Immune mediated Bullous Disorders

Immune mediated Bullous Disorders

Q. 1 In   pemphigus   vulgaris,   antibodies   are present against:
 A Basement membrane   
 B Intercellular substance
 C Cell nucleus 
 D Keratin
Q. 1 In   pemphigus   vulgaris,   antibodies   are present against:
 A Basement membrane   
 B Intercellular substance
 C Cell nucleus 
 D Keratin
Ans. B

Explanation:

In  Pemphigus  antibodies  are  directed  against desmoglein (a trans membrane desmosomal glycoprotein belonging to cadherin-a calcium dependent adhesion molecule) in intercellular substance causing loss of cohesion between epidermal cells (of st. basale layer) I/t formation of acantholytic cells which can be seen on Tzank smear. .


Q. 2

A person presents with hemorrhagic fluid in tense blister at dermoepidermal junction. Most probable diagnosis is?

 A Pemphigoid
 B

Pemphigus vulgaris

 C Pemphigus vegetans
 D

Drug induced pemphigus

Q. 2

A person presents with hemorrhagic fluid in tense blister at dermoepidermal junction. Most probable diagnosis is?

 A Pemphigoid
 B

Pemphigus vulgaris

 C Pemphigus vegetans
 D

Drug induced pemphigus

Ans. A

Explanation:

Pemphigoid REF: Rook’s dermatology 7th ed p. 41.3-41.19

Intraepidermal Bulla

Blister at Dermoepidermal Junction

Granular layer

Junctional

Pemphigus foliaceous

Junctional EB

Subcorneal pustular dermatosis

Bullous pemphigoid

Staphylococcal scalded skin syndrome

 

Bullous pemphigoid

Dermolytic

Spinous layer

Epidermolytic bullosa dystrophicans

Familial benign pemphigus

Epidermolytic bullosa aquistica

Suprabasal

Dermatitis herpetiformis

Pemphigus vulgaris

 

Darier’s disease

 

Basal layer

 

Erythema multiforme

 

Lupus erythematosus

 

Lichen planus

 

EBS

 

  • Pemphigus vegetans is a localized form of pemphigus vulgaris
  • The most common variant of pemphigus associated with drug exposure is pemphigus foliaceus, although pemphigus vulgaris has also been described

Q. 3

Epidermal bullae are seen in?

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Pemphigoid gestationalis

 D

Halley bailey disease

Q. 3

Epidermal bullae are seen in?

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Pemphigoid gestationalis

 D

Halley bailey disease

Ans. B

Explanation:

Ans:B.)Pemphigus vulgaris 


Q. 4

Which of the following shows deposition of IgA in dennal papilla –

 A

Dermatitis herpetiformis

 B

IgA papillomatosis of childhood

 C

Bullous pemphigoid

 D

Gestational herpes

Q. 4

Which of the following shows deposition of IgA in dennal papilla –

 A

Dermatitis herpetiformis

 B

IgA papillomatosis of childhood

 C

Bullous pemphigoid

 D

Gestational herpes

Ans. A

Explanation:

Dermatitis herpetiformis [Ref : Bobbin’s    1259, 1260]

Immtmopathological appearance of vesicobullous disorders
-• Bullous pemphigoid                              → Linear band of IgG or C3 in epidermal basement membrane
• Pemphigus vulgaris                                  Cell surface deposits of IgG on keratinocytes
• Pemphigus foliaceous                            → Cell surface deposits of IgG on keratinocytes
• Pemphigoid gesta tionis                            Linear band of C3 in epidermal basement membrane zone
• Dermatitis herpeteformis                        → Granular deposits of IgA in dermal papillae
• Chronic bullous dise ase of childhood       → IgA deposits along the dermoepidermal junction
• Linear IgA dennatosis of adults                   Linear deposits of IgA at dermoepidermal junction


Q. 5

The “dilapidated brick wall” appearance is classically seen in the histopathology of the following?

 A

Hailey Hailey disease

 B

Pemphigus vulgaris

 C

Darier’s disease

 D

Bullous pemphigoid

Q. 5

The “dilapidated brick wall” appearance is classically seen in the histopathology of the following?

 A

Hailey Hailey disease

 B

Pemphigus vulgaris

 C

Darier’s disease

 D

Bullous pemphigoid

Ans. A

Explanation:

Hailey Hailey disease/familial benign chronic pemphigus has full thickness partial acantholysis resulting in this appearance.

Ref: Rook Textbook of Dermatology, 8th Edition, Page 39.33.

Q. 6

A 30 year old pregnant woman presents to a physician with painful oral ulcers. Physical examination demonstrates widespread erosions of her mucous membranes. Close examination reveals a friable mucosa, but no well-defined aphthous ulcers. Biopsy of perilesional mucosa demonstrates acantholysis; direct immunofluorescence demonstrates an intraepidermal band of IgG and C3. Which of the following is the most likely diagnosis?

 A

Bullous pemphigoid

 B

Dermatitis herpetiformis

 C

Herpes simplex I

 D

Pemphigus vulgaris

Q. 6

A 30 year old pregnant woman presents to a physician with painful oral ulcers. Physical examination demonstrates widespread erosions of her mucous membranes. Close examination reveals a friable mucosa, but no well-defined aphthous ulcers. Biopsy of perilesional mucosa demonstrates acantholysis; direct immunofluorescence demonstrates an intraepidermal band of IgG and C3. Which of the following is the most likely diagnosis?

 A

Bullous pemphigoid

 B

Dermatitis herpetiformis

 C

Herpes simplex I

 D

Pemphigus vulgaris

Ans. D

Explanation:

This is pemphigus vulgaris, in which autoantibody directed against transmembrane cadherin adhesion molecules induced acantholysis (breakdown of epithelial cell-cell connections) with resulting intraepidermal blister formation.
It may develop spontaneously or following triggers such as drugs (thiols, penicillamine), physical injury (burns), cancer, pregnancy, other skin diseases, and emotional stress.
Pemphigus vulgaris is a relatively rare blistering disease; it is seen more commonly in patients with Jewish or Mediterranean heritage.
In addition to the usually prominent oral ulcers, uncomfortable skin erosions can also occur when the blisters rupture rapidly and are not observed. The epidermis at the edge of these erosions is often easily disrupted by sliding pressure (Nikolsky sign).
 
Bullous pemphigoid is characterized by deeper blisters, occurring at the dermal-epidermal junction.
 
Dermatitis herpetiformis is characterized by severe, intense pruritus and groups of papules and vesicles.
 
Herpes simplex I or II can show multinucleated giant cells on scrapings of the ulcer base.
 
Ref: Suurmond D. (2009). Section 34. Disorders of the Mouth. In D. Suurmond (Ed), Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 6e.

Q. 7

A 30 year old male presents with itchy papulo-vesicular lesions on the extremities, knees, elbows and buttocks since one year. Direct immunofluorescence staining of the lesions showed IgA deposition at dermoepidermal junction. Which of the following represents the most probable diagnosis?

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Dermatitis herpetiformis

 D

Nummular eczema

Q. 7

A 30 year old male presents with itchy papulo-vesicular lesions on the extremities, knees, elbows and buttocks since one year. Direct immunofluorescence staining of the lesions showed IgA deposition at dermoepidermal junction. Which of the following represents the most probable diagnosis?

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Dermatitis herpetiformis

 D

Nummular eczema

Ans. C

Explanation:

Patient is showing features of dermatitis herpetiformis.

Dermatitis herpetiformis is a chronic, intensely itchy blistering disease characterized by the presence of papules and vesicles occurring predominantly on the extensor surfaces of the body such as the elbows, knees, buttocks and scapula.

It is seen in association with gluten sensitive enteropathy.

Diagnosis is made by the presence of IgA deposits in the dermoepidermal junction by direct immunoflouresence examination of a perilesional or normal appearing skin biopsy sample. Treatment includes dapsone and gluten free diet.

