging sinus:** This suggests deep fungal infections (like Actinomycosis) or Scrofuloderma (cutaneous TB), which present with chronic inflammatory tracts rather than epidermal atrophy. * **D. Miliaria:** This is a common sweat gland disorder (prickly heat) caused by the occlusion of eccrine ducts, unrelated to lymphocytic malignancies. #### High-Yield Clinical Pearls for NEET-PG * **Stages of MF:** It progresses through three stages: **Patch** (cigarette paper atrophy/poikiloderma) → **Plaque** → **Tumor**. * **Sézary Syndrome:** The leukemic triad of MF consisting of erythroderma, generalized lymphadenopathy, and >1000/mm³ Sézary cells in peripheral blood. * **Immunophenotype:** Malignant cells are typically **CD3+ and CD4+** (T-helper cells). * **Treatment:** Early-stage MF is treated with topical steroids, PUVA, or Narrowband UVB; advanced stages may require systemic chemotherapy or electron beam therapy.

Question 49: These skin changes developed during treatment for end-stage renal failure. What is the diagnosis?

A. Amyloidosis
B. Nephrogenic fibrosing dermopathy (Correct Answer)
C. Syphilis
D. Scleroderma

Explanation: ***Nephrogenic fibrosing dermopathy*** - Occurs specifically in patients with **end-stage renal disease** following exposure to **gadolinium-based contrast agents** used in MRI. - Characterized by **woody thickening** of skin, **hyperpigmentation**, and **fibrosis** predominantly affecting the extremities and trunk. *Amyloidosis* - Presents with **waxy papules** and **periorbital purpura**, not the woody skin thickening seen in NFD. - While associated with chronic kidney disease, skin changes are typically **translucent plaques** around eyes and flexural areas. *Syphilis* - Secondary syphilis causes a **copper-colored rash** on palms and soles with **condyloma lata**. - No association with **renal failure** or **gadolinium exposure**, and would be accompanied by systemic symptoms. *Scleroderma* - Features **Raynaud's phenomenon**, **sclerodactyly**, and **telangiectasias** starting distally on fingers. - Skin changes begin with **puffy fingers** progressing to **tight, shiny skin**, unlike the woody texture of NFD.

Question 50: A 23-year-old male complains of recurrent scaly lesions on the glans penis. The lesions always occurred at the same site and healed with slight hyperpigmentation. What is the most likely diagnosis?

A. Fixed drug eruption (Correct Answer)
B. Herpes
C. Candida balanoposthitis
D. Behcet's disease

Explanation: ### Explanation **Correct Answer: A. Fixed Drug Eruption (FDE)** The clinical hallmark of a **Fixed Drug Eruption (FDE)** is the recurrence of a lesion at the **exact same anatomical site** upon re-exposure to the offending drug. The glans penis is the most common site for FDE in males. These lesions typically present as well-demarcated, dusky red or violaceous plaques that may blister and characteristically heal with **residual slate-grey hyperpigmentation** due to pigmentary incontinence (melanin dropping into the dermis). Common triggers include NSAIDs (like paracetamol), sulfonamides, and tetracyclines. **Why the other options are incorrect:** * **B. Herpes Simplex (Genital):** While recurrent and occurring at similar sites, herpes typically presents as clusters of painful, fluid-filled vesicles on an erythematous base. It heals without significant hyperpigmentation and is often preceded by a prodrome of tingling or burning. * **C. Candida Balanoposthitis:** This presents as diffuse erythema, white curd-like discharge, and satellite pustules. It is usually associated with poor hygiene or diabetes and does not strictly recur at the "same spot" with post-inflammatory hyperpigmentation. * **D. Behcet’s Disease:** This involves painful, deep, "punched-out" scrotal or penile ulcers as part of a systemic triad (including oral ulcers and uveitis). These ulcers heal with scarring rather than simple hyperpigmentation. **High-Yield Clinical Pearls for NEET-PG:** * **Most common site (Overall):** Extremities. * **Most common site (Genitalia):** Glans penis. * **Most common drug cause (India):** Cotrimoxazole and NSAIDs. * **Pathology:** Shows "Interface Dermatitis" with necrotic keratinocytes (Civatte bodies) and dermal melanophages. * **Refractory Period:** After a reaction, there is a brief period where re-exposure does not cause a flare at that site.

Practice by Chapter

Lupus Erythematosus: Cutaneous Forms

Practice Questions

Lupus Erythematosus: Systemic with Skin Manifestations

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Dermatomyositis

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Scleroderma and Morphea

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Mixed Connective Tissue Disease

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Sjögren's Syndrome: Cutaneous Manifestations

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Relapsing Polychondritis

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Autoimmune Thyroid Disease and the Skin

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Immunobullous Disorders

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Vasculitis

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Diagnostic Methods in Autoimmune Dermatoses

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Management of Autoimmune Skin Diseases

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