Autoimmune Skin Diseases — MCQs
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A 2-year-old child, born out of a consanguineous marriage, presented with a history of collodion membrane. Physical examination revealed large, thick, plate-like brown scaling with generalized distribution, but no erythroderma or ectropion. Which of the following is the most likely diagnosis?
What is the term used to refer to hidebound disease?
Gottron's sign is characteristic of which condition?
What is the percentage of patients with disseminated discoid lupus erythematosus who develop systemic lupus erythematosus?
Granular deposit of IgA at the dermoepidermal junction is seen in which of the following conditions?
IgA deposits are seen on a skin biopsy in which of the following conditions?
A 45-year-old patient presents with itchy, flat-topped, polygonal, violaceous papules on the inner wrists and flexors surfaces of the forearms. The lesions have a characteristic shiny surface and are arranged in a linear pattern. The patient denies any recent medication changes. What is the treatment?
A patient presented with itchy lesions, as shown below. What is the diagnosis?
A patient presents with a violaceous rash on the upper back and shoulders (img-16.jpeg) and Gottron's papules on hands (img-17.jpeg). What is the likely diagnosis?
A woman presents with pruritic rash on the elbows, buttocks with recent diagnosis of gluten sensitive enteropathy. On immunofluorescence IgA deposition is seen as shown in the image. What is the most likely diagnosis?
Autoimmune Skin Diseases Indian Medical PG Practice Questions and MCQs
Question 71: A 2-year-old child, born out of a consanguineous marriage, presented with a history of collodion membrane. Physical examination revealed large, thick, plate-like brown scaling with generalized distribution, but no erythroderma or ectropion. Which of the following is the most likely diagnosis?
- A. Ichthyosis vulgaris
- B. Lamellar ichthyosis (Correct Answer)
- C. X-linked ichthyosis
- D. Netherton syndrome
Explanation: **Explanation:** The clinical presentation of a **collodion membrane** at birth followed by the development of **large, thick, plate-like brown scales** in a generalized distribution is classic for **Lamellar Ichthyosis (LI)**. 1. **Why Lamellar Ichthyosis is correct:** LI is an autosomal recessive disorder (often associated with consanguinity) caused primarily by a mutation in the **TGM1 gene** (Transglutaminase-1). It typically presents at birth as a collodion baby. As the membrane sheds, it leaves behind large, dark, quadrilateral scales that are arranged in a "mosaic" or "plate-like" pattern. While ectropion is common in LI, its absence does not rule out the diagnosis, especially when compared to the specific scaling patterns of other options. 2. **Why other options are incorrect:** * **Ichthyosis vulgaris:** The most common type, but it **never** presents as a collodion membrane. It appears later in childhood with fine, white scales, sparing the flexures, and is associated with hyperlinear palms. * **X-linked ichthyosis:** Caused by **Steroid Sulfatase deficiency**. It presents with "dirty" brown scales (comma-shaped) on the neck and trunk, but it is not associated with a collodion membrane at birth. * **Netherton syndrome:** A triad of Ichthyosis linearis circumflexa, bamboo hair (trichorrhexis invaginata), and atopy. It typically presents with migratory, erythematous, polycyclic plaques with double-edged scales, not thick brown plates. **High-Yield Clinical Pearls for NEET-PG:** * **Collodion Membrane:** Seen in Lamellar Ichthyosis and Non-bullous Congenital Ichthyosiform Erythroderma (NBCIE). * **TGM1 Mutation:** Most common cause of Lamellar Ichthyosis; leads to defective cornified cell envelope formation. * **Steroid Sulfatase Deficiency:** Key feature of X-linked Ichthyosis; associated with **undescended testes** and **delayed labor** (due to low placental estrogen). * **Hyperlinear palms/Atopy:** Strongly associated with Ichthyosis Vulgaris (Filaggrin mutation).
Question 72: What is the term used to refer to hidebound disease?
