Oral Aspects of Dermatological Disorders Pathology Question And Answers

Oral Aspects of Dermatological Disorders Question And Answers

Question  1. Which type of disorder is ectodermal dysplasia?
Answer. Ectodermal dysplasia is a hereditary disorder, characterized by defective formations of ectodermal structures in the body.

Question  1a. What type of genetic defect initiates ectodermal dysplasia?
Answer. It is an X-linked recessive disorder.

Read And Learn More: Oral Pathology Short Notes Question And Answers

Question  2. Name the body structures which are often defective in their formation in ectodermal dysplasia.
Answer. In this disease, defects are often seen in the skin, teeth, nails, sweat glands, sebaceous glands and hair follicles, etc.

Question  3. What are the most outstanding features of hereditary ectodermal dysplasia?
Answer. There are three most outstanding features of ectodermal dysplasia and they are:

• Hypohydrosis—lack of sweating
• Hypotrichosis—absence of body hair
• Hypodontia—absence of teeth.

Question  4. In which gender, hereditary ectodermal dysplasia is more often seen?
Answer. The disease occurs more frequently among males than females.

Question  5. What are the effects of absence of sweat glands in hereditary ectodermal dysplasia?
Answer. The absence of sweat glands will have the following effects:

• Patients will have soft, dry and smooth skin with little or no tendency for sweating
• They cannot endure warm temperature and often have unexplained fever due to failure of body temperature control mechanism via sweating (release of body heat through sweating is not possible).

Question  6. What happens in hypotrichosis in ectodermal dysplasia?
Answer. Hypotrichosis or absence of body hair is an important feature of the disease; in patient, the hair over the scalp, eyelashes and eyebrows are often fie, scanty and blond.

Question  7. What are the functions of sebaceous gland?
Answer.

• Sebaceous glands secrete an oily substance known as sebum that lubricates the hair and skin of mammals
• Large quantities of sebum protect skin and hair from water
• Sebum also reduces the growth of microorganisms anywhere on the skin.

Question  8. What is asteatosis?
Answer. The defiiency or absence of sebaceous gland secretions is called asteatosis; which often causes persistent dry scaling of the skin.

Question  9. In hereditary ectodermal dysplasia, how the face of the patient often looks?
Answer. Patient with ectodermal dysplasia always have a typical facial appearance characterized by depressed nasal bridge, frontal bossing and protuberant lips, etc and because of this, patients from different families are often mistaken for siblings (brothers or sisters of one another).

Question  9a. Pseudorhagades are seen in which disease?
Answer. Rhagades are fisures, cracks, or linear scars in the skin, especially at the angles of the mouth and nose (seen in congenital syphilis due to bacterial infection). In ectodermal dysplasia, abnormally thin and dry skin often produces many foldings, which give rise to a rhagades-like appearance and hence are called ‘pseudo-rhagades’.

Question  10. Why xerostomia occurs in hereditary ectodermal dysplasia?
Answer. Xerostomia is a constant feature in this disease, which occurs due to the decreased salivary secretion secondary to hypoplasia of salivary glands.

Question  11. Describe the dental changes in hereditary ectodermal dysplasia.
Answer. Dental changes are most signifiant in this disease and are characterized by complete or partial anodontia; which involves both deciduous as well as the permanent dentition. Whenever the teeth are present these are often small and abnormal in shape (the incisors often look tapered, conical or pointed while the molars look narrow and much smaller in diameter).

Question  12. Which teeth are more likely to be seen whenever few teeth are present in the mouth of a patient with hereditary ectodermal dysplasia?
Answer. Canines.

Question  13. In case of anodontia, what happens to the alveolar bone in the jaw?
Answer. The alveolar bone does not develop unless the teeth erupt in the mouth, same thing happens in ectodermal dysplasia also.

Question  14. Name the mucosal changes in hereditary ectodermal dysplasia.
Answer.

• Patients often have dryness and cracking of the lips
• Rhinitis, sinusitis and pharyngitis etc, with dysphagia and hoarseness of voice are also common.

Question 14a. Give some other features, which may be found in ectodermal dysplasia.
Answer. Fine wrinkling with hyperpigmentation of periocular skin and malformed, brittle figer nails.

Question  15. What are the treatments of hereditary ectodermal dysplasia?
Answer.

• For the teeth which have not erupted artifiial dentures (with soft liners) are constructed and are changed from time to time to cope up with the growth of the jaws
• Artifiial saliva is often given to keep the mouth moist and make it easy to eat.

Question  16. What is psoriasis?
Answer. Psoriasis is a common self-limiting, chronic inflmmatory dermatological disorder characterized by an increased proliferative activity of skin and mucous membrane.

Question  17. Describe the dermal lesions of psoriasis.
Answer.

• Skin lesions of psoriasis clinically appear as well-demarcated, painless, dry, erythematous patches with silvery scale on the surface
• The lesions enlarge at the periphery and there may be periods of remissions and exacerbations
• These skin lesions are often symmetrically distributed; these are more severe during the winter and the condition is much better during the summer.

Question  18. What is Auspitz’s sign?
Answer. In psoriasis, dry erythematuos patches develop on the skin with silvery scales on the surface; it is often seen that if the deep scales are removed from the skin, one or two tiny bleeding points often appear and the phenomenon is known as Auspitz’s sign.

Question  19. Describe the mucosal lesions of psoriasis.
Answer. In psoriasis, oral lesions may appear as erythematous patches with white scaly surfaces over the lips, palate, gingiva and cheek, etc. Some oral lesions may resemble the geographic tongue.

Question  20. Which is one of the dermal diseases with minimal oral manifestation?
Answer. Psoriasis.

Question  21. What is abscess of Munro?
Answer. In psoriasis, oral epithelium shows atrophy with hyperparakeratosis, absence of granular cell layer and elongation or clubbing of the rete-pegs. However an important feature of the disease is the formations of intraepithelial microabscess, which are called abscess of Munro.

Question  21a. In which disease absence of granular cell layer and clubbing of rete-pegs in epithelium are seen?
Answer. Psoriasis.

