Autoimmune Skin Diseases Practice Questions

Q: Periungual telangiectasias are pathognomonic signs of the following except:

Polyarteritis nodosa. ***Polyarteritis nodosa*** - **Periungual telangiectasias** are generally *not* a characteristic or pathognomonic finding in Polyarteritis nodosa (PAN). - PAN is a **necrotizing vasculitis** primarily affecting medium-sized arteries, often presenting with systemic symptoms, **skin nodules**, livedo reticularis, and mononeuritis multiplex, but not typical periungual changes. *Systemic sclerosis* - **Periungual telangiectasias** are a common and characteristic finding in systemic sclerosis, often seen as part of the broader microvascular damage. - They tend to be prominent and can be associated with **Raynaud's phenomenon** and digital ischemia. - Part of the CREST syndrome features. *Dermatomyositis* - **Periungual telangiectasias** are a characteristic finding in dermatomyositis, along with other nail fold changes. - They are associated with other cutaneous features like **Gottron's papules**, heliotrope rash, and shawl sign. - Nail fold capillary changes are important diagnostic clues in inflammatory myopathies. *Lupus erythematosus* - **Periungual telangiectasias** are frequently observed in patients with systemic lupus erythematosus (SLE) and are considered a clinical sign of microvascular involvement. - They can be part of the broader mucocutaneous manifestations, which also include **malar rash** and discoid lesions.

Q: Acute febrile neutrophilic dermatosis is seen in-

Sweet syndrome. ***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.

Q: 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:

DLE. ***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.

Q: 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 –

Lichen planus. ***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.

Q: 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?

Butterfly rash sparing the nasolabial folds. ***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.

Q: Match the following autoantibodies with their associated conditions: 1. BP180 a. Pemphigus vulgaris 2. Desmoglein 3 b. Bullous pemphigoid 3. Type VII collagen c. Epidermolysis bullosa acquisita

1-b, 2-a, 3-c. ***1-b, 2-a, 3-c*** - **BP180** is an autoantigen targeted in **bullous pemphigoid**, a subepidermal blistering disease. - **Desmoglein 3** is a key autoantigen in **pemphigus vulgaris**, a life-threatening intraepidermal blistering disorder. - **Type VII collagen** is involved in anchoring fibrils and is the target of autoantibodies in **epidermolysis bullosa acquisita**, causing chronic blistering. *1-a, 2-b, 3-c* - This option incorrectly associates **BP180 with pemphigus vulgaris** and **Desmoglein 3 with bullous pemphigoid**; their associations are reversed. - While Type VII collagen is correctly linked with epidermolysis bullosa acquisita, the other pairings are incorrect. *1-c, 2-a, 3-b* - This option incorrectly links **BP180 with epidermolysis bullosa acquisita** and **Type VII collagen with bullous pemphigoid**. - While Desmoglein 3 is correctly paired with pemphigus vulgaris, the other two associations are wrong, making this overall incorrect. *1-b, 2-c, 3-a* - This option incorrectly links **Desmoglein 3 with epidermolysis bullosa acquisita** and **Type VII collagen with pemphigus vulgaris**. - While BP180 is correctly associated with bullous pemphigoid, the other two associations are incorrect.

Q: Gottron papules are seen in:

Dermatomyositis. ***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.

Q: Which skin manifestation is characteristically seen in dermatomyositis?

Gottron papules. ***Gottron papules*** - **Gottron papules** are raised, erythematous, or violaceous plaques over the **extensor surfaces of the metacarpophalangeal (MCP)** and **interphalangeal (IP)** joints. - They are considered **pathognomonic** (diagnostic) for dermatomyositis and are the most universally accepted characteristic cutaneous marker of the condition. *Heliotrope rash* - The **heliotrope rash** is a characteristic violaceous (purple-red) discoloration on the upper eyelids, often associated with **periorbital edema**. - While highly characteristic of **dermatomyositis**, Gottron papules are more universally accepted as the most pathognomonic feature. *Butterfly rash* - The **butterfly rash** (malar rash) is a classic manifestation of **systemic lupus erythematosus (SLE)**, characterized by redness over the cheeks and nasal bridge, sparing the nasolabial folds. - It is not typically seen in dermatomyositis and suggests a different underlying autoimmune condition. *Shawl sign* - The **shawl sign** refers to a diffuse, flat, erythematous rash over the **shoulders, upper back, and posterior neck**, often exacerbated by sun exposure. - Although seen in dermatomyositis, it is less specific than Gottron papules, which are directly diagnostic of the condition.