- A. Discoid Lupus Erythematosus (DLE)
- B. Scleroderma (Correct Answer)
- C. Acrodermatitis enteropathica
- D. None of the above
Explanation: **Explanation:** **Scleroderma** (Systemic Sclerosis) is referred to as **"Hidebound disease"** because of the characteristic progressive fibrosis and thickening of the skin. The term "hidebound" literally describes skin that is so tight and bound to the underlying subcutaneous structures that it cannot be pinched or moved. This occurs due to excessive collagen deposition, leading to the loss of skin elasticity. **Analysis of Options:** * **Scleroderma (Correct):** In addition to hidebound skin, clinical features include **Microstomia** (fish-mouth appearance), **Sclerodactyly** (tapering of fingers), and **Raynaud’s phenomenon** (often the earliest sign). * **Discoid Lupus Erythematosus (DLE):** This is a chronic cutaneous form of Lupus characterized by well-defined erythematous scaly plaques, follicular plugging, and scarring alopecia. It does not cause the generalized "hidebound" tightening seen in scleroderma. * **Acrodermatitis Enteropathica:** This is an autosomal recessive disorder caused by **Zinc deficiency**. It presents with a triad of periorificial/acral dermatitis, alopecia, and diarrhea, but does not involve skin fibrosis. **High-Yield Clinical Pearls for NEET-PG:** * **Salt and Pepper Pigmentation:** A classic sign of Scleroderma characterized by vitiligo-like depigmentation with sparing of the perifollicular areas. * **CREST Syndrome:** A limited form of systemic sclerosis (Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia). * **Antibody Markers:** **Anti-Scl-70** (Topoisomerase I) is specific for Diffuse Systemic Sclerosis, while **Anti-Centromere** antibody is specific for Limited Scleroderma (CREST). * **En coup de sabre:** A linear form of localized scleroderma (morphea) occurring on the scalp or forehead.
Question 73: Gottron's sign is characteristic of which condition?
- A. Lupus erythematosus
- B. Dermatomyositis (Correct Answer)
- C. Scleroderma
- D. Bell's palsy
Explanation: **Explanation:** **Dermatomyositis** is the correct answer. **Gottron’s sign** is a pathognomonic clinical feature of this idiopathic inflammatory myopathy. It presents as symmetric, erythematous to violaceous macules or plaques located over the dorsal aspect of the interphalangeal and metacarpophalangeal joints. These lesions may sometimes become atrophic or scaly. It is distinct from **Gottron’s papules**, which are palpable, lichenoid papules in the same distribution. **Analysis of Incorrect Options:** * **Lupus Erythematosus (SLE):** While SLE also presents with photosensitive rashes, the classic malar rash spares the nasolabial folds. Crucially, hand involvement in SLE typically affects the **interarticular skin** (the skin between the joints), specifically sparing the knuckles, which helps differentiate it from Dermatomyositis. * **Scleroderma:** This condition is characterized by skin thickening and tightening (sclerodactyly) and Raynaud’s phenomenon, but it does not feature Gottron’s sign. * **Bell’s Palsy:** This is an isolated lower motor neuron facial nerve palsy and has no primary dermatological manifestations. **High-Yield Clinical Pearls for NEET-PG:** * **Heliotrope Rash:** Violaceous edema of the upper eyelids (another pathognomonic sign). * **Shawl Sign/V-sign:** Erythema over the upper back/shoulders or anterior chest. * **Mechanic’s Hands:** Hyperkeratosis and fissuring of the lateral and palmar aspects of the fingers (associated with Anti-Jo-1 antibodies). * **Malignancy:** Dermatomyositis in adults is frequently associated with internal malignancies (paraneoplastic syndrome).
Question 74: What is the percentage of patients with disseminated discoid lupus erythematosus who develop systemic lupus erythematosus?