Question  21b. What are psoriasiform lesions?
Answer. There are some lesions, which histologically resemble psoriasis and are called psoriasiform lesions.

Question  21c. Give examples of oral psoriasiform lesions.
Answer. Reiter’s syndrome, geographic tongue and erythema circinata migrans (ectopic geographic tongue).

Question  22. What type of disease pityriasis rosea is?
Answer. Pityriasis rosea is a rare mucocutaneous disease which often shows seasonal outbreaks (mostly during spring or autumn).

Question  23. Describe the clinical features of pityriasis rosea.
Answer. Pityriasis rosea presents superfiial, light red, macules or papules over the skin which are preceded by a typical bright-red skin rash in the same area. Oral lesions present erythematous macules over the cheek, tongue and palate, etc.

Question  23a. What is herald patch of Pityriasis rosea?
Answer. Pityriasis rosea characteristically begins as an asymptomatic single, large pink, scaly (2–10 cm) patch on the trunk, which is called the “herald patch” or mother patch.

Question  24. What is incontinentia pigmenti?
Answer. It is a relatively rare, serious type of inherited genodermatosis which is transmitted as a sex-linked dominant trait.

Question  25. How does the lesion incontinentia pigmenti behave when it affects males?
Answer. The disease is more commonly seen among females; however, it is more lethal whenever affecting males.

Question  26. Describe the clinical features of incontinentia pigmenti.
Answer. It produces a typical slate-gray pigmentation of the skin after birth with formation of vesicles or bullae over the trunk and limbs. Teeth are often small and conical, while the oral mucosa exhibits patchy, plaque-like white lesions on the buccal mucosa.

Question  26a. What is Darier’s disease?
Answer. Darier’s disease is a hereditary disorder characterized by dark crusty patches on the skin, keratotic plaques of oral mucosa and fragile figer nails.

Question  27. What is erythema multiforme?
Answer. Erythema multiforme is an acute inflmmatory dermatological disorder characterized by extensive blistering and ulcerations.

Question  28. Name the factors which can precipitate erythema multiforme.
Answer.

• Infections, e.g. tuberculosis, Herpes simple type I & II etc.
• Drug reactions from barbiturates, sulfonamides, phenyl butazone and salicylates etc
• Hyperimmune reactions in the submucosal and dermal blood vessels
• Radiation therapy
• Vaccinations.

Question  29. Describe the clinical features of erythema multiforme.
Answer. The disease exhibits rapidly developing round erythematous macules, papules or vesicles over the skin and these lesions are often referred to as target, iris or bull’s eye, etc according to their typical appearance.

Question  30. Describe the target lesions seen in erythema multiforme.
Answer. The target lesions are mostly seen over the extremities and these are red macules of 1 centimeter or more in diameter with a bluish cyanotic center. They are often consisting of concentric erythematous rings, separated by rings of near normal color on the skin.

Question  31. How the mucosal lesions appear in erythema multiforme?
Answer. Mucosal lesions of this disease consist of rapidly developing macules, papules or vesicles, etc. and these are mostly seen on the tongue, labial mucosa, buccal mucosa and flor of the mouth, etc. The vesicles on the mucosal surfaces are often short-lived and they readily become eroded or ulcerated and bleed profusely.

Question  32. What happens in the serious form of erythema multiforme?
Answer. Severe form of erythema multiforme may cause widespread sloughing and ulceration of the skin and mucous membrane of the entire body. Patients may also develop tracheobronchial ulcerations and pneumonia, etc.

Question  33. What is Stevens Johnsons syndrome?
Answer. It is a severe form of erythema multiforme (also known as erythema multiforme major) that simultaneously involves the skin, eyes, oral mucosa and genitalia. This condition is often triggered by a drug and not by an infection.

Question  33a. In which syndrome simultaneous involvement of mouth, eye and genitalia is seen?
Answer. Stevens Johnsons syndrome.

Question  34. Describe the clinical manifestations of Stevens Johnsons syndrome.
Answer.

• Skin lesions: Severe lesions of macules, papules, vesicles and bullae, etc
• Mucosal lesions: Large vesicles or bullae with subsequent painful ulcerations and hemorrhage
• Eye lesions: Photophobia, conjunctivitis, corneal ulceration and uveitis, etc
• Genital lesions; urethritis, balanitis and vaginal ulceration, etc.

Question  35. Describe the histopathological changes seen in affected epithelium of erythema multiforme.
Answer. Histologically erythema multiforme presents severe non-specifi inflmmation in the epithelium with acanthosis, intra- or intercellular edema, widespread necrosis of the basal keratinocytes, vesicles or bullae formation within the epithelium and chronic inflmmatory cell infitration in the underlying connective tissue.

Question  36. Name the lesions commonly included in the differential diagnosis of erythema multiforme.
Answer.

• Acute primary herpetic stomatitis
• Pemphigus
• Pemphigoid
• Erosive lichen planus
• Aphthous ulcers.

Question  37. What is the treatment of erythema multiforme?
Answer. Topical and systemic steroid therapy, coupled with antibiotics.

Question  38. What is dermatitis herpetiformis?
Answer. Dermatitis herpetiformis is a rare chronic recurrent mucocutaneous disease of unknown etiology.

Question  39. Which dietary element is presumed to be responsible for development of Dermatitis herpetiformis?
Answer. Gluten (sticky part of wheat flwer).

Question  40. What are the clinical features of dermatitis herpetiformis?
Answer. There will be presence of symmetrically distributed erythematous, papules or vesicles on the skin or mucous membrane which often resemble herpes simplex. These lesions are preceded by pruritis with severe burning sensation in the affected areas of skin.

Question  41. What is keratosis follicularis?
Answer. Keratosis follicularis is a hereditary disorder of the skin and is characterized by the formation of multiple crusted, greasy lesions that often produce foul odor.

Question  42. Describe the clinical features of keratosis follicularis.
Answer. There is development of erythematous hyperkeratotic papules on the skin, which gradually turns grayish-brown with age. Accumulation of keratin often gives a rough texture to these lesions; moreover bacterial desquamation of these keratins often produces foul smell from the lesion.