Ref: NORD Guide to Rare Disorders: National Organization for Rare Disorders, Page 103; Atlas of Clinical Dermatology By Anthony Du Vivier, 3rd Edition, Pages 418, 426; Dermatology in Clinical Practice By S. W. Lanigan, Page 170


Q. 8

A 40 year old male developed persistant oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct immunofluorescence examination of a skin biopsy showed intercellular IgG deposits in the epidermis. The most probable diagnosis is:

 A

Pemphigus vulgaris

 B

Bullous Pemphigoid

 C

Bullous Lupus erythematosus

 D

Epidermolysis bullosa acquisita

Q. 8

A 40 year old male developed persistant oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct immunofluorescence examination of a skin biopsy showed intercellular IgG deposits in the epidermis. The most probable diagnosis is:

 A

Pemphigus vulgaris

 B

Bullous Pemphigoid

 C

Bullous Lupus erythematosus

 D

Epidermolysis bullosa acquisita

Ans. A

Explanation:

It is an autoimmune intraepidermal blistering disease due to antibodies against desmoglein 3.
It begins as painful oral erosions followed by flaccid blisters on the trunk, face, scalp.
 
Ref: Current Literature Dermatology By Pasricha, Pages 197, 202; Rook’s Textbook of Dermatology, 8th Edition.

Q. 9

Which of the following disorder is associated with acantholysis?

 A

Pemphigoid

 B

Pemphigus vulgaris

 C

Erythema multiforme

 D

Dermatitis herpetiformis

Q. 9

Which of the following disorder is associated with acantholysis?

 A

Pemphigoid

 B

Pemphigus vulgaris

 C

Erythema multiforme

 D

Dermatitis herpetiformis

Ans. B

Explanation:

Acantholysis refers to loss of cohesion between epidermal cells as a result of destruction of intercellular substance.

Acantholytic cells are round cells with large hyperchromatic nuclei and perinuclear halo.

The primary lesion of pemphigus vulgaris is acantholysis in the epidermis.

Its histologic hallmark is acantholysis which is suprabasal, and the basal cells remain attached to the basement membrane.

Ref: Tropical Dermatopathology By Singh, Page 121; Pathology of The Head and Neck By Antonio Cardesa, Page 74.


Q. 10

A 45 yr old female developed recurrent oral erosions followed by multiple flaccid bullae on trunk and extremities. A tzanck smear showed acantholytic cells and direct immunofluoroscence showed intercellular IgG deposits in the epidermis. The most probable diagnosis is:

 A

Bullous Pemphigoid

 B

Stevens Johnson syndrome

 C

Herpes simplex 1 infection

 D

Pemphigus vulgaris

Q. 10

A 45 yr old female developed recurrent oral erosions followed by multiple flaccid bullae on trunk and extremities. A tzanck smear showed acantholytic cells and direct immunofluoroscence showed intercellular IgG deposits in the epidermis. The most probable diagnosis is:

 A

Bullous Pemphigoid

 B

Stevens Johnson syndrome

 C

Herpes simplex 1 infection

 D

Pemphigus vulgaris

Ans. D

Explanation:

Recurrent, painful oral lesions and cutaneous lesions in the form of flaccid bullae are characteristic of phemphigus vulgaris

Positive Tzanck smear, IgG deposits on direct immunofluoroscence positive nikolsky’s sign are features of phemphigus vulgaris.

 
Ref: Textbook of Dermatology, Venereology and Leprology By Devinder Mohan Thappa, 2nd Edition, Chapter 12, Pages 152-53; Dermatology (A coloured Hand Textbook) By R. J. G. Rycroft, S. J. Robertson, Sarah H. Wakeli, 2nd Edition, Part 1, Pages 54, 55

Q. 11

Itching associated with linear IgA deposition in dermal papillae is a feature of:

 A

Bullous disease of childhood

 B

Lichenoid bullous disease

 C

Dermatitis herpetiformis

 D

Pemphigus vulgaris

Q. 11

Itching associated with linear IgA deposition in dermal papillae is a feature of:

 A

Bullous disease of childhood

 B

Lichenoid bullous disease

 C

Dermatitis herpetiformis

 D

Pemphigus vulgaris

Ans. C

Explanation:

The cutaneous lesions of Dermatitis herpetiformis (DH) in childhood resemble those in adult and are severely pruritic papulovesicles.

DH is associated with granular deposition of IgA antibody within the dermal papillae.

Ref: Cambridge Textbook of Accident and Emergency Medicine edited by David V. Skinner, Andrew Swain, Colin Robertson, J. W. Rodney Peyton, 1997, Page 1051


Q. 12

Which of the following drug is not associated with drug induced pemphigus?

 A

Rifampicin

 B

Penicillin

 C

Captopril

 D

Furosemide

Q. 12

Which of the following drug is not associated with drug induced pemphigus?

 A

Rifampicin

 B

Penicillin

 C

Captopril

 D

Furosemide

Ans. D

Explanation:

Penicillin, cephalosporins, penicillamine, rifampicin, captopril, piroxicam, phenylbutazone are drugs commonly associated with drug induced pemphigus.


Q. 13

Pemphigus vulgaris is characterized by all, except:

 A

Positive Nikolsky’s sign

 B

Oral erosions

 C

Subepidermal bulla

 D

Tzanck smear showing acantholytic cells

Q. 13

Pemphigus vulgaris is characterized by all, except:

 A

Positive Nikolsky’s sign

 B

Oral erosions

 C

Subepidermal bulla

 D

Tzanck smear showing acantholytic cells

Ans. C

Explanation:

Pemphigus vulgaris is an intraepidermal blistering disease.


Q. 14

Scenario: A 35 year old lady is diagnosed with Pemphigus Vulgaris by her dermatologist.
 
Assertion: Pemphigus vulgaris presents with flaccid blisters and oral erosions in a middle aged person.
 
Reason: It is a intraepidermal blistering disorder due to antibodies against desmogleins.
 A

Both Assertion and Reason are true, and Reason is the correct explanation for Assertion

 B

Both Assertion and Reason are true, and Reason is not the correct explanation for Assertion

 C

Assertion is true, but Reason is false

 D

Assertion is false, but Reason is true

Q. 14

Scenario: A 35 year old lady is diagnosed with Pemphigus Vulgaris by her dermatologist.
 
Assertion: Pemphigus vulgaris presents with flaccid blisters and oral erosions in a middle aged person.
 
Reason: It is a intraepidermal blistering disorder due to antibodies against desmogleins.
 A

Both Assertion and Reason are true, and Reason is the correct explanation for Assertion

 B

Both Assertion and Reason are true, and Reason is not the correct explanation for Assertion

 C

Assertion is true, but Reason is false

 D

Assertion is false, but Reason is true

Ans. A

Explanation:

Pemphigus vulgaris is an intraepidermal blistering disease due to antibodies against desmogleins (epidermal attachment complexes).

It  presents with flaccid blisters and oral erosions in a middle aged person.

The subepidermal blistering disorders present with tense blisters.

Ref: Rook’s Textbook of Dermatology, 8th Edition, Page 40.3

Q. 15

A person presents with hemorrhagic fluid in a tense blister at dermoepidermal junction. Most probable diagnosis is:

 A

Pemphigoid

 B

Pemphigus vulgaris

 C

Pemphigus vegetans

 D

Drug induced pemphigus

Q. 15

A person presents with hemorrhagic fluid in a tense blister at dermoepidermal junction. Most probable diagnosis is:

 A

Pemphigoid

 B

Pemphigus vulgaris

 C

Pemphigus vegetans

 D

Drug induced pemphigus

Ans. A

Explanation:

The pemphigoid group includes a series of chronic diseases in which blisters form within the lamina lucida of the basement membrane dermoepidermal junction).

The blister roof is thus the entire epidermis so it is stable.

Frequently the blisters are hemorrhagic, fluid filled and tense.

No accantholysis occurs, so the Tzanck test is negative.

Antibodies are directed against various components of the basement membrane zone. 

Characteristic of pemphigus vulgaris is an intradermal blister occurring immediately above the basal cell layer and associated with acantholytic cells.

The blisters rapidly ruptures, leaving non healing, painful erosions. 

Pemphigus vegetans usually presents clinically as serpiginous ulcers.

Microscopically, the epithelium tends to proliferate and become verruciform.

Acantholytic cells may not be conspicuous and eosinophil microabscesses are the most typical histological feature. 

Drug induced pemphigus can present as pemphigus foliaceous, pemphigus erythematosus, or as pemphigus vulgaris.

Moist erythematous crusted scaling plaques and superficial friable blisters are seen.