- A. 2%
- B. 22% (Correct Answer)
- C. 52%
- D. 82%
Explanation: **Explanation:** The progression of Discoid Lupus Erythematosus (DLE) to Systemic Lupus Erythematosus (SLE) depends significantly on the distribution of skin lesions. DLE is categorized into two types: **Localized** (lesions confined to the head and neck) and **Disseminated** (lesions occurring both above and below the neck). 1. **Why 22% is correct:** While only about **5%** of patients with localized DLE progress to SLE, the risk increases significantly in the disseminated form. Clinical studies and standard dermatology textbooks (such as Fitzpatrick) indicate that approximately **22%** of patients with disseminated DLE will eventually develop systemic involvement. The presence of lesions on the trunk and extremities often correlates with a higher frequency of immunological abnormalities (like ANA positivity). 2. **Why other options are incorrect:** * **A (2%):** This is too low; even localized DLE has a higher conversion rate (~5%). * **C (52%) & D (82%):** These figures are overestimates. While disseminated DLE carries a higher risk than localized DLE, the majority of patients (nearly 80%) still do not develop full-blown systemic disease. **High-Yield Clinical Pearls for NEET-PG:** * **Most common site for DLE:** The concha of the ear. * **Classic Histology:** Interface dermatitis, follicular plugging, and basement membrane thickening. * **Lupus Band Test (LBT):** In DLE, the LBT is positive in **lesional skin** only. In SLE, the LBT can be positive in both lesional and **sun-exposed non-lesional skin**. * **Risk Factor:** Patients with disseminated DLE and high-titer ANA are at the highest risk for progression to SLE.
Question 75: Granular deposit of IgA at the dermoepidermal junction is seen in which of the following conditions?
- A. Pemphigus vulgaris
- B. Bullous pemphigoid
- C. Dermatitis herpetiformis (Correct Answer)
- D. Pemphigus foliaceus
Explanation: **Explanation:** The correct answer is **Dermatitis herpetiformis (DH)**. This condition is a cutaneous manifestation of gluten-sensitive enteropathy (Celiac disease). The hallmark immunopathological finding in DH is the **granular deposition of IgA** at the tips of the dermal papillae (dermoepidermal junction). These deposits are directed against **epidermal transglutaminase (eTG)**. **Why the other options are incorrect:** * **Pemphigus vulgaris:** Characterized by **IgG and C3** deposits in a **"fish-net" or "lace-like"** pattern within the epidermis (intercellular), targeting Desmoglein 3. * **Bullous pemphigoid:** Shows **linear** (not granular) deposition of **IgG and C3** along the basement membrane zone (BMZ), targeting BP180 and BP230. * **Pemphigus foliaceus:** Similar to Pemphigus vulgaris, it shows a **fish-net IgG** pattern, but it is localized to the superficial layers of the epidermis, targeting Desmoglein 1. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Presentation:** Intensely pruritic, grouped vesicles (herpetiform) symmetrically distributed on extensor surfaces (elbows, knees, buttocks). * **Histopathology:** Characterized by **neutrophilic microabscesses** (Papillary tip abscesses) and subepidermal blisters. * **Association:** Strongly associated with **HLA-DQ2 and HLA-DQ8**. * **Treatment of Choice:** **Dapsone** (provides rapid symptomatic relief) along with a strict **gluten-free diet**. * **Gold Standard Diagnosis:** Direct Immunofluorescence (DIF) of perilesional skin showing granular IgA.
Question 76: IgA deposits are seen on a skin biopsy in which of the following conditions?
- A. Henoch-Schonlein purpura (Correct Answer)
- B. Giant cell arteritis
- C. Microscopic polyangiitis
- D. Wegener's granulomatosis
Explanation: **Explanation:** **Henoch-Schönlein Purpura (HSP)**, also known as **IgA Vasculitis**, is the correct answer because it is a small-vessel vasculitis characterized by the deposition of **IgA-dominant immune complexes** in the walls of small vessels (capillaries, venules, or arterioles). On Direct Immunofluorescence (DIF) of a skin biopsy, the hallmark finding is granular IgA deposits within the dermal vessel walls. Clinically, this presents as the classic tetrad of palpable purpura, arthralgia, abdominal pain, and renal involvement (IgA nephropathy). **Analysis of Incorrect Options:** * **B. Giant Cell Arteritis:** This is a large-vessel vasculitis. Diagnosis is made via temporal artery biopsy showing granulomatous inflammation; it is not characterized by IgA deposits. * **C. Microscopic Polyangiitis (MPA):** This is a small-vessel vasculitis associated with **p-ANCA** (anti-MPO). It is a "pauci-immune" vasculitis, meaning there are little to no immunoglobulin deposits on immunofluorescence. * **D. Wegener’s Granulomatosis (GPA):** Now called Granulomatosis with Polyangiitis, this is a small-vessel vasculitis associated with **c-ANCA** (anti-PR3). Like MPA, it is pauci-immune and lacks significant IgA deposition. **High-Yield Clinical Pearls for NEET-PG:** * **DIF in Dermatology:** IgA deposits are also the hallmark of **Dermatitis Herpetiformis** (granular deposits at the tips of dermal papillae) and **Linear IgA Bullous Dermatosis** (linear deposits along the BMZ). * **HSP Trigger:** Often follows an Upper Respiratory Tract Infection (URTI). * **Biopsy Timing:** For vasculitis, the best site for biopsy is a fresh lesion (<24–48 hours old) to capture the immune deposits before they are degraded by inflammation.