Question  43. Describe the histological appearances of keratosis follicularis.
Answer. Histologically the disease shows multiple clefts and lacunae within the epithelium; with narrow, elongated, taste-tube like rete-ridges. Each epithelial cleft or lacunae contains two types of cells, namely the corps grains (grain-like keratinized epithelial cells) and corps ronds (eosinophic cells).

Question  44. What is acanthosis nigricans?
Answer. Acanthosis nigricans is a rare cutaneous disease which usually affects the flxural surfaces of skin and it has an occasional oral mucosal component.

Question  45. Describe the clinical features of dermal lesions of acanthosis nigricans.
Answer. Dermal lesions clinically present numerous, fie, velvety, conflent plaques with brownish pigmentation. These lesions predominantly occur on the flxural surfaces of skin over axilla, palms, soles and face, etc.

Question  46. How many types of acanthosis nigricans can be seen?
Answer. Usually there are three types:

• Benign type
• Malignant type
• Pseudoacanthomatous type.

Question  47. Describe the clinical features of oral lesions of acanthosis nigricans.
Answer. Oral lesions can be seen in the tongue, gingiva, lips and cheek, etc and these oral lesions often occur in association with the malignant form of the disease.

• Tongue lesions are characterized by either hypertrophy of the fiiform papilla or formation of a papillomatous growth
• Lip is grossly enlarged (mainly the upper lip) and its surface is dotted with small papillomatous nodules especially at the commissure
• Givgiva also exhibits hyperplastic changes and the lesions are clinically similar to that of the firomatosis gingivae.

Question  47a. Name one important signifiance of development of acanthosis nigricans.
Answer. Its presence in the skin may be associated with an internal malignancy e.g. stomach cancer.

Question  48. Describe the clinical features of dyskeratosis congenita.
Answer.

• Grayish-brown pigmentations of skin over the trunk, neck and thighs
• Facial skin appears red due to atrophic or telangiectatic changes
• Formations of vesicles and bullae in the oral mucosa especially with occasional presence of leukoplakic or erythroplakic patches
  Presence of systemic conditions like-mental retardation, dysphagia, deafness, thrombocytopenic purpura, aplastic anemia and hyperhydrosis of the palms and soles, etc.

Question  49. Describe the changes in the oro-facial changes in dyskeratosis congenita.
Answer.

• Redness and atrophy of the facial skin with telangiectatic changes
• Formations of vesicles and bullae in the oral mucosa with erosive changes in mucosa
• Development of leukoplakic or erythroplakic patches in oral mucosa
• Dysphagia
• Severe periodontal tissue destruction and loss of alveolar bone is often seen
• Increased risk of development of squamous cell carcinoma from the leukoplakia lesions in the oral mucosa.

Question  50. Name the systemic conditions which may be associated with dyskeratosis congenita.
Answer.

• Mental retardation, dysphagia and deafness
• Thrombocytopenic purpura and aplastic anemia
• Hyperhydrosis of the palms and soles.

Question  51. Defie white sponge nevus.
Answer. White sponge nevus is a hereditary dermal lesion characterized by development of white, thickened, corrugated mucosal lesions in the oral cavity.

Question  51a. Name one important characteristic of white sponge nevus.
Answer. The disease often simultaneously affects several members of the same family.

Question  52. What is the underlying cause that leads to the development of white sponge nevus?
Answer. The disease occurs due to a genetic defect in the normal keratinization process of oral epithelium.

Question  53. What is the common age of occurrence of white sponge nevus?
Answer. The disease generally occurs at birth or during childhood.

Question  54. Describe the clinical features of white sponge nevus.
Answer. Clinically oral lesions exhibit symmetrically thickened, white, folded or corrugated diffuse plaques; which occur bilaterally over the cheek. The oral lesions are asymptomatic, soft and spongy with a peculiar opalescent hue.

Question  55. Describe the histopathological appearance of white sponge nevus.
Answer.

• Hyperparakeratosis of the epithelium with acanthosis and intercellular edema.
• Presence of some large vacuolated cells in the spinus cell layer with pyknotic nuclei. Oral Aspects of Dermatological Disorders 441
• Presence of abnormally prominent epithelial cell membranes with a typical basketweave appearance.

Question  56. Name the lesions which should fiure in the differential diagnosis of white sponge nevus.
Answer.

• Hereditary benign intraepithelial dyskeratosis
• Lichen planus
• Leukodema
• Hyperplastic candidiasis
• Leukoplakia
• Verrucous carcinoma.

Question  57. What is polymyositis?
Answer. Polymyositis is an inflmmatory myopathy of unknown etiology which commonly affects the skin in females.

Question  58. What are the clinical features of polymyositis?
Answer.

• The disease clinically presents muscle pain, fever, malaise, arthralgic pain and weight loss, etc
• A violaceous skin rash commonly develops over the cheek, eyelids and hands, etc
• Oral mucosa reveals erythematous patches having lichen planus like appearance with swelling and a high-risk malignancy.

Question  59. What is pemphigus?
Answer. Pemphigus is a group of vesiculobullous lesions of the skin and mucosa membrane, which is characterized by the formation of intraepithelial vesicles or bullae causing separation of the epithelium above the basal cell layer.

Question  60. What type of disease pemphigus is?
Answer. It is an autoimmune disease.

Question  61. Defie autoimmune diseases.
Answer. These are the diseases in which antibodies are produced against some of the body’s own tissues or tissue components, which results in immune mediated damage to that particular tissue.

Question  61a. Name the common autoimmune diseases which have some oral manifestations.
Answer.

• Addison’s disease
• Ankylosing spondylitis
• Pemphigus
• Pemphigoid
• Celiac disease
• Crohn’s disease
• Idiopathic thrombocytopenic purpura
• Lupus erythematosus
• Scleroderma
• Psoriasis
• Pernicious anemia
• Rheumatoid arthritis
• Sjogren’s syndrome
• Wegener’s granulomatosis.

