A young patient has white thickening of buccal mucosa since birth and it also occurs in nose and esophagus. Most likely diagnosis is:

 # A young patient has white thickening of buccal mucosa since birth and it also occurs in nose and esophagus. Most likely diagnosis is: 

a. accumulation of fordyces granules 

b. chronic hyperplastic candidiasis 

c. white sponge nevus 

d. porokeratosis 

The correct answer is C. White sponge nevus. 

This mucosal abnormality is congenital in many instances. In other cases, it does not appear until

infancy, childhood, or even adolescence, by which time it has generally reached the full extent of its severity. The oral lesions may be widespread, often involving the cheeks, palate, gingiva, floor of the mouth, and portions of the tongue. The mucosa appears thickened and folded or corrugated with a soft or spongy texture and a peculiar white opalescent hue. There is sometimes a minimal amount of folding present. Ragged white areas may also be present which can be removed sometimes by gentle rubbing without any ensuing bleeding. 

Organ involvement of SLE is in:

 # Organ involvement of SLE is in:

a. kidney and Lungs 

b. kidney and heart 

c. lungs and heart 

d. heart and spleen 

The correct answer is B. Kidney and heart.

Systemic lupus erythematosus is a serious cutaneous-systemic disorder which characteristically manifests repeated remissions and exacerbations. This disease has its peak age of onset at about 30 years in females but about 40 years in males. The disease may occur in childhood as reported by Jacobs. Prevalence rates are higher in females than in males. A female-to-male ratio of approximately 2 : 1 occurs before puberty, and a ratio of 4 : 1 occurs after puberty.

The generalized manifestations of the systemic disease are referable to the involvement of various organs, including the kidney and heart. In the kidney, fibrinoid thickening of glomerular capillaries occurs, producing the characteristic ‘wire loops,’ which may be sufficient to result in renal insufficiency. The heart may suffer from an atypical endocarditis involving the valves, as well as fibrinoid degeneration of the epicardium and myocardium. The widespread tissue involvement and the nature of the lesions have led to the inclusion of this disease in that group known as the ‘collagen diseases,’ which also includes rheumatic fever, rheumatoid arthritis, polyarteritis nodosa, scleroderma and dermatomyositis.

Prodromal symptom of OSMF:

 # Prodromal symptom of OSMF:

a. hypersalivation 

b. dryness of mouth 

c. both of the above 

d. none of the above 

The correct answer is C. Both of the above.

Prodromal Symptoms (Early OSF):

 This includes a burning sensation in the mouth when consuming spicy food, appearance of blisters especially on the palate, ulcerations or recurrent generalized inflammation of the oral mucosa,

excessive salivation, defective gustatory sensation and dryness of the mouth. There are periods of exacerbation manifested by the appearance of small vesicles in the cheek and palate. The intervals between such exacerbation vary from three months to one year. Focal vascular dilatations manifest

clinically as petechiae in the early stages of the disease. This may be part of a vascular response due to hypersensitivity of the oral mucosa towards some external irritant like arecanut products. Petechiae are observed in about 22% of OSF cases (Rajendran, 1994), mostly on the tongue followed by the labial and buccal mucosa with no sign of blood dyscrasias or systemic disorders. Pain in areas where submucosal fibrotic bands are developing when palpated is a useful clinical test. Histologically, they revealed a slightly hyperplastic epithelium, sometimes atrophic with numerous dilated and blood-filled capillaries juxtaepithelially. The inflammatory cells seen are mainly lymphocytes, plasma cells and occasional eosinophils. The presence together of large numbers of lymphocytes and fibroblasts as well as plasma cells in moderate numbers, suggests the importance of a sustained lymphocytic infiltration in the maintenance of the tissue reaction in OSF. 

Pathergy test is done in:

# Pathergy test is done in: 

a. bechet syndrome 

b. senear usher syndrome 

c. weber cockayna syndrome 

d. epidermolysis bullosa 

The correct answer is: A. Bechet syndrome.