Question 77: A 45-year-old patient presents with itchy, flat-topped, polygonal, violaceous papules on the inner wrists and flexors surfaces of the forearms. The lesions have a characteristic shiny surface and are arranged in a linear pattern. The patient denies any recent medication changes. What is the treatment?
- A. Anti-fungal
- B. Topical steroids (Correct Answer)
- C. Antibiotics
- D. Immunosuppressants
Explanation: ***Topical steroids***- **Lichen planus** is an inflammatory, immune-mediated disorder, and high-potency **topical corticosteroids** are the first-line treatment for localized cutaneous disease on flexural surfaces.- They reduce the inflammation (T-cell-mediated injury to the **basal layer** of the epidermis) and effectively alleviate symptoms like intense **pruritus** (itching).*Antibiotics*- Lichen planus is a **sterile inflammatory process**, not caused by a bacterial infection, making antibiotics ineffective for treating the underlying pathology.- Antibiotics are reserved for infectious conditions like cellulitis or **secondary bacterial infection** of the lesions, which is not indicated here.*Immunosuppressants*- Systemic **immunosuppressants** (e.g., methotrexate, cyclosporine) are reserved for severe, widespread, or treatment-refractory cases, or extensive subtypes like **erosive oral lichen planus**.- For localized cutaneous disease, the risk profile of systemic immunosuppression does not justify its use over topical therapy.*Anti-fungal*- Anti-fungal agents are used to treat infections caused by fungi, such as **Tinea corporis** (ringworm), which presents with scaly, erythematous plaques, not flat-topped violaceous papules.- Lichen planus is an autoimmune/inflammatory dermatosis; therefore, anti-fungals have no role in its primary management.
Question 78: A patient presented with itchy lesions, as shown below. What is the diagnosis?
- A. Scabies
- B. Lichen planus (Correct Answer)
- C. Psoriasis
- D. Warts
Explanation: ***Lichen planus*** - The image displays classic features of lichen planus, which are often described by the **'5 P's'**: **P**ruritic (itchy), **P**olygonal, **P**lanar (flat-topped), **P**urple **P**apules and plaques. - A characteristic sign, though not always clearly visible, is the presence of fine white lines on the surface of the lesions, known as **Wickham's striae**. It is also associated with **Hepatitis C** infection. *Scabies* - Scabies presents with intensely pruritic small papules, vesicles, and pathognomonic **burrows**, which are not seen in the image. The lesions shown are large plaques, not typical for a mite infestation. - The distribution of scabies is characteristic, favoring **finger web spaces**, wrists, axillae, and the genital area, whereas the lesions shown are on a broader surface. *Psoriasis* - Psoriasis typically appears as well-demarcated, erythematous plaques covered with a thick, **silvery-white scale**. The lesions in the image are violaceous and lack the prominent silvery scale. - A key clinical sign in psoriasis is the **Auspitz sign**, where pinpoint bleeding occurs after the scale is removed. Lesions are commonly found on **extensor surfaces** like the elbows and knees. *Warts* - Warts (verruca vulgaris) are caused by the **Human Papillomavirus (HPV)** and present as hyperkeratotic, exophytic papules with a rough, papillomatous surface, unlike the flat-topped lesions in the image. - On close inspection, warts often show thrombosed capillaries appearing as **black dots** (pepper pot sign), which are absent in these lesions.