Question  62. What are autoantibodies?
Answer. The antibodies which are produced against the body’s own tissues in autoimmune disorders are known as autoantibodies.

Question  63. In pemphigus, autoantibodies are produced against which tissue components of the body?
Answer. Autoantibodies (IgG and complements) are produced in pemphigus specifially against the intercellular cement substances (desmosomes) of the skin and mucous membrane.

Question  64. What is the pathogenesis of pemphigus?
Answer. Once the autoantibodies are produced against the desmosomes of the skin or mucous membrane, they initiate an antigen-antibody reaction leading to destruction and dissolution of the desmosomal attachments between the individual spinus cells. Destruction of desmosomal attachments thus cause loss of adhesion between one cell to the other (acantholysis) with development of intraepithelial bullae; if the bullae are multiple in number and they coalesce together, it may lead to a suprabasilar split in the skin or epithelium.

Question  65. What is acantholysis?
Answer. In pemphigus, autoantibodies cause destruction and dissolution of the desmosomal attachments between the individual cells in the spinus layer; this leads to loss of adhesion between one cell to the other and cells fall apart, this phenomenon is known as acantholysis.

Question  66. What are the different clinical types of pemphigus?
Answer. There are about fie types of pemphigus seen, which are as follows:

• Pemphigus vulgaris
• Pemphigus vegetans
• Pemphigus foliaceus
• Pemphigus erythematosus
• Brazilian pemphigus.

Question  67. Which variety is the most common among all types of pemphigus lesions?
Answer. Pemphigus vulagaris.

Question  68. What is the age of occurrence of pemphigus vulgaris and in which gender it is more common?
Answer. Pemphigus vulgaris generally occurs between the ages of 40 and 70 years and it is more prevalent among females.

Question  69. Describe the clinical features of pemphigus vulgaris.
Answer. In pemphigus vulgaris, rapidly developing vesicles or bullae appear on several areas of the skin and mucous membrane. The vesicles contain clear flids initially, but later on there is formation of pus; vesicles rupture quickly and leave extremely painful, superfiial, erythematous ulcers with ragged borders.

Question  70. Describe the typical characteristics of oral lesions in pemphigus vulgaris.
Answer. In pemphigus vulgaris, oral lesions are clinically very important because of the following:

• The vesicles and bullae fist develop in the mouth and then these lesions spread widely on to the skin
• Over 60 percent cases of pemphigus vulgaris, oral lesions precede the skin lesions and in about 25 percent cases, oral lesions are the only manifestations of the disease
• Oral lesions often begin as bleb-like blisters or as diffuse gelatinous plaques
• Although they appear fist in the mouth, intact vesicles are rarely found in the mouth, as they rupture soon after they form.

Question  71. Which parts of oral mucosa are involved in oral lesions in pemphigus vulgaris?
Answer. Oral lesions in pemphigus vulgaris may involve any part of the oral cavity but are more frequently found in those areas, which are often subjected to trauma, e.g. cheek, labial mucosa, ventral surface of tongue, palate and gingiva, etc.

Question  72. What does oral mucosal lesions of pemphigus often resemble to once the vesicles are ruptured?
Answer. Erosive lichen planus.

Question  73. What is Nikolsky’s sign?
Answer. In case of pemphigus, gentle traction or oblique pressure on the unaffected areas of the skin or mucous membrane around the lesion causes stripping of the normal skin or mucosa, this phenomenon is known as Nikolsky’s sign.

Question  74. Do the lesions of pemphigus heal with scarring?
Answer. Skin lesions of pemphigus usually heal by scar formation but the mucosal lesions heal without scarring.

Question  75. What are the causes of death in patients with pemphigus?
Answer. Patient may die of dehydration and septicemia.

Question  76. Describe pemphigus vegetans.
Answer. This is also a common form of pemphigus, it resembles the pemphigus vulgaris in all respects, except the development of papillomatous hyperplasias (vegetations), following the rupture of the bullae.

Question  77. What is pemphigus foliaceus and pemphigus erythematosus?
Answer. Both are rare forms of pemphigus lesions and here the bullae formation is often associated with a marked erythema of the involved skin. Both these lesions resemble exfoliative dermatitis and, rarely, there are any oral manifestations.

Question  78. What is Brazilian pemphigus?
Answer. It is an endemic form of the disease, which affects several members of the same family and is common in the coastal areas of Brazil.

Question  78a. What is paraneoplastic pemphigus (PNP).
Answer. Paraneoplastic pemphigus is a life-threatening autoimmune vesiculobulous disease, which is almost invariably linked to some internal malignancies in the body.

Question  78b. Name the internal neoplasms/malignancies commonly associated with paraneoplastic pemphigus.
Answer.

• Non-Hodgkin’s lymphoma
• Chronic lymphocytic leukemia
• Sarcoma
• Thymoma.

Question  79. Describe the chief histopathological features of pemphigus.
Answer.

• Loss of intercellular bridges and collection of edema flid results in acantolysis within the spinus cell layer, which causes disruption of the prickle cells
• This leads to formation of the vesicle or bullae within the epithelium that often results in a suprabasilar split or separation
• Accumulation of Tzanck cells at the bottom of the intraepithelial vesicles or bullae
• Presence of few polymorphonuclear neutrophil (PMN) and lymphocytes within the vesicular flid.

Question  80. What are Tzanck cells?
Answer. These are large, round, hyperchromatic desquamated epithelial cells, which are often seen in pemphigus and these cells form as a result of acantholysis in the spinus cell layer of the epithelium.

Question  80a. Name the diseases where Tzanck cells can also be found.
Answer.

• Herpes simplex
• Herpes zoster
• Chicken pox
• Pemphigus vulgaris
• Cytomegalovirus infection.

Question  80b. What is Tzanck test?
Answer. It is a dermatological test, in which scraping of an ulcer base is done to look for Tzanck cells.

Question  81. How the lamina propria appears in pemphigus in terms of inflmmatory changes?
Answer. In pemphigus, unlike any other vesiculobullous lesion, very little inflmmatory cell infitration is seen in the underlying connective tissue.

