Autoimmune Skin Diseases — MCQs

Autoimmune Skin Diseases Indian Medical PG Practice Questions and MCQs

Question 91: The skin condition shown in the image is associated with?

A. Diabetes mellitus (Correct Answer)
B. Hypothyroidism
C. Hyperthyroidism
D. Sarcoidosis

Explanation: ***Diabetes mellitus*** - The image shows **diabetic dermopathy** (also known as "shin spots"), which presents as hyperpigmented, atrophic macules or papules, usually on the shins. This condition is a common cutaneous manifestation of **diabetes mellitus**. - Other dermatological conditions associated with diabetes include **necrobiosis lipoidica diabeticorum**, **acanthosis nigricans**, and **erythrasma**, which are important to recognize in patients with diabetes. *Hypothyroidism* - Hypothyroidism is associated with **myxedema**, which typically manifests as non-pitting edema, dry and coarse skin, and hair loss. - While it can cause skin changes, it does not typically present with the pigmented, atrophic lesions seen in the image. *Hyperthyroidism* - Hyperthyroidism can cause skin changes such as **pretibial myxedema** (a specific form of localized skin thickening, typically on the shins, that is often associated with Graves' disease) and warm, moist skin due to increased metabolism. - The lesions shown in the image are not consistent with the typical presentation of pretibial myxedema or other hyperthyroid skin manifestations. *Sarcoidosis* - Sarcoidosis can present with various skin lesions, including **erythema nodosum**, lupus pernio, plaques, and papules. - The skin changes seen in the image, characterized by small, atrophic, hyperpigmented macules, do not fit the typical pattern of cutaneous sarcoidosis.

Question 92: A 45-year-old Ulcerative colitis patient presents with multiple painful lesions on both legs. What is the diagnosis?

A. Pyoderma gangrenosum (Correct Answer)
B. Febrile neutropenic dermatosis
C. Necrotizing fasciitis
D. Granulomatosis with polyangiitis

Explanation: ***Pyoderma gangrenosum*** - This patient has **ulcerative colitis**, which is strongly associated with **pyoderma gangrenosum**, a neutrophilic dermatosis. - The image shows characteristic **painful, rapidly expanding ulcers** with violaceous, undermined borders, typical of pyoderma gangrenosum. *Febrile neutropenic dermatosis* - This condition (also known as **Sweet syndrome**) occurs in patients with **neutropenia** and **fever**, presenting with painful erythematous plaques or nodules. - While systemic illness like ulcerative colitis can predispose to skin conditions, the specific presentation and lack of mentioned neutropenia make this less likely. *Necrotizing fasciitis* - **Necrotizing fasciitis** is a rapidly progressive, life-threatening infection of the deep fascia and subcutaneous tissue, typically presenting with severe pain, erythema, swelling, and crepitus. - The lesions in the image appear to be chronic ulcers with specific borders rather than acute, rapidly spreading infection of necrotizing fasciitis. *Granulomatosis with polyangiitis* - Also known as **Granulomatosis with polyangiitis (GPA)**, formerly **Wegener's granulomatosis**, this is an autoimmune vasculitis primarily affecting the respiratory tract and kidneys, and can cause skin lesions such as palpable purpura, nodules, or ulcers. - While skin lesions can occur, the characteristic features of **pyoderma gangrenosum** and its strong association with inflammatory bowel disease make it a more probable diagnosis in this context.

Question 93: Which one of the following is NOT true of Pyoderma gangrenosum?

