Autoimmune Skin Diseases Practice Questions

Q: 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?

Dermatomyositis. ***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.

Q: 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?

Dermatitis herpetiformis. ***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.

Q: All the following statements regarding the image given are true except: (Recent Neet Pattem 2016-17)

Most commonly seen in reproductively active females. ***Most commonly seen in reproductively active females*** - **Lichen sclerosus** predominantly affects **prepubertal girls** and **postmenopausal women**, not reproductively active females. - The disease is associated with **hormonal changes**, explaining its bimodal age distribution. *Lichen sclerosis* - The image displays characteristic **whitish, atrophic, thin skin** with areas of **erythema and erosions** in the anogenital region, consistent with vulvar lichen sclerosus. - This chronic inflammatory skin condition primarily affects the **genital and perianal areas**. *Premalignant* - While not inherently malignant, lichen sclerosus is considered a **premalignant condition** with a small risk of transforming into **squamous cell carcinoma**. - Long-standing or inadequately treated lichen sclerosus can lead to chronic inflammation and cellular atypia, increasing the risk of malignant change. *Can involve anus* - Lichen sclerosus commonly involves the anogenital region, forming a **figure-of-eight or hourglass shape** around the vulva and anus. - Involvement of the anus can lead to **pruritus, pain**, and, in severe cases, anal fissures and scarring.

Q: What is the most likely diagnosis of the image given below?

Dermatomyositis. ***Dermatomyositis*** - The image displays a characteristic **heliotrope rash** (violaceous/purple-red discoloration) around the eyes and periorbital area, which is pathognomonic for dermatomyositis. - This condition is an **inflammatory myopathy** distinguished by distinct cutaneous manifestations (including Gottron's papules, shawl sign, and V-sign) alongside proximal muscle weakness. - The periorbital violaceous erythema with associated edema is highly characteristic and helps differentiate it from other facial rashes. *Acne rosacea* - Characterized by **facial erythema**, papules, pustules, and telangiectasias, primarily affecting the central face (cheeks, nose, chin, forehead). - Does not typically involve the marked periorbital (heliotrope) rash with violaceous discoloration seen in dermatomyositis. - Usually presents with flushing and visible blood vessels rather than periorbital edema. *Dermatitis herpetiformis* - Presents with intensely **pruritic (itchy) papules and vesicles** (blisters), typically symmetrical on extensor surfaces like elbows, knees, buttocks, and scalp. - Strongly associated with **celiac disease** (gluten-sensitive enteropathy). - Does not present as facial erythema or periorbital discoloration; the lesions are vesicular and intensely itchy. *Erythema marginatum* - A skin manifestation of **acute rheumatic fever**, characterized by an evanescent, **serpiginous (snake-like) migratory rash** with distinct, raised borders and clear centers. - Typically appears on the trunk and proximal limbs, sparing the face. - Does not present as periorbital discoloration and has a distinctive migrating, annular pattern.

Q: A patient presents with violaceous papules over the knuckles and mottled pigmentation on the dorsum of hands. Identify the lesion:

Dermatomyositis. ***Dermatomyositis*** - The image exhibits **Gottron's papules**, which are violaceous, erythematous papules over the **dorsal aspects of the interphalangeal joints** (knuckles), a **pathognomonic cutaneous finding** in dermatomyositis - Additionally, the **spotted hyperpigmentation and hypopigmentation** (poikiloderma) on the dorsum of the hands is another common skin manifestation of dermatomyositis, especially in sun-exposed areas - Dermatomyositis is an **idiopathic inflammatory myopathy** with characteristic cutaneous and muscular involvement *Vitiligo* - Vitiligo presents as **well-demarcated depigmented macules or patches** due to destruction of melanocytes, resulting in a stark **white/chalky appearance** - The lesions in the image are **papular and violaceous** with combination of hyper- and hypopigmentation, not uniform complete depigmentation - Lacks the characteristic milky-white patches of vitiligo *Dermatitis herpetiformis* - Characterized by **intensely pruritic, grouped vesicles** on erythematous base, mainly on extensor surfaces (elbows, knees, buttocks) - Associated with **celiac disease** (gluten sensitivity) - The lesions in the image lack the typical **vesicular/blistering** appearance and are located specifically over the knuckles, not the classic distribution *Erythema marginatum* - Presents as **migratory, transient, non-pruritic, erythematous macules** with clear centers forming annular/serpiginous patterns - Classic cutaneous manifestation of **acute rheumatic fever** (Jones criteria) - The lesions in the image are **papular, fixed, and violaceous**, not flat erythematous rings

Q: A 26-year-old female with a history of extensive exposure to sun comes to your clinic with presentation shown below. What is the most likely diagnosis?