Question  82. Describe the results of immunofloresence tests in pemphigus.
Answer. Immunofloresence tests in pemphigus demonstrate deposition of specifi immunoglobulins (IgG) within the intercellular areas of epithelium.

Question  83. How does the intensity of florescence vary in different parts of the epithelium following immunohistochemical staining in pemphigus?
Answer. In pemphigus, intensity of the florescence is greatest in the suprabasilar region (spinous cell layers) and gradually it becomes decreased toward the surface of the epithelium.

Question  84. Name the lesions often included in the differential diagnosis of pemphigus.
Answer.

• Pemphigoid
• Erythema multiforme
• Epidermolysis bullosa
• Toxic epidermal necrolysis
• Aphthous ulcers.

Question  85. Name the outlines of treatment in pemphigus.
Answer.

• Administration of high-doses of steroid to resist autoimmune reaction
• Immunosuppressive drugs therapy by azathioprine or methotrexate, etc.
• Antibiotics to prevent secondary infections
• Maintenance flid and electrolyte in the body.

Question  86. What is pemphigoid?
Answer. Pemphigoid is an autoimmune vesiculobullous lesion which is characterized histologically by subepithelial bulla formation in the basement membrane zone (BMZ) of the skin and epithelium.

Question  87. What is cicatrization?
Answer. Unlike pemphigus, pemphigoid lesions usually heal up by scar formation and the process is called cicatrization.

Question  88. In which organ scar formation occurs most frequently in case of pemphigoid?
Answer. The scarring mostly occurs in eye lesions in case of pemphigoid and sometimes it can cause even blindness.

Question  89. Describe in brief the pathogenesis of pemphigoid.
Answer. First of all, there is formation of autoantibodies, which specifially act against the hemidesmosome associated antigenic components in the basement membrane zone. After binding of autoantibodies, there will be activation of complements, which attract inflmmatory cells. After this, there is synthesis of chemotactic factors and release of proteolytic enzymes (especially protease) by inflmmatory cells. Protease causes destruction of hemidesmosomal junctions of the basement membrane zone leading to the formation of subepithelial vesicle or bullae.

Question  90. Name the different clinical types of pemphigoids.
Answer. Pemphigoids are generally of two types:

1. Cicatricial pemphigoid and
2. Bullous pemphigoid.

Question  91. Which tissue is predominantly affected by cicatricial pemphigoid?
Answer. Cicatricial pemphigoid commonly affects oral mucous membrane.

Question  92. Which tissue is predominantly affected by bullous pemphigoid?
Answer. Bullous pemphigoid commonly affects the skin.

Question  93. What is the mean age of occurrence of pemphigoid?
Answer. The mean age of occurrence is above 60 years.

Question  94. Which gender is often vulnerable to the disease pemphigoid?
Answer. Females are affected twice as often as males.

Question  95. What is the other name of cicatricial pemphigoid?
Answer. Cicatricial pemphigoid is also known as benign mucous membrane pemphigoid

Question  96. Name the sites of involvement of cicatricial pemphigoid.
Answer. The most common site of occurrence is the oral mucous membrane, followed by the conjunctiva. Sometimes the disease also involves the nose, pharynx, larynx, esophagus and genitalia, etc.

Question  97. Describe the oral lesions of cicatricial pemphigoid.
Answer.

• Erythema, erosion, desquamation and ulceration in the gingiva
• Mucosal erythema followed by development of vesicle or bulla in the palate, buccal mucosa, alveolar mucosa and tongue, etc
• Bleeding into the bullae can make them look-like blood blisters
• Once the bullae rupture, they leave large painful, eroded or ulcerated areas, which heal up slowly.

Question  98. Why the vesicle or bullae in pemphigoid often persist in the mouth for longer durations as compared to those of pemphigus?
Answer. The vesicles or bullae in pemphigoid often persist in the mouth for several days as compared to those of pemphigus, which rupture as soon as they form. It happens because the vesicles or bullae in pemphigoid are tougher because these are usually covered by a full thickness epithelium.

Question  99. Is Nikolsky’s sign also positive in pemphigoid?
Answer. Nikolsky’s sign is typically positive.

Question  100. Which oral condition can often be the only manifestation of cicatricial pemphigoid?
Answer. Desquamative gingivitis; which is characterized by patchy or widespread erythema in the gingiva with mild discomfort, widespread desquamation and ulceration, etc.

Question  101. Name the common eye lesions occurring in pemphigoid.
Answer.

• Conjunctivitis
• Blister formation in the eye
• Corneal ulceration
• Swelling of the fornix and corneal opacity
• Fibrosis and scarring of the lacrimal ducts which often lead to dry eye
• Blindness on rare occasions.

Question  102. What are the basic features of bullous pemphigoid?
Answer.

• Bullous pemphigoid occurs more commonly on the skin and it seldom affects the oral mucosa
• These lesions also follow the usual clinical patterns of vesiculation, followed by ulceration and fially healing (without scarring).

Question  103. Describe the basic histological features of pemphigoid.
Answer. Histologically pemphigoid presents formation of subepithelial vesicles or bullae at the basement membrane zone which causes separation of full thickness epithelium from the underlying connective tissue.

Question  104. What is the treatment of choice in pemphigoid?
Answer. Systemic steroid therapy is the treatment of choice in both forms of pemphigoid.

Question  105. Defie epidermolysis bullosa.
Answer. Epidermolysis bullosa is a generalized desquamating condition of the skin and mucous membrane, which is often associated with scarring, contractures and dental defects.

Question  106. Describe the basic pathologic defect as may be seen in epidermolysis bullosa.
Answer. In epidermolysis bullosa, there is separation of the epithelium from the underlying connective tissue with formation of large blisters, which often heal with extensive and often immobilizing scar formations.

Question  107. What are the different types of epidermolysis bullosa?
Answer. Several forms of epidermolysis bullosa have been recognized and these lesions are divided into two broad categories:

• Hereditary epidermolysis bullosa
• Acquired epidermolysis bullosa.