Common drugs responsible are thiol compounds, drugs having a sulph-hydryl group, penicillamine.


Q. 16

Fogo selvagem is a type of:

 A

Pemphigus vulgaris

 B

Pemghigus vegetans

 C

Pemphigus foliaceus

 D

Bullous pemphigoid

Q. 16

Fogo selvagem is a type of:

 A

Pemphigus vulgaris

 B

Pemghigus vegetans

 C

Pemphigus foliaceus

 D

Bullous pemphigoid

Ans. C

Explanation:

Endemic pemphigus foliaceus common in rural parts of South America, particularly certain states of Brazil is known as fogo selvagem (wild fire) caused by bite of black fly.

Ref: Rook’s textbook of dermatology, 8th edition, Pg 40.13


Q. 17

U-serrated pattern in direct immunofluorescence is seen in:

 A

Epidermolysis bullosa acquisita

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Dermatitis herpetiformis

Q. 17

U-serrated pattern in direct immunofluorescence is seen in:

 A

Epidermolysis bullosa acquisita

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Dermatitis herpetiformis

Ans. A

Explanation:

Epidermolysis bullosa acquisita: Direct immunofluorescence shows, linear IgG, Ig A, Ig M at the basement-membrane zone with diagnostic U-serrated pattern. Patients’ skin split through the lamina lucida with 1 mol/L salt demonstrates the IgG antibodies to be bound to the dermal aspect of the blister. N serrated pattern is seen in bullous pemphigoid. 

 
Ref: Rook’s textbook of dermatology, 8th edition, Pg 40.53.

Q. 18

Which of the following presents with a “string of pearl appearance”?

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Dermatitis herpetiformis

Q. 18

Which of the following presents with a “string of pearl appearance”?

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Dermatitis herpetiformis

Ans. C

Explanation:

The lesions in Linear IgA disease with a bimodal age distribution ie 60 yrs  comprise of urticated plaques and papules, and annular, polycyclic lesions often with blistering around the edge, the string of pearls sign/cluster of jewels sign.


Q. 19

A biopsy of affected skin in Pemphigus vulgaris would show which of the following?

 A

Acantholysis

 B

Balloon degeneration

 C

Reticular changes

 D

Spongiosis

Q. 19

A biopsy of affected skin in Pemphigus vulgaris would show which of the following?

 A

Acantholysis

 B

Balloon degeneration

 C

Reticular changes

 D

Spongiosis

Ans. A

Explanation:

The pathologic changes in pemphigus vulgaris are acantholysis, clefts, and blister formation in the intraepidermal areas.

Acantholysis is the separation of keratinocytes from one another.

The loss of cohesion or contact between cells begins with the detachment of tonofilaments from desmosomes.

Evidence indicates that an IgG autoantibody actually induces these changes.

Good to know:

  • Balloon degeneration and reticular changes are both histologic markers of herpes simplex viral-induced lesions.
  • Spongiosis is a general term referring to serous exudates between cells of the epidermis, with an inflammatory infiltrate in the dermis. This is frequently seen in dermatitis lesions.
Ref:Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L., Jameson J.L., Loscalzo J. (2012). Chapter 54. Immunologically Mediated Skin Diseases. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison’s Principles of Internal Medicine, 18e.

Q. 20

Which of the following tests would be MOST useful for confirmation of Pemphigus vulgaris?

 A

Bacterial culture

 B

Complete blood count

 C

Fungal culture

 D

Tissue biopsy with direct immunofluorescence

Q. 20

Which of the following tests would be MOST useful for confirmation of Pemphigus vulgaris?

 A

Bacterial culture

 B

Complete blood count

 C

Fungal culture

 D

Tissue biopsy with direct immunofluorescence

Ans. D

Explanation:

Direct immunofluorescence (DIF) is of great value in the early diagnosis of pemphigus vulgaris.

DIF shows intercellular IgG throughout the epidermis or the oral epithelium.

IgG is found in both involved and clinically normal skin in nearly all patients with pemphigus.

In acantholytic areas, C3 deposition is also reliably found. DIF results remain positive for a long time and may still be positive many years after clinical remission.

  • Bacterial culture is helpful in superinfected lesions, but does not help in confirming a diagnosis of pemphigus.
  • Complete blood count is helpful in infectious processes.
  • Fungal culture is used in tinea lesions.
Ref: Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L., Jameson J.L., Loscalzo J. (2012). Chapter 54. Immunologically Mediated Skin Diseases. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison’s Principles of Internal Medicine, 18e.

Q. 21

A 30 year old male presented with itchy, papulovesicular lesions in the extensor aspect of elbows and knees. Biopsy of the lesion showed dermoepidermal blister with micro-abscesses. Immunofluorescence showed deposits of IgA in the tips of dermal papillae. What is the MOST likely diagnosis?

 A

Bullous Impetigo

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Dermatitis herpetiformis

Q. 21

A 30 year old male presented with itchy, papulovesicular lesions in the extensor aspect of elbows and knees. Biopsy of the lesion showed dermoepidermal blister with micro-abscesses. Immunofluorescence showed deposits of IgA in the tips of dermal papillae. What is the MOST likely diagnosis?

 A

Bullous Impetigo

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Dermatitis herpetiformis

Ans. D

Explanation:

sensitive enteropathy. In this condition absorption of gluten induces the formation of circulating immune complexes which deposit in the dermal papillae causing inflammation and dermoepidermal split. 

 
Skin lesions are in the form of extremely itchy grouped papulovesicular lesions that develop on normal or erythematous skin.
The most frequently involve the extensors(elbow, knee, buttocks, shoulder and sacral area).
 Biopsy shows: dermoepidermal blister with collection of polymorphs at the tip of dermal papillae.
Immunofluorescence showed deposits of IgA in the tips of dermal papillae.
Serology: Anti endomysial and anti reticulin antibodies are present in most patients.
 
Ref: Illustrated Synopsis Of Dermatology & Sexually Transmitted Diseases (3Nd  By Khanna page 71.

Q. 22

Intraepidermal bullae formation occur in which of the following condition?

 A

Bullous Impetigo

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Dermatitis herpetiformis

Q. 22

Intraepidermal bullae formation occur in which of the following condition?

 A

Bullous Impetigo

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Dermatitis herpetiformis

Ans. C

Explanation:

Pemphigus vulgaris is an intraepidermal bullous disorder where the split is suprabasal.
Pemphigus is an autoimmune disorder characterised by the production of IgG autoantibodies against desmoglein which are involved in intercellular adhesion.
These antibodies induce keratinocytes to release serine proteases which dissolve the intercellular substance.
 
The site of split in 4 main variants of pemphigus are:
  • Pemphigus vulgaris: split is suprabasal
  • Pemphigus vegetans: split is suprabasal
  • Pemphigus foliaceus: blister is either in the granular layer or just below the horny layer
  • Pemphigus erythematosus: blister is either in the granular layer or just below stratum corneum
Ref: Illustrated Synopsis Of Dermatology & Sexually Transmitted Diseases By Khanna page 64.

Q. 23

A 60 year old male presents to the dermatologist with a complaint of tender blisters on his arm and flank. Physical examination reveals blisters and flaccid bullae; a few have ruptured, leaving red, sore, denuded areas. Which of the following findings would suggest the diagnosis of pemphigus vulgaris as opposed to bullous pemphigoid?

 A

Eosinophils within bullae

 B

IgA deposits on basement membrane

 C

Negative Nikolsky sign

 D

Oral mucosal lesions

Q. 23

A 60 year old male presents to the dermatologist with a complaint of tender blisters on his arm and flank. Physical examination reveals blisters and flaccid bullae; a few have ruptured, leaving red, sore, denuded areas. Which of the following findings would suggest the diagnosis of pemphigus vulgaris as opposed to bullous pemphigoid?

 A

Eosinophils within bullae

 B

IgA deposits on basement membrane

 C

Negative Nikolsky sign

 D

Oral mucosal lesions

Ans. D

Explanation:

 
Pemphigus vulgaris is associated with chronic, severe bullae formation on the skin and oral mucosa.
Mucosal lesions are extremely rare in bullous pemphigoid.
This can be used clinically to guide therapy, although a skin biopsy should be taken to confirm the diagnosis.
Both diseases are characterized by formation of tender bullae that can rupture,leaving red, raw areas.
Pemphigus vulgaris patients eventually became febrile and lose weight, and if untreated, most will die within one year.
Bullous pemphigoid lesions tend to heal and the patients do very well. This prognostic difference is an important distinction in dermatological medicine.
 