A. It is often secondary to heightened immunological reactivity from another disease process
B. Cultures often show Gram positive Staphylococci (Correct Answer)
C. Lesions generally respond to steroids
D. It is characterized by cutaneous ulceration with purple undermined edges

Explanation: ***Cultures often show Gram positive Staphylococci*** - Pyoderma gangrenosum is a **sterile inflammatory dermatosis**, meaning that the ulcers are not caused by bacterial infection. - While secondary infection can occur, the primary lesion itself is **non-infectious**, and therefore, routine cultures of the ulcer base would typically be negative for primary pathogens like *Staphylococci*. *It is often secondary to heightened immunological reactivity from another disease process* - Pyoderma gangrenosum is well-known for its association with underlying systemic conditions, particularly **inflammatory bowel disease**, **rheumatoid arthritis**, and certain **hematologic malignancies**. - Its pathogenesis is thought to involve **dysregulated neutrophils** and an underlying autoimmune or autoinflammatory process. *Lesions generally respond to steroids* - **Corticosteroids** are the first-line treatment for Pyoderma gangrenosum, used to reduce inflammation and promote healing. - Both topical and systemic corticosteroids, such as **prednisone**, are effective in managing the condition. *It is characterized by cutaneous ulceration with purple undermined edges* - The classic presentation of Pyoderma gangrenosum is a rapidly evolving, painful, **necrotic ulcer** with a characteristic **violaceous (purple) undermined border**. - This distinctive appearance helps differentiate it from other types of skin ulcers.

Question 94: A 32-year-old woman presents with multiple pruritic papules and vesicles on her elbows and knees. She reports a history of gluten sensitivity. Skin biopsy shows granular IgA deposits in dermal papillae. Which of the following is most likely to be elevated in this patient's serum?

A. Anti-DNA antibodies
B. Anti-type VII collagen antibodies
C. Anti-BP180 antibodies
D. Anti-tissue transglutaminase antibodies (Correct Answer)

Explanation: ***Anti-tissue transglutaminase antibodies*** - The patient's presentation with **pruritic papules and vesicles on elbows and knees**, history of **gluten sensitivity**, and **IgA deposits in dermal papillae** points to **dermatitis herpetiformis**. - **Dermatitis herpetiformis** is strongly associated with **celiac disease**, which involves an immune response to **gluten**, leading to elevated **anti-tissue transglutaminase (tTG) antibodies**. *Anti-DNA antibodies* - **Anti-DNA antibodies**, particularly **anti-dsDNA**, are characteristic markers for **systemic lupus erythematosus (SLE)**, a systemic autoimmune disease not suggested by the clinical picture or biopsy findings. - SLE presents with a wide range of symptoms including joint pain, malar rash, renal involvement, and serositis, which are not described here. *Anti-type VII collagen antibodies* - **Anti-type VII collagen antibodies** are associated with **epidermolysis bullosa acquisita (EBA)**, a subepidermal blistering disease. - EBA lesions are typically more **tense bullae** and often associated with trauma, and the immunofluorescence pattern is different from the granular IgA deposits seen in dermatitis herpetiformis. *Anti-BP180 antibodies* - **Anti-BP180 antibodies** (also known as anti-BPAg2) are the primary autoantibodies found in **bullous pemphigoid**, another subepidermal blistering disease. - **Bullous pemphigoid** typically presents with large, tense blisters in elderly patients, and direct immunofluorescence shows **linear IgA/IgG deposition along the basement membrane zone**, not granular IgA in dermal papillae.

Question 95: 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 96: 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 97: A young female presented with lacy linear lesions on tongue for a month with elongation of nail fold beyond the nail bed. What is the diagnosis –

A. Candidiasis
B. Psoriasis
C. Geographic tongue
D. Lichen planus (Correct Answer)

Explanation: ***Lichen planus*** - **Lacy linear lesions on the tongue** are characteristic of **Wickham's striae**, a hallmark feature of oral lichen planus. - **Elongation of the nail fold beyond the nail bed** (known as pterygium formation) is a specific nail finding seen in lichen planus. *Candidiasis* - Oral candidiasis typically presents as **white, creamy patches** that can be scraped off, unlike the lacy lesions described. - It does not typically cause **nail fold elongation** or similar specific nail changes. *Psoriasis* - While psoriasis can affect the tongue (geographic tongue-like lesions) and nails, the classic oral lesions are not described as **lacy linear patterns**. - Nail changes in psoriasis include **pitting, onycholysis, and oil spots**, not usually elongation of the nail fold beyond the nail bed. *Geographic tongue* - Geographic tongue presents as **irregular, depapillated red patches with white borders** that migrate over time. - It is a benign inflammatory condition of the tongue and is not associated with **nail changes** like elongated nail folds.