Question  108. Name the different forms hereditary epidermolysis bullosa.
Answer. The hereditary forms of epidermolysis bullosa include three types:

• Epidermolysis bullosa simplex
• Epidermolysis bullosa dystrophic
• Junctional epidermolysis bullosa.

Question  109. How many types of acquired epidermolysis bullosa exist?
Answer. There is only a single form of acquired epidermolysis bullosa, and it is named as epidermolysis bullosa acquistia.

Question  110. Name the diseases, which may be associated with the acquired epidermolysis bullosa.
Answer. This acquired form of the disease may be associated with multiple myeloma, diabetes mellitus, tuberculosis, amyloidosis and Crohn’s disease, etc.

Question  111. Describe the clinical features of epidermolysis bullosa.
Answer.

• Formation of multiple vesicles or bullae on the pressure areas of skin (i.e. elbows and knees)
• The bullae rupture and leave raw, painful ulcers, which heal up with scarring
• The healing of skin lesions cause scarring with pigmentation or depigmentation of the area
• Nails often shed or exfoliate due to formation of blisters in the nail beds
• Patients with hereditary epidermolysis bullosa may also exhibit stunted growth, mental retardation and ectodermal dysplasia, etc.

Question  112. Which type is generally more severe among the acquired and hereditary forms of epidermolysis bullosa?
Answer. The hereditary forms of the disease are usually very severe than the acquired type.

Question  113. Oral lesions are more often seen in relation to which form of epidermolysis bullosa?
Answer. Oral lesions are particularly common and more severe in relation to the hereditary forms of the disease.

Question  114. Describe the oral manifestations of epidermolysis bullosa.
Answer.

• Rapidly developing, multiple, fragile and hemorrhagic blisters in the areas of trauma (particularly in the palate)
• The blisters rupture soon and leave painful ulcers, which heal with scarring
• Repeated blistering and scarring around the oral cavity may result in decreased mouth opening (microstomia), ankyloglossia and loss of vestibular sulci
• Some patients may develop perioral and perinasal crusted and hemorrhagic granular lesions
• The dental changes include hypoplastic pitted enamel of the molar teeth, delayed eruption of teeth, increased caries susceptibility and increased periodontal diseases, etc.

Question  115. What is the most serious consequence of epidermolysis bullosa?
Answer. Sometimes oral lesions may transform into squamous cell carcinoma in this disease.

Question  116. Describe the histopathological features of oral lesions of epidermolysis bullosa.
Answer.

• Extensive destruction of the basal or the suprabasal layers of the oral epithelium, resulting in the formation of vesicles or bullae.
• Similar bullae formation may also be seen within enamel organ of the developing tooth germ.

Question  117. Where does the epithelial bulla develop in different clinical forms of the disease in epidermolysis bullosa?
Answer. Mucosal bulla formation occurs at different planes in various forms of epidermolysis bullosa:

• Simplex type: Bulla forms within the epithelium (intraepithelial)
• Junctional type: Bulla forms at the level of lamina lucida
• Dystrophic type: Bulla forms at the level of sublamina densa
• Acquired type: Bulla forms at the level of sublamina densa.

Question  118. Name the outline of treatment and management in epidermolysis bullosa.
Answer.

• Systemic steroid therapy
• Immunosuppressive drug therapy
• Avoidance of trauma.

Question  119. Defie lupus erythematosus.
Answer. Lupus erythematosus is an autoimmune disorder, characterized by destruction of the tissue due to the deposition of autoantibody and immune complexes within it.

Question  120. Which types of autoantibodies are often present in lupus erythematosus?
Answer. Antinuclear antibodies are the most predominant type; however in this disease autoantibodies can also develop against the antigenic determinants of basal cells, collagen, vascular tissue, DNA and other nuclear and ribonuclear proteins.

Question  121. Name the different types of lupus erythematosus lesions.
Answer. These are two major types:

• Systemic lupus erythematosus (SLE)
• Discoid lupus erythematosus (DLE).

Question  122. Defie systemic lupus erythematosus (SLE).
Answer. SLE is the autoimmune disorder, which frequently produces lesions in the skin and oral mucous membrane, and besides this, it also involves multiple other body systems.

Question  123. Describe the typical appearance of skin lesion in SLE.
Answer. Skin lesions of SLE are characterized by the development of fied, erythematous rashes that often have a butterfl confiuration over the malar region and across the bridge of the nose. The skin rash produces itching or burning sensation, which is often aggravated by the exposure to sunlight. The disease often causes hyperpigmentation of the skin.

Question  124. What happens to the hair over the scalp in SLE?
Answer. There is often patchy or extensive loss of hair from the scalp and this is an important clinical fiding in lupus erythematosus (hair may grow once again after successful treatment in case of SLE, but not in case of DLE).

Question  125. Describe the oral lesions of SLE.
Answer. Oral lesions appear as white, hyperkeratotic plaque-like areas in oral mucosa that often resemble lichen planus. Occasionally, there may be presence of erythematous patches, hemorrhagic nodules or painful ulcers in the mouth.

Question  126. What is lupus cheilitis?
Answer. In SLE sometimes the white, hyperkeratotic plaque-like lesions may appear on the vermillion border of the lower lip and the condition is known as lupus cheilitis.

Question  127. Name the systemic manifestations of SLE.
Answer.

Question  128. Name the diseases which often coexist with SLE.
Answer.

• Sjogren’s syndrome
• Raynaud’s phenomenon
• Scleroderma
• Pemphigoid
• Erythema multiforme
• Pemphigus.

Question  129. Describe the histopathological appearance of oral lesions of SLE.
Answer. The oral lesions of SLE often exhibit atrophy with hyperkeratinization of the oral epithelium, liquefactive degeneration of the basal cell layer and lymphocytic infitration and firinoid degeneration of the collagen fiers.

Question  130. What is discoid lupus erythematosus (DLE)?
Answer. DLE is a dermal lesion in which, oral lesions are also commonly seen, and unlike SLE this disease is not associated with autoantibody productions.