Q: Which finding is most specific for pemphigus vulgaris?

Intercellular IgG deposits. ***Intercellular IgG deposits*** - **Direct immunofluorescence** (DIF) showing **intercellular IgG deposits** in the epidermis is the **gold standard diagnostic finding** and most specific for pemphigus vulgaris. This reflects the presence of autoantibodies targeting **desmogleins 1 and 3**, key components of desmosomes. - These antibodies disrupt cell-to-cell adhesion, leading to the characteristic **flaccid bullae** seen clinically. - This immunopathological finding has near 100% sensitivity and specificity for pemphigus vulgaris. *Suprabasal acantholysis* - While **suprabasal acantholysis** is the most characteristic **light microscopic (H&E) finding** in pemphigus vulgaris, it is a morphological consequence of the autoantibody attack and not as specific as the immunofluorescence pattern. - **Acantholysis** refers to the loss of cohesion between keratinocytes, leading to the formation of intraepidermal blisters with a characteristic "row of tombstones" appearance at the base. - This finding can occasionally be seen in other acantholytic conditions, making it less specific than DIF. *Granular IgA deposits* - **Granular IgA deposits** along the dermoepidermal junction are characteristic of **dermatitis herpetiformis**, a pruritic papulovesicular disease associated with celiac disease. - These deposits are typically found in the dermal papillae and are distinct from the intercellular IgG seen in pemphigus. *Basement membrane thickening* - **Basement membrane thickening** is a non-specific finding that can be seen in various chronic skin conditions, including **lichen sclerosus et atrophicus** or in some forms of **lupus erythematosus**. - It is not a primary or specific feature of pemphigus vulgaris, which is characterized by intraepidermal blistering due to loss of cell-to-cell adhesion.

Question 1

Periungual telangiectasias are pathognomonic signs of the following except:

Accepted Answer

Polyarteritis nodosa

Answer

Systemic sclerosis

Answer

Dermatomyositis

Answer

Lupus erythematosus

Question 2

Acute febrile neutrophilic dermatosis is seen in-

Accepted Answer

Sweet syndrome

Answer

Behcet's syndrome

Answer

Haberman syndrome

Answer

Kasabach-Merritt syndrome

Question 3

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:

Accepted Answer

DLE

Answer

Scleroderma

Answer

SLE

Answer

Lichen planus

Question 4

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 –

Accepted Answer

Lichen planus

Answer

Candidiasis

Answer

Psoriasis

Answer

Geographic tongue

Question 5

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?

Accepted Answer

Butterfly rash sparing the nasolabial folds

Answer

Heliotrope rash on upper eyelid, bilateral hilar lymphadenopathy

Answer

Port-wine stain with CNS malformations

Answer

Silvery scales or plaques

Question 6

A 70-year-old woman with bullous pemphigoid develops severe disease flare while tapering prednisone from 40mg daily. Current dose is 20mg daily. She has diabetes with HbA1c 8.5% and osteoporosis. Most appropriate next step is:

Accepted Answer

Add rituximab

Answer

Start doxycycline and niacinamide

Answer

Add azathioprine

Answer

Increase prednisone back to 40mg

Question 7

Match the following autoantibodies with their associated conditions:
1. BP180 a. Pemphigus vulgaris
2. Desmoglein 3 b. Bullous pemphigoid
3. Type VII collagen c. Epidermolysis bullosa acquisita

Accepted Answer

1-b, 2-a, 3-c

Answer

1-a, 2-b, 3-c

Answer

1-c, 2-a, 3-b

Answer

1-b, 2-c, 3-a

Question 8

Gottron papules are seen in:

Accepted Answer

Dermatomyositis

Answer

Behcet's syndrome

Answer

Sarcoidosis

Answer

Scleroderma

Question 9

Which skin manifestation is characteristically seen in dermatomyositis?

Accepted Answer

Gottron papules

Answer

Butterfly rash

Answer

Shawl sign

Answer

Heliotrope rash

Question 10

Which finding is most specific for pemphigus vulgaris?

Accepted Answer

Intercellular IgG deposits

Answer

Granular IgA deposits

Answer

Suprabasal acantholysis

Answer

Basement membrane thickening

Autoimmune Skin Diseases — MCQs

Autoimmune Skin Diseases — MCQs

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