Dermatomyositis. ***Dermatomyositis*** - The image shows **Gottron's papules** (violaceous, flat-topped papules over bony prominences like knuckles) or a **heliotrope rash** (periorbital violaceous rash), which are classic skin findings in dermatomyositis, an inflammatory myopathy. - The history of **sun exposure** is relevant as dermatomyositis rashes are often **photosensitive**, and the patient's age and gender are consistent with typical presentation. *Urticaria* - Urticaria presents as rapidly appearing and disappearing **wheals** (hives) that are typically itchy and erythematous with central pallor. - The lesions in the image appear more fixed and papular rather than evanescent wheals, making urticaria less likely. *Dermatitis herpetiformis* - Dermatitis herpetiformis is characterized by intensely **pruritic vesicles and bullae** symmetrically distributed on extensor surfaces, often associated with celiac disease. - The appearance in the image does not show blistering or the characteristic distribution of dermatitis herpetiformis. *Erythema marginatum* - Erythema marginatum is a rare, transient, and non-pruritic serpiginous (snake-like) rash with sharply demarcated borders and central clearing, typically seen in **acute rheumatic fever**. - The rash in the image lacks the characteristic migratory, annular, or serpiginous pattern of erythema marginatum.

Q: Identify the lesion:

Dermatomyositis. ***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.

Q: A patient presents with oral mucosal lesions. Identify the condition shown in the image:

Lichen planus. - ***Lichen planus*** - The image exhibits **Wickham's striae**, which are characteristic fine, white, lacy patterns seen on the surface of papules and plaques in lichen planus, especially on mucosal surfaces. - Lichen planus often presents as **pruritic, purple, polygonal papules**, and this appearance is consistent with its oral manifestation. - *Dermatomyositis* - Dermatomyositis is characterized by **Gottron's papules** (violaceous papules over bony prominences, especially knuckles) and a **heliotrope rash** on the eyelids. - The lesion in the image does not show these typical features of dermatomyositis. - *Psoriasis* - Psoriasis typically presents as **erythematous plaques with silvery scales**, often on extensor surfaces, and can show pinpoint bleeding (Auspitz sign) when scales are removed. - The lacy, reticular white pattern (Wickham's striae) seen in the image is not a feature of psoriasis. - *Dermatitis herpetiformis* - Dermatitis herpetiformis is characterized by intensely **pruritic vesicles and bullae** symmetrically distributed, often on extensor surfaces, associated with celiac disease. - The lesion in the image is not vesicular or bullous, nor does it present with the characteristic distribution of dermatitis herpetiformis.

Q: An elderly patient presents with itchy tense blisters on normal looking skin as well as on urticarial plaques as shown below. The most probable diagnosis is: (AIIMS Nov 2015)

Bullous pemphigoid. ***Bullous pemphigoid*** - This condition typically presents in **elderly patients** with **itchy, tense blisters** on either normal or erythematous/urticarial skin, which aligns with the clinical description and image. - The blisters in bullous pemphigoid are characterized by **subepidermal blistering**, meaning the epidermis separates from the dermis, resulting in tense, fluid-filled lesions. *Pemphigus vulgaris* - Characterized by **flaccid blisters** that rupture easily, leading to erosions, unlike the tense blisters seen in the image. - Pemphigus vulgaris frequently involves **mucous membranes** and is caused by autoantibodies against desmoglein 1 and 3, leading to intraepidermal blistering (acantholysis). *Linear IgA disease* - This autoimmune blistering condition typically presents with **annular or rosette-shaped lesions** with small peripheral blisters, often referred to as a "string of pearls" appearance, which is not evident in the image. - On **direct immunofluorescence**, it shows a linear deposition of IgA at the dermoepidermal junction. *Dermatitis herpetiformis* - Often presents with **intensely pruritic papules and vesicles** typically found on extensor surfaces (elbows, knees, buttocks), and the lesions are often excoriated due to scratching. - Strongly associated with **celiac disease** and characterized by granular IgA deposits in the dermal papillae on direct immunofluorescence, distinguishing it from the tense blisters seen.