Eosinophils within blisters provide an important clue supporting bullous pemphigoid as the diagnosis which must be ascertained with histologic examination. The vesicles in pemphigus vulgaris mostly contain rounded acantholytic keratinocytes “floating” within.
 
IgA deposits seen with immunofluorescence on the basement membrane is characteristic of a third bullae-forming disease known as dermatitis herpetiformis. Vesicles form between the dermal-epidermal junction. The disease is associated with gluten sensitivity and celiac disease.
 
IgG autoantibody activity is common to both pemphigus vulgaris and bullous pemphigoid.
 
Ref: Payne A.S., Stanley J.R. (2012). Chapter 54. Pemphigus. In K. Wolff (Ed),Fitzpatrick’s Dermatology in General Medicine, 8e.

Q. 24

‘Bulla spread sign’ is seen in :

 A

Herpes gestational

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Herpes simplex

Q. 24

‘Bulla spread sign’ is seen in :

 A

Herpes gestational

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Herpes simplex

Ans. C

Explanation:

In the traditional “bulla spread” sign or Lutz sign, the margin of an intact bullae is first marked by a pen.
Slow, careful and unidirectional pressure applied by a finger to the bulla causes peripheral extension of the bulla beyond the marked margin.
The bulla thus extended has an irregular angulated border in pemphigus vulgaris, while a regular rounded border is observed in bullous pemphigoid or other subepidermal blistering disorders.
 
Ref :Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L., Jameson J.L., Loscalzo J. (2012). Chapter 54. Immunologically Mediated Skin Diseases. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison’s Principles of Internal Medicine, 18e.

Q. 25

A female presented with a skin lesions all over the body which shows positive ‘bulla spread sign’. It is characteristic of:

 A

Herpes gestationis

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Herpes simplex

Q. 25

A female presented with a skin lesions all over the body which shows positive ‘bulla spread sign’. It is characteristic of:

 A

Herpes gestationis

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Herpes simplex

Ans. C

Explanation:

Pemphigus vulgaris is characterized by cutaneous and mucosal blisters.
On application of tangential pressure on normal skin results in formation of new bulla (Nikolsky sign) or if applied to pre-existing bulla results in the spread of bulla (bulla spread sign).
Cutaneous lesions predominantly present on face, trunk, axillae, groins, and scalp.
 
Ref: Illustrated Synopsis Of Dermatology & Sexually Transmitted Diseases By Khanna, 3rd edition, Page 64.

Q. 26

In which of the following are linear IgA deposits in mesangium noted –

 A

Henoch Schonlein purpura

 B

Malaria

 C

Good Pasture’s syndrome

 D

Wegener’s granulomatosis

Q. 26

In which of the following are linear IgA deposits in mesangium noted –

 A

Henoch Schonlein purpura

 B

Malaria

 C

Good Pasture’s syndrome

 D

Wegener’s granulomatosis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Henoch Schonlein Purpura


Q. 27

Acantholytic cells in Pemphigus are derived from ‑

 A

Stratum granulosum

 B

Stratum basale

 C

Langerhans cell

 D

Stratum spinosum

Q. 27

Acantholytic cells in Pemphigus are derived from ‑

 A

Stratum granulosum

 B

Stratum basale

 C

Langerhans cell

 D

Stratum spinosum

Ans. D

Explanation:

Ans. is ‘d’ i.e., Stratum spinosum

o First know about the layers of skin

A.  Epidermis (from superficial to deep) –

Stratum corneum (Horny layer)

Stratum granulosum (granular cells layer)

Stratum spinosum (Prickle cell layer)

Stratum basale (Basal cell layer)

B.  Dermis

Papillary dermis

Reticular dermis

o Pemphigus is characterized by acantholysis. Acantholysis is separation of epidermal cells from one another. This separation occurs due to antibodies (IgG) against the intercellular substances.

Acantholysis occurs in different layers of epidermis in different types of pemphigus.

o Pemphigus vulgaris and —–> Acantholysis occur in between basal cells and cells of prickle cell layer (Stratum pemphigus vegetans spinosum). Some books also state that acantholysis occurs in suprabasal layer. Suprabasal layer is most probably, stratum spinosum as it is seen above basal layer.

 Pemphigus foliaceous and  —–> Acantholysis occurs in stratum granulosum.

pemphigus erythematous

So, Stratum spinosum is involved in pemphigus vulgaris and pemphigus vegetans and stratum granulosum is involved in pemphigus foliaceous and pemphigus erythematosus.

But, the answer should be stratum spinosum, as pemphigus vulgaris is the most common type of pemphigus and most probably they have asked about it.


Q. 28

‘Row of tombstones ‘ appearance is seen in

 A

Irritant dermatitis

 B

Pemphigus

 C

Pemphigoid

 D

Harpes zoster

Q. 28

‘Row of tombstones ‘ appearance is seen in

 A

Irritant dermatitis

 B

Pemphigus

 C

Pemphigoid

 D

Harpes zoster

Ans. B

Explanation:

Ans. is ‘b’ i.e., Pemphigus

o The Suprabasal acantholytic blister is characteristic of pemphigus vulgaris. The single layer of intact basal cells that forms the blister base has been likened to a row of tombstones.


Q. 29

Subepidermal lesion are

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Hailey-Hailey disease

 D

Darier’s disease

Q. 29

Subepidermal lesion are

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Hailey-Hailey disease

 D

Darier’s disease

Ans. A

Explanation:

A i.e. Bullous pemphigoid


Q. 30

Subepithelial Bullae are seen in:

 A

Dermatitis herpatiforms

 B

Molluscum contagiosum

 C

Pemphigus

 D

All

Q. 30

Subepithelial Bullae are seen in:

 A

Dermatitis herpatiforms

 B

Molluscum contagiosum

 C

Pemphigus

 D

All

Ans. A

Explanation:

A i.e. Dermatitis herpetiformis


Q. 31

Subepidermal bulla are seen in –

 A

Pemphigoid

 B

Pemphigus

 C

Pityriasis rosea

 D

Psoriasis

Q. 31

Subepidermal bulla are seen in –

 A

Pemphigoid

 B

Pemphigus

 C

Pityriasis rosea

 D

Psoriasis

Ans. A

Explanation:

A. i.e. Pemphigoid


Q. 32

Sub-epidermal splitting is not found in:

 A

Bullous pemphigoid

 B

Pemphigus foliaceus

 C

Dermatitis herpetiformis

 D

Burns

Q. 32

Sub-epidermal splitting is not found in:

 A

Bullous pemphigoid

 B

Pemphigus foliaceus

 C

Dermatitis herpetiformis

 D

Burns

Ans. B

Explanation:

B i.e. Pemphigus foliaceous


Q. 33

Subepidermal bistreing is seen in all except ‑

 A

Pemphigus vulgaris

 B

Dermatitis herpetiformis

 C

Toxic epidermal necrolysis

 D

Pemphigus

Q. 33

Subepidermal bistreing is seen in all except ‑

 A

Pemphigus vulgaris

 B

Dermatitis herpetiformis

 C

Toxic epidermal necrolysis

 D

Pemphigus

Ans. D

Explanation:

Ans:D. i.e. Pemphigus 

  • Causes of Subepidermal Blistering includes :Bullous pemphigoid, dermatitis herpetiformis, erythema multiforme, TEN, friction blisters.
  • Causes of Intraepidermal Blistering includes :Acute eczema, varicella, herpes simplex, pemphigus

Q. 34

Intra epidermal blisters are seen in:

 A

Bullous pemphigoid

 B

Pemphigus folliaceous

 C

Dermatitis herpeteformis

 D

Bullous SLE

Q. 34

Intra epidermal blisters are seen in:

 A

Bullous pemphigoid

 B

Pemphigus folliaceous

 C

Dermatitis herpeteformis

 D

Bullous SLE

Ans. B

Explanation:

B i.e. Pemphigus folliaceous


Q. 35

Intra-epidermal acantholytic (blisters) vesicles are seen in

 A

Pemphigus vulgaris

 B

Carcinomatous (paraneoplastic) pemphigus

 C

Dermatitis herpetiformis

 D

a and b

Q. 35

Intra-epidermal acantholytic (blisters) vesicles are seen in

 A

Pemphigus vulgaris

 B

Carcinomatous (paraneoplastic) pemphigus

 C

Dermatitis herpetiformis

 D

a and b

Ans. D

Explanation:

A & B i.e. (Pemphigus vulgaris) & (Carcinomatous pemphigus)


Q. 36

Acantholytic cell in pemphigus is derived from :

 A

Stratum granulosum

 B

Stratum basale

 C

Stratum spinosum

 D

Langerhan’s cell

Q. 36

Acantholytic cell in pemphigus is derived from :

 A

Stratum granulosum

 B

Stratum basale

 C

Stratum spinosum

 D

Langerhan’s cell

Ans. C

Explanation:

C i.e. Stratum spinostun

Pemphigus (vulgarisQ, vegetans, foliaceus, erythematous, endemic, paraneoplasticQ) causes intra epidermalQ and PemphigoidQ (bullous, cicatricial, gestations, linear IgA disease) causes sub epidermal bullae formation(2. Blisters (vesicles, bullae or split) are intra epidermal in pemphigus and subepidermal in pemphigoid and dermatitis herpetiformisQ


Q. 37

Acantholysis is characteristic of:

 A

Pemphigus vulgaris

 B

Pemphigoid

 C

Erythema multoforme

 D

Dermatitis herpetiformis

Q. 37

Acantholysis is characteristic of:

 A

Pemphigus vulgaris

 B

Pemphigoid

 C

Erythema multoforme

 D

Dermatitis herpetiformis

Ans. A

Explanation:

A i.e. Pemphigus Vulgaris


Q. 38

In pemphigus vulgaris, antibodies are present against:

 A

Basement member

 B

Intercellular substance

 C

Keratin

 D

Cell nucleus

Q. 38

In pemphigus vulgaris, antibodies are present against:

 A

Basement member

 B

Intercellular substance

 C

Keratin

 D

Cell nucleus

Ans. B

Explanation:

B. i.e. Intercellular substance


Q. 39

Acantolytic cells in pemphigus is-

 A

Cell with hyperchromatic nuclei and perinuclear halo

 B

Cell with hypochromatic nuclei and perinuclear halo

 C

Multinucleated cells

 D

None of the above

Q. 39

Acantolytic cells in pemphigus is-

 A

Cell with hyperchromatic nuclei and perinuclear halo

 B

Cell with hypochromatic nuclei and perinuclear halo

 C

Multinucleated cells

 D

None of the above

Ans. A

Explanation:

A. i.e. Cells with hyper chromatic nuclei & perinuclear halo

Acantholysis is seen in epidermis as loss of cohesion between epidermal cells d/t lysis of intercellular substance (intercellular adhesion sitesQ). Acantholytic cells are rounded keratinocytes with hyperchromatic nuclei & perinuclear haloQ. Acantholysis is seen in pemphigus, Darier’s disease, Grover’s disease and Hailey-Hailey disease (but not in pemphigoid) Q.

Primary acantholysis (i.e. acantholysis is primary pathological change) is seen in pemphigus variants (vulgaris, vegetans, paraneoplastic, foliaceous, erythematosus), Hailey-Hailey disease (familial benign chronic pemphigus), Darrier’s disease (Keratosis follicularis), Grover’s disease (transient acantholytic dermatosis), persistent acantholytic dermatosis, warty dyskeratoma, and eosinophilic spongiosis. Acantholysis (i.e. loss of cohesion b/w keratinocytes d/t breakdown of intercellular bridges) result in formation of intraepidermal clefts, vesicles & bullae.

Secondary acantholysis (i.e. secondary to some other pathological change like damage in epidermal cells) occurs in bullous impetigo, SSSS, viral disorders, solar keratosis & squamous cell carcinoma.


Q. 40

Nikolsky sign is positive in all of the following except:

 A

Staphylococcal scalded skin syndrome

 B

Toxic epidermonecrolysis

 C

Bullous pemphigoid

 D

Pemphigus

Q. 40

Nikolsky sign is positive in all of the following except:

 A

Staphylococcal scalded skin syndrome

 B

Toxic epidermonecrolysis

 C

Bullous pemphigoid

 D

Pemphigus

Ans. C

Explanation:

C. i.e. Bullous pemphigoid


Q. 41

Nikolsky sign is seen in –

 A

Pemphigus vulgaris

 B

Herpes zoster

 C

Herpes simlex

 D

All

Q. 41

Nikolsky sign is seen in –

 A

Pemphigus vulgaris

 B

Herpes zoster

 C

Herpes simlex

 D

All

Ans. D

Explanation:

D. i.e. All


Q. 42

Nikolsky sign not present in:

 A

Pemphigus

 B

Pemphigoid

 C

Vitiligo

 D

c and b

Q. 42

Nikolsky sign not present in:

 A

Pemphigus

 B

Pemphigoid

 C

Vitiligo

 D

c and b

Ans. D

Explanation:

C. i.e. Vitiligo > B Pemphigoid


Q. 43

An auto immune disease is:

 A

Pemphigus Vulgaris

 B

Psoriasis

 C

Lichen Planus

 D

Acne Vulgaris

Q. 43

An auto immune disease is:

 A

Pemphigus Vulgaris

 B

Psoriasis

 C

Lichen Planus

 D

Acne Vulgaris

Ans. A

Explanation:

A i.e. Pemphigus vulgaris

Disease

Etiology

Pemphigus

Pemphigoid, Epidermolysis bullosa acquisita

Autoinimune(2

Lichen planus

Inflamatory disorder of unknown origin

Acne vulgaris

Obstruction of pilo-sebaceous gland.Q

Epidermolysis bullosa congenita

Psoriasis

Genetic diseaseQ of unknown origin

Genetic dsQ unknown (inflamatory) origin


Q. 44

Genetic predisposition is seen in which disease:

 A

Lichen planus

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Epidermolysis Bullosa

Q. 44

Genetic predisposition is seen in which disease:

 A

Lichen planus

 B

Bullous pemphigoid

 C

Pemphigus vulgaris

 D

Epidermolysis Bullosa

Ans. D

Explanation:

D i.e. Epidermolysis bullosa


Q. 45

Hailey – hailey disease is:

 A

Benign familial chronic pemphigus

 B

Pemphigus acutus

 C

Pemphigus

 D

Lyell’s syndrome

Q. 45

Hailey – hailey disease is:

 A

Benign familial chronic pemphigus

 B

Pemphigus acutus

 C

Pemphigus

 D

Lyell’s syndrome

Ans. A

Explanation:

A. i.e. Benign Familial Chronic Pemphigus


Q. 46

Commonest/rarest veriety of Pemphigus‑

 A

Pemphigus vulgaris/vegetans

 B

Pemphigus vegetans/vulgaris

 C

Pemphigus fliaceus/erythematosis

 D

Pemphigus erythematosia/foliaceous

Q. 46

Commonest/rarest veriety of Pemphigus‑

 A

Pemphigus vulgaris/vegetans

 B

Pemphigus vegetans/vulgaris

 C

Pemphigus fliaceus/erythematosis

 D

Pemphigus erythematosia/foliaceous

Ans. A

Explanation:

A. i.e. Pemphigus vulgaris/vegetans


Q. 47

‘Row of tombstones’ appearance is seen in :

 A

Irritant dermatitis

 B

Pemphigus

 C

Pemphigoid

 D

Harpes zoster

Q. 47

‘Row of tombstones’ appearance is seen in :

 A

Irritant dermatitis

 B

Pemphigus

 C

Pemphigoid

 D

Harpes zoster

Ans. B

Explanation:

B. i.e. Pemphigus


Q. 48

In pemphigus vulgaris, antibodies are present against 

 A

Basement membrane

 B

Intercellular substance

 C

Cell nucleus

 D

Keratin

Q. 48

In pemphigus vulgaris, antibodies are present against 

 A

Basement membrane

 B

Intercellular substance

 C

Cell nucleus

 D

Keratin

Ans. B

Explanation:

B i.e. Intercellular substance


Q. 49

True about pemphigus vulgaris A/E:

 A

Subepidermal

 B

Autoimmune disease

 C

Tzanck smear shows acanthoyltic cells

 D

Antibody are formed against desmogleins

Q. 49

True about pemphigus vulgaris A/E:

 A

Subepidermal

 B

Autoimmune disease

 C

Tzanck smear shows acanthoyltic cells

 D

Antibody are formed against desmogleins

Ans. A

Explanation:

A i.e. Subepidermal


Q. 50

A patient with Bullous eruption on lower limb and trunk, biopsy show epidermal bullae. The correct diagnosis is:

 A

Pemphigoid

 B

Pemphigus Vulgaris

 C

Impetigo

 D

Internal Malignany

Q. 50

A patient with Bullous eruption on lower limb and trunk, biopsy show epidermal bullae. The correct diagnosis is:

 A

Pemphigoid

 B

Pemphigus Vulgaris

 C

Impetigo

 D

Internal Malignany

Ans. B

Explanation:

B i.e. Pemphigus vulgaris


Q. 51

A 24 years old (middle aged) female has flaccid bullae in the skin and persistent painful oral erosions (palatal & vestibular lesions in buccal mucosa). Histopathology shows intraepidermal acantholytic blister. The most likely diagnosis is

 A

Bullous Pemphigoid.

 B

Erythema multiforme

 C

Pemphigus vulgaris.

 D

Dermatitis herpetiformis.

Q. 51

A 24 years old (middle aged) female has flaccid bullae in the skin and persistent painful oral erosions (palatal & vestibular lesions in buccal mucosa). Histopathology shows intraepidermal acantholytic blister. The most likely diagnosis is

 A

Bullous Pemphigoid.

 B

Erythema multiforme

 C

Pemphigus vulgaris.

 D

Dermatitis herpetiformis.

Ans. C

Explanation:

C i.e. Pemphigus vulgaris


Q. 52

A 40 year old male developed persistant oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct examination of a skin biopsy immunoflurescence showed intercellular IgG deposits in the epidermis. The most probable diagnosis is: 

 A

Pemphigus vulgaris

 B

Bullous Pemphigoid

 C

Bullous Lupus erythematosus

 D

Epidermolysis bullosa acquisita

Q. 52

A 40 year old male developed persistant oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct examination of a skin biopsy immunoflurescence showed intercellular IgG deposits in the epidermis. The most probable diagnosis is: 

 A

Pemphigus vulgaris

 B

Bullous Pemphigoid

 C

Bullous Lupus erythematosus

 D

Epidermolysis bullosa acquisita

Ans. A

Explanation:

A i.e. Pemphigus Vulgaris

– Pemphigus vulgaris is the commonest and pemphigus vegetans is the rarest variety.

In pemphigus vulgaris (an auto immune disease), autoantibodies (IgG) are directed against intercellular substance desmogleins (Dsgs)Q, a transmembrane desmosomal glycoprotein that belong to cadherin supergene family of calcium dependent adhesion molecules causing loss of cohesion (acantholysis)Q between epidermal cells of deep stratum basal layer Q. That is why, histology (tzanck smear) show (intra) epidermal acantholytic bullae (blister) in deep supra basal layer of epidermisQ along with single layer of intact stratum basale cells attached to basement membrane (row of tomb stone appearance) that form the base of blister. Indirect immuno fluorescence microscopy demonstrate circulating autoantibodies against keratinocyte cell surface antigen whereas diagnostic (gold standard) direct immunofluorescence microscopy (DIM) show deposits of IgG on the surface keratinocyte in fishnet like patternQ and in intercellular space (causing it to widen).

Pemphigus vulgaris presents as muco-cutaneous lesions (blister or erosions) predominantly in middle aged patients (> 40 year age). It typically begins on mucosal surface and nearly all patients have mucosal involvement (oral in 70% Q >> ocular, nasal, pharyngeal, larynx, oesophagus, urethra, vulva, cervix). PV

often progresses to widespread skin involvement with a predilection for scalp, face, neck, axillae, groin, trunk Q and pressure points.

PV is characterized by painful, mostly non pruritic, fragile flaccid blistersQ filled with clear fluid on a normal or erythematous base that ruptures to produce extensive denudation of mucous membranes and

skin resulting in ill defined irregular shaped erosions. Healing occurs without scarring but with temporary hyperpigmentation.


Q. 53

A 50 year old male known case of myasthe-nia with erythemated shallow erosions with few blisters and scales. Oral mucosa is not involved. Immunopathology demon­strates IgG deposition on keratinocytes and auto antibodies against Dsg -1. The diagnosis is

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus foliaceus

 D

Dermatitis herpetiformis

Q. 53

A 50 year old male known case of myasthe-nia with erythemated shallow erosions with few blisters and scales. Oral mucosa is not involved. Immunopathology demon­strates IgG deposition on keratinocytes and auto antibodies against Dsg -1. The diagnosis is

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus foliaceus

 D

Dermatitis herpetiformis

Ans. C

Explanation:

C i.e. Pemphigus foliaceus

Pemphigus foliaceous (PF) is distinguished from PV by following features‑

Distribution of lesion in both is much the same (i.e. seborrheic distribution including face, scalp and upper trunk) except that in PF the mucous membranes are almost always sparedQ.

–  Characteristic clinical lesions of PF are well demarcated, shallow erosions on an erythematous base with scales, crust formation, erythemaQ and sometimes surrounded with small vesicles along the borders. Patients often complain of both pain & burning in the leions. Eventually patient becomes erythrodermic with crusted, oozing & red skin.

Sun exposure (UV rays), environmental stimuli (insect bites), auto immune diseases particularly thymoma &/or myasthenia gravisQ, and drug exposure (eg drug containing thiol group like penicillamine, captopril, enalapril and non thiol drugs like penicillins, cephalosporins and piroxicams) may be associated and aggrevating factor.

PF is generally less severe and carries better prognosis than PV.

Histology (tzank smear) shows superficial acantholytic blisters just below stratum corneum and in granular layer. And DIM demonstrates autoantibodies against Dsg1Q. Because of lack of mucosal involvement autoantibodies against Dsg3 are not foundQ. Because of superifical cleavage, the small flaccid blisters rupture easily and PF rarely demonstrate intact blisters.


Q. 54

Drug induced pemphigus is seen in all except

 A

Penicillin

 B

Phenopthelein

 C

Iodine

 D

Frusemide

Q. 54

Drug induced pemphigus is seen in all except

 A

Penicillin

 B

Phenopthelein

 C

Iodine

 D

Frusemide

Ans. D

Explanation:

D i.e. Frusemide

–  Induced pemphigus is induced by radiotherapy (PV & PF ), thermal burns and electrical injury (PV), vegetables of Allium group, Human herpes virus 8 DNA and drugs.

–  Drug induced pemphigus usually resembles PF (>PV) and is caused by thiol (sulphydryl) and non thiol containing drugs via biochemical and immunological mechanisms respectively. The sulfhydryl group in desmoglein 1,3 becomes more antigenic because of interference of sulfhydryl group of thiol drugs. So the prognosis is better upon drug withdrawl of thiol group drugs.

Drugs containing thiol (sulphydryl) group such as penicillamine, captopril, enalaprilQ are most commonly associated with drug induced pemphigus (Harrison). Non thiol drugs linked to pemphigus include – penicillinsQ, cephalosporins, piroxicam, pyrazolam derivatives (eg. phenylbutazone), propranolol, chloroquine, hydroxy chloroquine, rifampicin, montelukast, interferon, other ACE inhibitors eg enalapril, ramapril, fosinopril and angiotension II receptor blockers condesartan and telmisartan (Rook’s). Iodine (radioactive & non radioactive) and phenopthaleinQ may also lit pemphigus.


Q. 55

All are asociated with pemphigus except:

 A

Thymoma

 B

CLL

 C

Myasthenia gravis

 D

Atrophic gastritis

Q. 55

All are asociated with pemphigus except:

 A

Thymoma

 B

CLL

 C

Myasthenia gravis

 D

Atrophic gastritis

Ans. D

Explanation:

Di.e. Atropine gastritis

Paraneoplastic pemphigus may be associated with non Hodgkin’s lymphomaQ (NHL), Chronic lymphocytic leukemiaQ (CLL), Castleman’s disease, thymomaQ and spindle cell tumors. And pemphigus folliaceus is associated with thymoma & /or mysthenia gravisQ.