Question  131. Describe the skin lesions of DLE.
Answer. The skin lesions in DLE appear as slightly elevated, red or purple macules, which are often covered by a yellow or gray scale. Upon forceful removal of the covering scale, numerous carpet track extensions of the pialo-sebaceous channels appear.

Question  131a. In which disease carpet track extensions are seen?
Answer. Discoid lupus erythematosus.

Question  132. Describe the oral lesions of DLE.
Answer. The oral lesions of DLE include multiple white plaques in the mucosa with central atrophy; there can also be presence of shallow ulcers or reddish-purple erosions, etc. The white plaque-like lesion extends peripherally and gradually it’s central area becomes red and ulcerated; while the border remains white and keratotic.

Question  133. How the gingival lesions appear clinically in DLE?
Answer. Small slit-like ulcers are often seen near the gingival margin in DLE.

Question  134. What is the common feeling in the oral mucosa among patients with DLE?
Answer. Pain and burning sensations are common in the mouth in patients with DLE.

Question  135. Which other pathological entity often resemble oral lesions of DLE?
Answer. Often these oral lesions resemble leukoplakia.

Question  136. Describe the histology of oral lesions of DLE.
Answer. Atrophy with Hyperortho- or parakeratinization of the surface epithelium, liquefactive degeneration of the basal cell layer and inflmmatory cell infitration in the connective tissue.

Question  137. What is lupus erythematous (LE) cell inclusion phenomenon in SLE?
Answer. Lupus erythematous (LE) cell inclusion phenomenon is a specifi laboratory test for SLE. If the serum from a patient suffering from SLE is added to the buffy coat of normal blood, a typical LE cell will develop, which is characterized by a large, circular, basophilic inclusion within a neutrophil. These are neutrophilic leukocytes, which have phagocytosed other leukocytes.

Question  138. Is LE cell inclusion phenomenon also positive in DLE?
Answer. Positive LE cell test is rare in DLE.

Question  139. Besides the LE cell test, what are the other common laboratory investigations done in SLE?
Answer.

• Anti-nuclear antibodies are present in the serum
• Anti-DNA-antibodies are also present
• Polyclonal hyperactivity of the B-lymphocytes
• Decrease in the number of suppressor cells
• Blood examination reveals-leukopenia, thrombocytopenia, hemolytic anemia
• Hypergammaglobulinemia and profuse proteinuria.

Question  139a. What are antinuclear antibodies (ANA)?
Answer. Anti-nuclear antibodies are autoantibodies which specifially react against the normal contents (proteins) of cell nucleus.

Question  139b. What is the other name of antinuclear antibody (ANA)?
Answer. It is also called anti-nuclear factor (ANF).

Question  139c. Name the disease in which antinuclear antibody (ANA) are positive.
Answer.

• Lupus erythematosus
• Scleroderma
• Sjögren’s syndrome
• Polymyositis
• Rheumatoid arthritis.

Question  140. Describe the result of direct immunoflorescence test in SLE and DLE.
Answer. Direct immunoflorescence test reveals deposition of immunoglobulins (e.g. IgG, IgA, IgM and complement 3) in the basement membrane zone of the epithelium and skin, it can be observed in both SLE and DLE.

Question  141. What is the treatment of choice in lupus erythematosus?
Answer. Systemic steroid therapy is the treatment of choice in lupus erythematosus.

Question  142. Defie scleroderma.
Answer. Scleroderma or progressive systemic sclerosis is a complex multisystem autoimmune disease, characterized by progressive diffuse subcutaneous firosis (sclerosis) of the skin, widespread submucous firosis in oral cavity and disorders in multiple internal organs including the GI tract, lungs, heart and kidney, etc.

Question  143. How does the disease scleroderma develop?
Answer. It occurs because of circulatory insuffiiency in the tissue due to abnormalities in the arterioles and blood capillaries which cause replacement of the normal connective tissue by the dense collagen bundles leading to tissue firosis or sclerosis.

Question  144. Name the types or forms of scleroderma.
Answer. Scleroderma usually occurs in two basic forms:

• Localized or morphea form
• Generalized or diffuse form.

Question  145. Which type of scleroderma is more common among the two?
Answer. Generalized or diffuse scleroderma is the more common type.

Question  146. How the localized or morphea form of scleroderma clinically appears?
Answer. It produces localized firosis in the skin with development of single or multiple, red orivory colored, smooth, hard patches.

Question  147. Which facial anomaly can develop as a result of morphea form of scleroderma in children?
Answer. Childhood morphea form of scleroderma may be responsible for the development of facial hemihypertrophy.

Question  148. Which type of environmental hazard can be responsible for the occurrence o f scleroderma?
Answer. Scleroderma is more prevalent in areas where silicosis is a common environmental hazard.

Question  149. What is the age and gender predilection of scleroderma?
Answer. Scleroderma usually occurs among children or young adults and it frequently affects the females.

Question  150. How the diffuse scleroderma begins?
Answer. The disease begins with edema of the skin over the face, hands or trunk.

Question  151. Describe the clinical manifestations of diffuse scleroderma.
Answer.

• Thinning, stiffening and fiation of skin to the underlying tissues, e.g. muscles and bones, etc.
• Presence of multiple telangiectatic spots, contractures, reddish patch and scar in the skin
• Skin becomes yellow, gray or ivory white in color due to deposition of calcium in the subcutaneous tissue
• Fixation and stiffness of the skin leading to progressive immobility of hands and joints along with many internal and external organs
• Weakening of the limbs with a typical claw-like appearance of the figers.

Question  152. Why claw-like appearance of the figers occurs in diffuse scleroderma?
Answer. Claw-like appearance of the figers means patient is unable to straighten the figers of her/his hands; it can happen in diffuse scleroderma due to resorption of terminal phalanges and flxion contractures.

Question  153. What is calcinosis cutis?
Answer. In diffuse scleroderma, the skin gradually becomes yellow, gray or ivory white in color due to calcium deposition in the subcutaneous tissue; and this phenomenon is called calcinosis cutis.