Question 79: A patient presents with a violaceous rash on the upper back and shoulders (img-16.jpeg) and Gottron's papules on hands (img-17.jpeg). What is the likely diagnosis?
- A. Systemic lupus erythematosus
- B. Dermatomyositis (Correct Answer)
- C. Systemic sclerosis
- D. Cushing syndrome
Explanation: ***Dermatomyositis*** - The presence of a **heliotrope rash** (violaceous eruption on the upper eyelids) and **Gottron's papules** (violaceous papules over the knuckles) are pathognomonic for this inflammatory myopathy. - Other characteristic skin findings include the **shawl sign** (a photosensitive rash on the upper back, shoulders, and posterior neck, as seen in the image), the **V-sign**, and associated proximal muscle weakness. *Systemic lupus erythematosus* - SLE is classically associated with a **malar rash** (butterfly rash) over the bridge of the nose and cheeks, which spares the nasolabial folds. - While photosensitivity is a common feature, the specific findings of a heliotrope rash and Gottron's papules are not typical for SLE. *Systemic sclerosis* - This condition is characterized by progressive **skin thickening** and fibrosis, particularly **sclerodactyly** (tightening of the skin on the fingers). - Key differentiating features include **Raynaud phenomenon** and internal organ involvement, which are absent in this presentation. *Cushing syndrome* - This endocrine disorder is caused by excess **cortisol** and presents with distinct features like a **moon face**, **buffalo hump**, and purple **striae**. - It does not cause the inflammatory, photosensitive rashes that are characteristic of dermatomyositis.
Question 80: A woman presents with pruritic rash on the elbows, buttocks with recent diagnosis of gluten sensitive enteropathy. On immunofluorescence IgA deposition is seen as shown in the image. What is the most likely diagnosis?
- A. Psoriasis
- B. Dermatitis herpetiformis (Correct Answer)
- C. Bullous pemphigoid
- D. Pemphigus vulgaris
Explanation: ***Dermatitis herpetiformis*** - The combination of severely **pruritic, grouped vesicles (herpetiform)** on extensor surfaces (elbows, buttocks) and underlying **gluten-sensitive enteropathy** is pathognomonic. - The immunofluorescence image demonstrates characteristic **granular IgA deposition** specifically in the **dermal papillae**, which confirms the diagnosis. *Pemphigus vulgaris* - DIF typically shows a **"fishnet" pattern** of **IgG and C3** deposition in the **intercellular spaces** of the epidermis. - Clinically presents with **flaccid intraepidermal bullae** and extensive mucosal erosions, which is distinct from the patient's presentation. *Bullous pemphigoid* - DIF is characterized by **linear deposition of IgG and C3 along the basement membrane zone** (BMZ), not granular IgA in the dermal papillae. - Clinically, it presents with **tense bullae**, often in the elderly, and is not linked to gluten-sensitive enteropathy. *Psoriasis* - This condition is characterized by **erythematous plaques with silvery scales** (Koebner phenomenon is common) and is a papulosquamous disorder, not a bullous disease. - The histopathology involves acanthosis and Munro microabscesses, and it lacks the specific **IgA deposits** seen in the image.
Practice by Chapter
Lupus Erythematosus: Cutaneous Forms
Practice Questions
Lupus Erythematosus: Systemic with Skin Manifestations
Practice Questions
Dermatomyositis
Practice Questions
Scleroderma and Morphea
Practice Questions
Mixed Connective Tissue Disease
Practice Questions
Sjögren's Syndrome: Cutaneous Manifestations
Practice Questions
Relapsing Polychondritis
Practice Questions
Autoimmune Thyroid Disease and the Skin
Practice Questions
Immunobullous Disorders
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Vasculitis
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Diagnostic Methods in Autoimmune Dermatoses
Practice Questions
Management of Autoimmune Skin Diseases
Practice Questions
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