Pathergy phenomenon has been well known to dermatologists since it was first described in 1937 by Blobner as a state of altered tissue reactivity in response to minor trauma. The pathergy test is a nonspecific hypersensitivity skin reaction induced by needle prick that is performed to look for evidence of this phenomenon. Pathergy lesions are generally manifested clinically by erythematous induration at the location of skin trauma, which may remain as papules or progress to sterile pustules. Although the precise mechanism of pathergy has not yet been entirely elucidated, the skin injury by needle prick in patients exhibiting pathergy is thought to trigger a cutaneous inflammatory response that is exaggerated and more prominent than that seen in normal skin. An increased release of cytokines from cells in the dermis or epidermis is implicated in this aberrant reaction, which results in the perivascular infiltrates that are characteristically observed on histopathologic studies. While pathergy has been reported in numerous diseases, pathergy testing is primarily used in the diagnosis of Behcet Disease (BD).

Which of the following is not a characteristic of Paterson-brown-kelly syndrome?

 # Which of the following is not a characteristic of Paterson-brown-kelly syndrome? 

a. koilonychia 

b. dysphagia 

c. iron deficiency anemia 

d. none of the above 

The correct answer is D. None of the above.

Iron Deficiency Anemia and Plummer-Vinson Syndrome

(Paterson-Brown-Kelly syndrome, Paterson-Kelly syndrome, sideropenic dysphagia)

Iron deficiency is an exceedingly prevalent form of anemia, particularly in females. Iron deficiency is the most prevalent single deficiency state on a worldwide basis. It has been estimated that between 5 and 30% of women in the United States are iron deficient, while in some parts of the world, this may reach 50% . Men are only rarely affected. 

While an iron-deficiency anemia may occur at any age, the Plummer-Vinson syndrome occurs chiefly in women in the fourth and fifth decades of life. Presenting symptoms of the anemia and the syndrome

are cracks or fissures at the corners of the mouth (angular cheilitis), a lemon-tinted pallor of the skin, a smooth, red, painful tongue (glossitis) with atrophy of the filiform and later the fungiform papillae, and dysphagia limited to solid food resulting from an esophageal stricture or web. These oral findings are reminiscent of those seen in pernicious anemia. The mucous membranes of the oral cavity and esophagus are atrophic and show loss of normal keratinization. Koilonychia (spoon-shaped fingernails) or nails that are brittle and break easily have been reported in many patients; splenomegaly has also been reported in 20–30% of the cases.

Osteogenesis imperfecta:

 # Osteogenesis imperfecta: 

a. Is a sex linked disorder of bones that develop in cartilage

b. Manifests with blue sclera which are pathognomonic of this disease 

c. May be associated with deafness 

d. Has associations with amelogenesis imperfecta

The correct answer is B. Manifests with blue sclera which are pathognomonic of this disease.

A second characteristic clinical feature of osteogenesis imperfecta is the occurrence of pale blue sclerae. The sclerae are abnormally thin, and for this reason the pigmented choroid shows through and produces the bluish color. However, the appearance of blue sclera is not confined to this disease since it may also be seen in osteopetrosis, fetal rickets, Turner syndrome, Paget’s disease, Marfan syndrome, and Ehlers-

Danlos syndrome, as well as in normal infants. While the blue sclerae are a prominent sign in this disease, they are not invariably present. In a series of 42 patients reported by Bauze and his associates, 12 of the patients had white sclerae, and these were generally found in the older patients with the more severe disease and earlier onset of fractures. 

Radiographic finding in Pindborg tumor:

 # Radiographic finding in Pindborg tumor: 

a. Sun-burst appearance 

b. Onion-peel appearance 

c. Driven-snow appearance 

d. Cherry-blossom appearance 

The correct answer is C. Driven snow appearance.

A calcifying epithelial odontogenic tumor (Pindborg tumor) may show considerable radiographic variation. In some cases, the lesion appears as either a diffuse or a well-circumscribed unilocular radiolucent area, while in other cases there may appear to be a combined pattern of radiolucency and radiopacity with many small, irregular bony trabeculae traversing the radiolucent area in many directions, producing a multilocular or honeycomb pattern. Scattered flecks of calcification throughout the radiolucency have given rise to the descriptive term of a ‘driven snow’ appearance. In some instances, the lesion is totally radiolucent and is in association with an impacted tooth, thus leading to a mistaken clinical diagnosis of a dentigerous cyst.