Question 98: 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 99: A 26-year-old female patient presented with oral ulcers, sensitivity to light and rash over the malar area of the face sparing the nasolabial folds of both sides. Which of the following is most characteristic of this condition?

A. Butterfly rash sparing the nasolabial folds (Correct Answer)
B. Heliotrope rash on upper eyelid, bilateral hilar lymphadenopathy
C. Port-wine stain with CNS malformations
D. Silvery scales or plaques

Explanation: ***Butterfly rash sparing the nasolabial folds*** - The patient's presentation with oral ulcers, sensitivity to light (photosensitivity), and a rash over the malar area **sparing the nasolabial folds** is highly characteristic of **Systemic Lupus Erythematosus (SLE)**, a condition where a butterfly rash is a hallmark. - This specific distribution of the malar rash is a key diagnostic feature differentiating it from other facial rashes. *Heliotrope rash on upper eyelid, bilateral hilar lymphadenopathy* - A **heliotrope rash** on the upper eyelids is characteristic of **dermatomyositis**, which also presents with muscle weakness, not typically oral ulcers or a malar rash. - **Bilateral hilar lymphadenopathy** is a classic finding in **sarcoidosis**, a granulomatous disease, not directly related to the patient's symptoms. *Port-wine stain, CNS malformations: seen in Sturge-Weber syndrome* - A **port-wine stain** (nevus flammeus) is a congenital capillary malformation, often on the face, and is a principal feature of **Sturge-Weber syndrome**, which involves CNS malformations and seizures. - This condition has no association with oral ulcers, photosensitivity, or the described malar rash. *Silvery scales or plaques: seen in psoriasis* - **Silvery scales or plaques** are the characteristic dermatological lesions of **psoriasis**, a chronic inflammatory skin condition. - Psoriasis typically presents with well-demarcated erythematous plaques and is not associated with oral ulcers, photosensitivity, or a lupus-specific malar rash.

Question 100: Gottron papules are seen in:

A. Behcet's syndrome
B. Sarcoidosis
C. Dermatomyositis (Correct Answer)
D. Scleroderma

Explanation: ***Dermatomyositis*** - **Gottron papules** are pathognomonic for **dermatomyositis**, presenting as violaceous, flat-topped papules or plaques over the extensor surfaces of the **metacarpophalangeal (MCP)** and **interphalangeal (IP)** joints. - They are one of the characteristic cutaneous manifestations, often accompanied by **heliotrope rash** and **proximal muscle weakness**. *Behcet's syndrome* - Characterized by **recurrent oral and genital aphthous ulcers**, **ocular inflammation**, and skin lesions like **erythema nodosum**, but not Gottron papules. - It is a **vasculitis** affecting various organs, primarily due to immune dysregulation. *Sarcoidosis* - A **multisystem granulomatous disorder** that can affect the skin with lesions such as **lupus pernio** or **erythema nodosum**, but not Gottron papules. - It is often associated with pulmonary involvement and can cause **hypercalcemia**. *Scleroderma* - Also known as **systemic sclerosis**, is characterized by **skin thickening** and **fibrosis** due to excessive collagen deposition. - Common skin manifestations include **Raynaud phenomenon**, **sclerodactyly**, and **telangiectasias**, distinct from Gottron papules.

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

Practice Questions

Vasculitis

Practice Questions

Diagnostic Methods in Autoimmune Dermatoses

Practice Questions

Management of Autoimmune Skin Diseases

Practice Questions

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