Question  154. Name the typical facial appearance of patients suffering from diffuse scleroderma.
Answer. In this disease patient often have a typical mask-like face, which is also called Monalisa face because wrinkles or lines of facial expression do not develop in patient face and their eyes become narrow because of the disease.

Question  155. Why the mask-like facial appearance develops in patients with scleroderma?
Answer. This typical facial appearance or expression in scleroderma develops due to fiation of the facial skin to the underlying structures.

Question  156. What is ‘mouse facies’?
Answer. Patients with severe scleroderma may have atrophy of the ala of the nose with a pinched appearance of the nose; this often gives rise to facial profie called mouse facies.

Question  157. What is CREST syndrome?
Answer. CREST syndrome is often associated with scleroderma and it includes the following

• symptoms:
• Calcinosis cutis
• Reynaud’s phenomenon
• Esophageal dysfunction
• Scleroductyly
• Telangiectasia.

Question  158. What is Reynaud’s phenomenon?
Answer. In Reynaud’s phenomenon, digits of patient’s hands and feet initially become dead white in appearance when exposed to cold; it occurs due to vasospasm. In the later stages, the digits become bluish due to venous stasis.

Question  159. Why esophageal dysfunction occurs in scleroderma?
Answer. In scleroderma, esophageal dysfunction occurs due to firosis of the esophageal mucosa with stricture formation.

Question  160. What is scleroductyly?
Answer. Abnormal collagen deposition in scleroderma causes stiffness and flxure of figers with a claw-like deformity; this phenomenon is called scleroductyly.

Question  161. What is telangiectasia?
Answer. Bleeding from superfiial dilated capillaries produces multiple red macules on the skin, especially on the face and this phenomenon is called telangiectasia.

Question  161a. What is a coup de sabre lesion?
Answer. Coup de sabre is a lesion of linear scleroderma on the forehead and scalp, often associated with hemiatrophy of the face.

Question  162. Name the oral manifestations of scleroderma.
Answer.

Question  163. Describe the radiological features of scleroderma.
Answer.

• Generalized widening or thickening of the periodontal ligament space
• Resorption of mandibular condyle or ramus due to persistent pressure from the contracting facial skin, muscles and joints
• Severe mandibular bone resorption may lead to pathological fracture
• Increased risk of development of osteomyelitis of jaw.

Question  164. Describe the histopathological changes of oral mucosa in scleroderma.
Answer.

• Atrophy of oral epithelium with flttening of the rete-pegs and areas of pigmentations
• Thickening and hyalinization of the collagen fiers in lamina propria
• Atrophy of the minor salivary glands and perivascular infitration of inflmmatory cells
• Decreased vascularity in connective tissue with narrowing of vessels due to perivascular firosis
• Thickening of the periodontal ligament due to increased synthesis of collagen and oxytalan fiers
• Absence of sweat glands, sebaceous glands and hair follicles in the facial skin.

Question  165. What is the treatment of scleroderma?
Answer. No specific treatment is available for scleroderma; systemic steroid therapy may produce partial remission.

Question  166. What is Ehlers-Danlos syndrome?
Answer. Ehlers-Danlos syndrome is a group of hereditary disorders characterized by defective or abnormal collagen synthesis in various body organs.

Question  167. Describe the clinical features of Ehlers-Danlos syndrome.
Answer.

• Hypermobility of joints and increased laxity or hyperelasticity of the skin (patient is often considered rubber man)
• Excessive bruising tendency and defective wound healing due to increased fragility of the skin and blood vessels
• Abnormal scarring of skin in response to minor injuries (papyraceous scarring) and the scarred area of skin appears as crumpled cigarette papers
• Development of aortic aneurysm and its rupture can be life-threatening for the patient.

Question  168. Describe the oral manifestations of Ehlers-Danlos syndrome.
Answer.

• Increased fragility of the oral mucosa
• Positive Gorlin’s sign: Most patients can touch the tip of their nose with the tongue
• Bleeding from the gingiva and oral mucosa
• Mobility of teeth and marked periodontal weakness
• Retarded wound healing
• Enamel hypoplasia
• Loss of normal scalloping of the dentino-enamel junctions of the tooth
• Large pulp stones in the teeth and formation of irregular type of dentine
• Hypermobility and subluxation of the temporomandibular joint
• Diffiulty in suturing the oral wounds due to extreme tissue fragility
• Easy bruising and bleeding following minor oral surgical procedures.

Question  169. How many types of Ehlers-Danlos syndrome exist?
Answer. There are fie types of this syndrome, type I to type V.

Question  170. Describe the type I Ehlers-Danlos syndrome.
Answer. This is the classic and most severe form of the disease; it presents both hyperextensibility of joints and hyperelasticity of the skin along with easy bruisability. The skin often shows ‘crumpled cigarette–paper’ like scars.

Question  171. Describe the type II Ehlers-Danlos syndrome.
Answer. Type II Ehlers-Danlos syndrome often presents features similar to type I, however it is of much milder in nature.

Question  171a. What is the type III Ehlers-Danlos syndrome?
Answer. Type III is characterized by joint instability with frequent joint dislocations with or without trauma; patients often suffer from persistent and severe joint pain and osteoarthritis. However, Joint hypermobility is the hallmark feature of this type, with less severe skin manifestations.

Question  172. Compare between type I and type III Ehlers-Danlos syndrome.
Answer. In type I Ehlers-Danlos syndrome, skin changes are more prominent, e.g. increased fragility (skin tears easily) and easy bruisability, etc. Whereas in type III Ehlers-Danlos syndrome joint hypermobility is the more prominent feature as compared to the skin changes.

Question 173. Describe the type IV Ehlers-Danlos syndrome.
Answer. Excessive scars formation and hyperpigmentation of skin over the areas of bony prominence; the skin is so thin that the subcutaneous vessels are often visible.

Question  173a. What kind of risk is often involved in type V Ehlers-Danlos syndrome?
Answer. Patients may face sudden death due to rupture of large vessels and hollow organs