Q. 56

Mucous lesions are seen in :

 A

Sec. syphilis

 B

Dermatitis herpetiformis

 C

Psoriasis

 D

Pemphigus

Q. 56

Mucous lesions are seen in :

 A

Sec. syphilis

 B

Dermatitis herpetiformis

 C

Psoriasis

 D

Pemphigus

Ans. D

Explanation:

D i.e. Pemphigus

Mucosa involvement is seen in Behcet’s syndrome, reactive arthritis (Reiter’s syndrome), Peutz Jegher syndromeQ, erythema multiforme major, Steven Johnson syndrome, TEN, lichen planusQ, lupus erythematousus, leprosy, measles (Koplik spots) Q, Fordyce spot & disease, syphilis (congenital and secondary), inflammatory bowel disease, acute HIV infection and pemphigus vulgarisQ.

Pemphigus folicaeus and bullous pemphigoid do not involve mucous membraneQ. However, • ocular/oral/scarring/cicatrical/benign mucosal/or mucous membrane – pemphigoid or bursting perry pemphigoid involve mucosa.


Q. 57

A 56 year old male lallu presents with painful bullous lesion in lower extrimity, the most likely diagnosis is

 A

Pemphigus Vulgaris

 B

Bullous pemphigoid

 C

Necrotic Pemphigus

 D

Contact eczema

Q. 57

A 56 year old male lallu presents with painful bullous lesion in lower extrimity, the most likely diagnosis is

 A

Pemphigus Vulgaris

 B

Bullous pemphigoid

 C

Necrotic Pemphigus

 D

Contact eczema

Ans. B

Explanation:

B i.e. Bullous pemphigoid


Q. 58

A 85 year old woman with Nikolsky sign-ve, blisters on thigh & trunk, lesions come on & off. What is the cause :

 A

Pemphigus vulgaris

 B

Pemphigoid

 C

Lichen planus

 D

Dermatitis herpetiformis

Q. 58

A 85 year old woman with Nikolsky sign-ve, blisters on thigh & trunk, lesions come on & off. What is the cause :

 A

Pemphigus vulgaris

 B

Pemphigoid

 C

Lichen planus

 D

Dermatitis herpetiformis

Ans. B

Explanation:

B i.e. Pemphigoid 


Q. 59

Inter cellular IgG deposition in epidermis is seen in:

 A

Pemphigus

 B

Sub corneal pustular dermatosis

 C

Bullus pemphigoid

 D

Dermatitis Herpetiformis

Q. 59

Inter cellular IgG deposition in epidermis is seen in:

 A

Pemphigus

 B

Sub corneal pustular dermatosis

 C

Bullus pemphigoid

 D

Dermatitis Herpetiformis

Ans. A

Explanation:

A. i.e. Pemphigus


Q. 60

Direct immunofluorescence is positive in

 A

Atopic dermatitis

 B

SLE

 C

Pemphigus

 D

c and b

Q. 60

Direct immunofluorescence is positive in

 A

Atopic dermatitis

 B

SLE

 C

Pemphigus

 D

c and b

Ans. D

Explanation:

C. i.e. Pemphigus > B. i.e. SLE


Q. 61

A 40 year old male had multiple blisters over the trunk & Extremities. Direct Immuno fluoresce studies showed linear IgG deposits along the Basement membrane, which of the following is the most likely diagnosis.

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus foliaceous

 D

Dermatitis herpetiformis

Q. 61

A 40 year old male had multiple blisters over the trunk & Extremities. Direct Immuno fluoresce studies showed linear IgG deposits along the Basement membrane, which of the following is the most likely diagnosis.

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus foliaceous

 D

Dermatitis herpetiformis

Ans. B

Explanation:

B i.e. Bullous pemphigoid


Q. 62

All are true about linear IgA disease except

 A

Subepidermal involvement

 B

Severe itching

 C

Granular deposition of IgA

 D

All

Q. 62

All are true about linear IgA disease except

 A

Subepidermal involvement

 B

Severe itching

 C

Granular deposition of IgA

 D

All

Ans. C

Explanation:

C i.e. Granular deposition of IgA

Linear IgA disease presents with severely pruritic, subepidermal blistersQ. On DIM, linear (not granular) deposits of IgAQ is seen along EBM. It is a separate & distinct entity different from dermatitis herpetiformis and so gluten free diet is not requiredQ.


Q. 63

Acantholysis is seen in:

March 2012

 A

Psoriasis

 B

Pemphigus vulgaris

 C

Lichen planus

 D

Pityriasis versicolor

Q. 63

Acantholysis is seen in:

March 2012

 A

Psoriasis

 B

Pemphigus vulgaris

 C

Lichen planus

 D

Pityriasis versicolor

Ans. B

Explanation:

Ans: B i.e. Pemphigus vulgaris

Dermatologic conditions and features

  • Psoariasis is a chronic dermatosis characterized by presence at typical sites of well defined erythematous papules and plaques, which are surmounted with large, silvery loose scales.
  • Pemphigus is an intraepidermal bullous disorder, which is characterized clinically by presence of cutaneous and mucosal blisters and histologically by acantholysis (due to deposition of intercellular antibodies)
  • Lichen planus is characterized by 5P’s; Pruritic, polygonal, purple (violaceous), plane (flat topped), papules with Wickham’s striae on surface
  • Pityriasis versicolor features include perifollicular, hypopigmented (or hyperpigmented), macules surmounted with branny scales

Q. 64

Most common type of pemphigus in India is:

September 2008

 A

P. foliaceus

 B

P. vulgaris

 C

P. erythematosus

 D

P. vegetans

Q. 64

Most common type of pemphigus in India is:

September 2008

 A

P. foliaceus

 B

P. vulgaris

 C

P. erythematosus

 D

P. vegetans

Ans. B

Explanation:

Ans. B: P.vulgaris

P.vulgaris is commonest variant of pemphigus.

There are flaccid bullae which ruptures to form painful erosions which take long time to heal. Oral erosions universally present.


Q. 65

Which of the following is the histological feature of pemphigus:          

March 2010

 A

Acanthosis

 B

Colloid bodies

 C

Acantholysis

 D

Basal cell degeneration

Q. 65

Which of the following is the histological feature of pemphigus:          

March 2010

 A

Acanthosis

 B

Colloid bodies

 C

Acantholysis

 D

Basal cell degeneration

Ans. C

Explanation:

Ans. C: Acantholysis

Pemphigus:

  • Epidemiology: Rare, equal in men and women, onset at 40 to 60 years of age, more common in Jewish
  • Clinical features: Flaccid blisters or crushed erosions, located on head, upper trunk, intertriginous areas, and mucosa; often begins on oral mucosa, may lead to hoarseness; skin is usually painful and Nikolsky’s sign positive
  • Diagnosis:

– Light microscopy: suprabasilar blister with acantholysis

– Direct Immunoflorescence (DIF): intercellular IgG and C3

– Biopsy of the margin of a bulla, when examined by light microscopy, will reveal a suprabasilar blister with acantholysis.

The acantholysis is caused by a loss of cohesion between cells in the lower epidermis, resulting in the formation of a blister just above the basal cell layer

Acanthosis is diffuse epidermal hyperplasia. It is not to be confused with acantholysis.


Q. 66

A 65-year old male presented with tense blisters associated with itching on normal looking skin with urticarial plaques as seen in the figure. What is the most likely diagnosis?

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Dermatitis herpetiformis

 D

IgA pemphigus

Q. 66

A 65-year old male presented with tense blisters associated with itching on normal looking skin with urticarial plaques as seen in the figure. What is the most likely diagnosis?

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Dermatitis herpetiformis

 D

IgA pemphigus

Ans. A

Explanation:

Ans. a. Bullous pemphigoid


Q. 67

Flaccid bullae with mucosal involvement and intraepidermal acantholysis are characteristic of‑

 A

Pemphygus vulgaris

 B

Pemphigus foliaceous

 C

Psoriasis

 D

Vitiligo

Q. 67

Flaccid bullae with mucosal involvement and intraepidermal acantholysis are characteristic of‑

 A

Pemphygus vulgaris

 B

Pemphigus foliaceous

 C

Psoriasis

 D

Vitiligo

Ans. A

Explanation:

Ans. is ‘a’ i.e., Pemphygus vulgaris 

Among the given options, two are vesicullabullous disorders :-

  1. Pemphigus vulgaris
  2. Pemphigus foliaceous
  • Both of these cause flaccid bullae with intraepidermal blisters.
  • but, mucosal involvement is common in pemphigus vulgaris (not in P foliaceous).