Q: The following lesion appears on the leg of a patient of ulcerative colitis. All are useful in management except:

Sulfapyridine. ***Sulfapyridine*** - The image shows **pyoderma gangrenosum**, a painful ulcerative skin condition often associated with inflammatory bowel disease like ulcerative colitis. Among the given options, **sulfapyridine** has the **least established role** in pyoderma gangrenosum management. - **Sulfapyridine** is an inactive component of **sulfasalazine** and primarily acts as an **antibacterial agent**. While sulfasalazine has been reported in some PG cases, sulfapyridine alone is not a recognized treatment for the inflammatory, non-infectious nature of pyoderma gangrenosum. - Unlike the other options which have well-established roles, sulfapyridine lacks strong evidence for efficacy in PG. *Steroids* - **Corticosteroids** (oral or topical) are the **first-line treatment** for pyoderma gangrenosum due to their potent anti-inflammatory and immunosuppressive effects. - They help to reduce the inflammation and promote healing of the painful ulcers. *Procto-colectomy* - In cases of severe, refractory pyoderma gangrenosum associated with ulcerative colitis, **colectomy** can be a **definitive treatment** as it removes the underlying inflammatory trigger. - This surgical intervention is considered when medical therapies are unsuccessful or when the colonic disease itself necessitates surgery. *Infliximab* - **Infliximab**, a **TNF-alpha inhibitor**, is a biologic agent effective in treating both ulcerative colitis and pyoderma gangrenosum. - It is used in cases that are refractory to steroids or when patients cannot tolerate steroid therapy.

Question 1

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?

Accepted Answer

Dermatomyositis

Answer

Systemic lupus erythematosus

Answer

Systemic sclerosis

Answer

Cushing syndrome

Question 2

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?

Accepted Answer

Dermatitis herpetiformis

Answer

Psoriasis

Answer

Bullous pemphigoid

Answer

Pemphigus vulgaris

Question 3

All the following statements regarding the image given are true except: (Recent Neet Pattem 2016-17)

Accepted Answer

Most commonly seen in reproductively active females

Answer

Lichen sclerosus

Answer

Premalignant

Answer

Can involve anus

Question 4

What is the most likely diagnosis of the image given below?

Accepted Answer

Dermatomyositis

Answer

Acne rosacea

Answer

Dermatitis herpetiformis

Answer

Erythema marginatum

Question 5

A patient presents with violaceous papules over the knuckles and mottled pigmentation on the dorsum of hands. Identify the lesion:

Accepted Answer

Dermatomyositis

Answer

Vitiligo

Answer

Dermatitis herpetiformis

Answer

Erythema marginatum

Question 6

A 26-year-old female with a history of extensive exposure to sun comes to your clinic with presentation shown below. What is the most likely diagnosis?

Accepted Answer

Dermatomyositis

Answer

Urticaria

Answer

Dermatitis herpetiformis

Answer

Erythema marginatum

Question 7

Identify the lesion:

Accepted Answer

Dermatomyositis

Answer

Psoriasis

Answer

Dermatitis herpetiformis

Answer

Erythema marginatum

Question 8

A patient presents with oral mucosal lesions. Identify the condition shown in the image:

Accepted Answer

Lichen planus

Answer

Dermatomyositis

Answer

Psoriasis

Answer

Dermatitis herpetiformis

Question 9

An elderly patient presents with itchy tense blisters on normal looking skin as well as on urticarial plaques as shown below. The most probable diagnosis is: (AIIMS Nov 2015)

Accepted Answer

Bullous pemphigoid

Answer

Pemphigus vulgaris

Answer

Linear IgA disease

Answer

Dermatitis herpetiformis

Question 10

The following lesion appears on the leg of a patient of ulcerative colitis. All are useful in management except:

Accepted Answer

Sulfapyridine

Answer

Steroids

Answer

Procto-colectomy

Answer

Infliximab

Autoimmune Skin Diseases — MCQs

Autoimmune Skin Diseases — MCQs

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