Q. 68

Ig A deposition in dermoepidermal junction skin seen in ‑

 A

Dermatitis herpetiformis

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Epidermolysis bullosa

Q. 68

Ig A deposition in dermoepidermal junction skin seen in ‑

 A

Dermatitis herpetiformis

 B

Bullous pemphigoid

 C

Linear IgA disease

 D

Epidermolysis bullosa

Ans. C

Explanation:

Ans. is ‘c’ i.e., Linear IgA disease


Q. 69

Antigen defect in Pemphigus Vulgaris –

 A

Desmoglein-1

 B

Desmoglein-3

 C

Desmocollin-3

 D

Desmocollin-2

Q. 69

Antigen defect in Pemphigus Vulgaris –

 A

Desmoglein-1

 B

Desmoglein-3

 C

Desmocollin-3

 D

Desmocollin-2

Ans. B

Explanation:

Ans. is ‘b’ i.e., Desmoglein-3 


Q. 70

30 years old male presents with painful flaccid bullae on right face and oral mucosa without evidence of inflammation or redness; what is the most probable diagnosis?

 A

Pemphigus vulgaris

 B

Pemphigus foliaceous

 C

Bullous pemphoid

 D

Dermatitis herpetiformis

Q. 70

30 years old male presents with painful flaccid bullae on right face and oral mucosa without evidence of inflammation or redness; what is the most probable diagnosis?

 A

Pemphigus vulgaris

 B

Pemphigus foliaceous

 C

Bullous pemphoid

 D

Dermatitis herpetiformis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Pemphigus vulgaris


Q. 71

Acantholytic cells in blister cavity ‑

 A

Pemphigus

 B

Bullous pemphigoid

 C

Epidermolysisbullosa

 D

Dermatitis herpetiformis

Q. 71

Acantholytic cells in blister cavity ‑

 A

Pemphigus

 B

Bullous pemphigoid

 C

Epidermolysisbullosa

 D

Dermatitis herpetiformis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Pemphigus

Important Tzank smear finding of bullous lesions

  • VZV and HSV infection  —>       Multinucleated giant cells
  • Pemphigus                   —>       Acontholytic cells
  • Pemphigoid                  —>       Predominantly eosinophils

Q. 72

Which of the following is a subepidermalvesiculobullous disorder‑

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus vegetans

 D

Pemphigus erythematosus

Q. 72

Which of the following is a subepidermalvesiculobullous disorder‑

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Pemphigus vegetans

 D

Pemphigus erythematosus

Ans. B

Explanation:

Ans. is ‘b’ i.e., Bullous pemphigoid 


Q. 73

60 year female with tense bulla in lower extremity and subepidermal bullous lesion on microscopy, diagnosis is‑

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Erythema multiforme

 D

Dermatitis herpetiformis

Q. 73

60 year female with tense bulla in lower extremity and subepidermal bullous lesion on microscopy, diagnosis is‑

 A

Bullous pemphigoid

 B

Pemphigus vulgaris

 C

Erythema multiforme

 D

Dermatitis herpetiformis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Bullous pemphgoid

  • See explanation- 1 of session- 10 of Skin of All India 2014-15 pattern of this book.

Q. 74

Tense itching bulla is seen in ‑

 A

Bullous pemphigoid

 B

Dermatitis herpetiformis

 C

Pemphigous vulgaris

 D

Erythema multiforme

Q. 74

Tense itching bulla is seen in ‑

 A

Bullous pemphigoid

 B

Dermatitis herpetiformis

 C

Pemphigous vulgaris

 D

Erythema multiforme

Ans. A

Explanation:

Ans. is ‘a’ i.e., Bullous pemphigoid


Q. 75

Immunoflorescence pattern in pemphigus vulgaris is suggestive of ‑

 A

Intercellular IgG deposition

 B

C3 and IgG deposition at dermo-epidermal junction

 C

IgA deposition at dermo-epidermal junction

 D

IgA at pappilary tip

Q. 75

Immunoflorescence pattern in pemphigus vulgaris is suggestive of ‑

 A

Intercellular IgG deposition

 B

C3 and IgG deposition at dermo-epidermal junction

 C

IgA deposition at dermo-epidermal junction

 D

IgA at pappilary tip

Ans. A

Explanation:

Ans. is ‘a’ i.e., Intercellular IgG deposition


Q. 76

Subepidermal bullae is seen in ‑

 A

Pemphigus vulgaris

 B

Darriers disease

 C

Bullous pemphigoid

 D

HSV infection

Q. 76

Subepidermal bullae is seen in ‑

 A

Pemphigus vulgaris

 B

Darriers disease

 C

Bullous pemphigoid

 D

HSV infection

Ans. C

Explanation:

Ans. is ‘c’ i.e., Bullous pemphigoid


Q. 77

Bullae of bullous pemphigoid are ‑

 A

Intraepiderma

 B

Subepidermal

 C

Subepidermal

 D

None

Q. 77

Bullae of bullous pemphigoid are ‑

 A

Intraepiderma

 B

Subepidermal

 C

Subepidermal

 D

None

Ans. B

Explanation:

Ans. is ‘b’ i.e., Subepidermal

Intra-epidermal immunobullous disorder

Sub-epidermal immunobullous disorder

Pemphigus vulgaris

Bullous pemphigoid

Pemphigus vegetan

Mucous memb. Pemphigoid

Pemphigus foliaceu

Pemphigoid gestationalis

Pemphigus foliaceu

Linear IgA disease

Intercellular IgA dermatosis

Epidermolysis bullosa aquisita

Paraneoplastic Pemphigus Dermatitis herpetiformis

Q. 78

Acantholysis is seen in all except ‑

 A

Pemphigus vulgaris

 B

Darrier’s disease

 C

SSSS

 D

Bullous pemphigoid

Q. 78

Acantholysis is seen in all except ‑

 A

Pemphigus vulgaris

 B

Darrier’s disease

 C

SSSS

 D

Bullous pemphigoid

Ans. D

Explanation:

Ans. is ‘D’.e., Bullous pemphigoid

Acantholysis

  • Separation of epidermal keratinocytes due to loss of intercellular bridge is referred to as acantholysis .
  •  Diseases causing acantholysis are : ‑
  1. Pemphigus (Pemphigus vulgaris, P. vegetans, P.foilaceous, P. erythematosus)
  2. Eosinophilic spongiosis
  3. Darrier’s disease
  4. Grover’s disease (Transient acantholytic dermatosis)
  5. Hailey-Hailey disease (Familial benign chronic pemphigus)
  6. Infections with HSV-1 & 2.
  7. Staphylococcus scalded skin syndrome.

Q. 79

Granular deposit of IgA at dermoepidermal junction is seen in ‑

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Dermatitis herpatiformis

 D

Pemphigus foliaceous

Q. 79

Granular deposit of IgA at dermoepidermal junction is seen in ‑

 A

Pemphigus vulgaris

 B

Bullous pemphigoid

 C

Dermatitis herpatiformis

 D

Pemphigus foliaceous

Ans. C

Explanation:

Ans. is ‘c’ i.e., Dermatitis herpatiformis

Immunopathological appearance of vesicobullous disorders

  • Bullous pemphigoid                                                → Linear band of IgG or C3 in epidermal basement membrane
  • Pcmphigus vulgaris                                                →  Cell surface deposits of IgG on keratinocytes
  • Pemphigus foliaceous                                             →  Cell surface deposits of IgG on keratinocytes —>
  • Pemphigoid gestationis                                           →   Linear band of C3 in epidermal basement membrane zone
  • Dermatitis herpeteformis                                        →   Granular deposits of IgA in dermal papillae –>
  • Chronic bullous disease of childhood                        →   IgA deposits along the dermoepidermal junction
  • Linear IgA dermatosis of adults                               →    Linear deposits of IgA at dermoepidermal junction


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